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190 Cards in this Set
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What are the compensations for decr red cell mass to maintain O2 to tissue? x4
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i)incr CO (HR and SV) ii)Incr extraction ratio iii)Rt shift of curve (incr 2,3 DPG) iv)Expand plasma vol
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When is blood xfusion needed? x2
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i)Hb less than 7 ii)Need for incr O2 carrying capacity (If have CAD or cardiopulm dz)
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What are imp history things to ask in anemics?
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i)hemophilia in family ii)chronic dz (ESRD) iii)Bleeding (melena, recent trauma/sx, hematemesis) iv)EtOHism
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What are 5 non specific complaints for anemics? What are 3 specific things?
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A)i)nausea ii)diarrhea iii)fatigue iv)poor concentration v)h/a. B)i)pallor ii)hypotension iii)tachycardic
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How much does 1 unit pRBCs increase the Hb? What is pseudoanemia? How much does 1 unit plt xfusion increase plt count?
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i)1 Hb ii)dilution of the plasma, causing decr in Hb/Hct
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What is not in pRBCs? What should and shouldn't you mix with? What to do after xfusion?
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i)no plts, clot factors ii)mix w/NS, not Lactated ringer b/c Ca causes coagulation in IV line. iii)check CBC after
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What does FFP have? What doesn't it have? What is it for x3? When to give x2?What to do after?
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i)all clotting factors ii)no RBC, WBC, plt iii)a)coagulopathy b)high PT/PTT c)deficiency of clotting factors iv)a)if can' wait for Vit K b)liver dz
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What is in cryoprecipitate and when to give x3?
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i)Factor VIII and Fibrinogen ii)a)Hemophilia A b)decr fibrinogen (DIC) c)vWD
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What is effective erythropoiesis dependant on x4?
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i)adequate raw materials (Fe, Vit B12, folate)in bone marrow ii)absence of intrinsic BM dz (aplastic anemia) iii)adequate EPO from kidney iv)survival of reticulocytes
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What does retic count >2% mean?
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excessive loss or hemolysis
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What are the 2 types of hemolytic xfusion rxns? what are they due to? What are 3 sxs of each? What is management for each?
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i)intravascular hemolysis (complement mediated) and immediate: a)ABO mismatch b)Fever/chills, N/V, pain in flanks/back, CP c) stop xfusion immediately and replace fluid to avoid shock and RF ii)extravascular hemolysis (3-4 weeks)a)minor RBC Ags b)Fever, jaundice, anemia c)no management
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What to do if Retic Index <2? What is ddx of microcytic x4? Macrocytic x3?Of normocytic? x5
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i)examine smear and RBC indices ii)a)Fe def b)Chronic dz c)thalassemia d)sideroblastic (Pb, pyridoxine, toxic effects of EtOH) iii)a)nuclear defect (B12, folate) b)liver dz b/c of altered plasma lioprotein metab into the membrane, making it bigger c)EPO stimulation iv)a)aplastic anemia b)BM fibrosis c)tumor d)ACD e)RF (decr EPO made)
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What are 2 categories of causes of microcytic anemia? What are 2 examples of each?
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i)chronic blood loss: a)menstrual loss b)GI loss ii)Dietar def/incr need: a)infant and toddlers b/c accel growth and occurs if diet is human milk (low in Fe). b)Adolescents: rapid growth, esp adolescents b/c of period c)pregnant woman needs Fe
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What are 5 clinical features of Fe def anemia?
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i)pallor ii)fatigue, general weakness iii)DOE iv)orthostatic lightheadedness v)hypotension and tachycardic
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What lab tests are seen in microcytic anemia? When is BM bx indicated?
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i)a)low Fe b)incr TIBC/transferrin c)low ferritin d)small RDW ii)indicated if lab evidence w/o source of blood loss
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What is treatment of Fe def anemia? when do you use each kind? x3
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i)oral Fe; need to also find source of blood. S/E of dyspepsia, constipate, nausea ii)parenteral: if poor absorption, need more oral tx than can be given, or if oral not tolerated. iii)xfusion: only if anemia is severe or if cardiopulm dz is present
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What is pathophys of B thal? What people are they seen in? What is pathophys of a thal? Who are they seen in?
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i)excess a chains bind to and damage RBC membrane. ii)seen in mediterranean, middle eastern, indian iii)decreased a chain which is part of all Hb, and B chains form tetramers (abnl Hb). iv)Afr Am
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Who does thal major occur in? what are 5 clinical features? How to DX? What is treat?
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i)Mediterranean ii)a)severe anemia b)massive HSM c)expanded marrow space->get distorted bones d)growth retard e)die of progressive CHF if untreated iii)Dx w/Hb electrophoresis (incr HbF) iv)Treat w/RBC xfusions, and can get Fe o/l, so get CHF (like hemochromatosis). Give desferrioxamine (chelator) w/therapy
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What is clinical features of thal minor? How to Dx? What is Treat? How are thal's usually dx'd?
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i)asx, just hypochromic micro anemia ii)Hb electrophoresis iii)No treatment necessary iv)if not responding to Fe, do an electrophoresis
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What are the 4 kinds of a thal and what are 2 things imp for each?
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i)silent carrier: 1 locus; asx, nl H/H. ii)a thal trait: 2 loci; mild micro hypochromic anemia; Afr Am. iii)HbH dz: 3 a loci; hemolytic anemia, splenomegaly; Electrophoresis shows HbH and micro hypochromic anemia; Treat is RBC xfusions iv)hydrops fetalis and fatal.
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most common thalassemia?
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B minor thal>a thal minor. Both mistaken for Fe def
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What is sideroblastic anemia caused by? What are acquired causes x3? What are clinical findings x3? What is treat?
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i)abnormality in RBC Fe metab ii)drugs (INH, EtOH); Pb exposure; Coll Vasc Dz; neoplastic dz iii)incr Fe and ferritin; normal TIBC; ringed sideroblasts in BM. iv)remove offending agents; consider pyridoxine
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What situations does anemia of chronic dz occur in? What are the lab findings? what kind of anemia is it? how to tx?
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i)chronic infection (TB, lung abscess); Cancer; inflamm; trauma. Inflamm cytokines suppresses effect on Erythropoiesis. ii)Low serum Fe, incr ferritin, low TIBC, low transferrin. iii)normocytic and chromic, but can be microcytic and hypochromic iv)Tx disease, no Fe
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What is pancytopenia? What are causes of aplastic anemia x4?
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i)neutropenia, thrombocytopenia, anemia. ii)a)idiopathic b)rads exposure c)meds: chloramphenicol, sulfonamides, carb d)virus: parvo, HCV, HBV, EBV, CMV, HIV, HZV
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What are symptoms and signs of pancytopenia x3? What can it progress to? How to DX for definite dx? What is treatment x3?
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i)a)sxs of anemia: fatigue, dyspnea b)signs and sxs of thrombocytopenia: petechiae, easy bruising c)incr incidence of infections: neutropenia d)can progress acute leukemia ii)a)BM bx: hypocellular marrow and absence of progenitors b)a)BM xplant b)xfuse pRBCs and plts c)immunosuppression
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What is Vit B12 for? x2. What are the main sources in diet? How and where is it absorbed?
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i)a)cofactor in conversion of homocysteine to methionine b)cofactor in conversion of methylmalonyl CoA to succ CoA. ii)a)meat b)fish iii)bound to IF (made by parietal) so absorbed by terminal ileum
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what are 5 causes of B12 def macro anemia?
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i)pernicious ii)diet: EtOH or vegetarian iii)gastrectomy iv)Crohn's: lose terminal ileum v)organisms: D. latum; blind loop syndrome (bacterial overgrowth)
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What are clinical features B12 def anemia? x4
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i)sore tongue, glossitis ii)neuropathy (diff from folate def): a)demyelination in posterior columns, LCST, spinocerebellar tracts so get loss of position/vibratory sensation in lower extremities, ataxia, UMN loss b)urinary and fecal incontinence, impotence c)dementia
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How to DX B12 def? x4
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i)peripheral blood smear: large RBCs and hypersegmented neutrophils ii)Low serum B12 iii)serum methylmalonic acid and homocysteine levels: elevated in B12 def and useful if B12 is borderline iv)Schilling test: separates pernicious from malabsorption
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How to treat B12 def? What is one possible presentation that makes it imp to DX?
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i)parenteral ii)mod to severe neuro sxs w/little to no anemia
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Where does folate come from and how can this be removed? What are 5 causes? How to treat? What is diff b/w folate and B12 def in terms of homocys and methylmalonic?
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i)from green leafy veggies, removed by cooking ii)a)tea and toast (no intake); b)EtOH c)Longterm Abx d)folate antags e)hemolysis iii)oral folate iv)both incr homocys, but methylmalonic levels only incr w/B12 def.
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What are 2 ways of classifying hemolytic anemias? x2
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i)extrinsic causes (acquired) vs intrinsic causes (inherited) ii)intravascular hemolysis vs extravascular hemolysis (mostly spleen)
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What are types of extrinsic and intrinsic RBC defects? x3 for each
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i)extrinsic: a)immune hemolysis b)mechanical hemolysis: prosthetic heart valves, MAHA c)meds, burns, toxins (brown recluse), infection ii)intrinsic: a)Hb abnormality like SCD, HbC, thalassemia b)membrane defect: hereditary sphero, PNH c)G6PD def, PK def
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What are clinical features of hemolytic anemias other than obvious? x3
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i)dark urine->hemoglobinuria ii)cholelithiasis iii)lymphadenopathy
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What does spherocytes suggest? Shistocytes? Heinz Body?
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i)extravascular hemolysis ii)intravasc hemolysis iii)Heinz bodies in G6PD.
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What is haptoglobin levels in hemolytic anemias? Why?
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i)low in hemolytic anemia, esp intravascular hemolysis ii)b/c hapto binds to Hb, so absence means Hb was destroyed.
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What does elevated unconj bili mean? What does direct coombs test mean?
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i)degradation of heme b/c RBCs destroyed ii)detects Ig or complement on RBC membrane: positive in AIHA
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How to treat hemolytic anemia? x3
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i)treat underlying cause ii)xfuse RBCs if severe or hemodynamically compromised. iii)folate supplements b/c lost in hemolysis
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What is genetics of SCD? Under what conditions do peeps get exacerbation and why? Which people get sickle cell trait? x4. What does survival correlate with?
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i)AR ii)hypoxia, acidosis, change in temp, dehydration, infection: decreased oxygen causes polymerization of Hb molecules, and get sickled RBCs->ischemia. iii)AA, italian, greek, Saudi arabian iv)more frequent crises means shorter lifespan.
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What are the findings of SCD in terms of anemia?
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i)jaundice, pallor ii)gallstones iii)rarely xfusion dependent iv)high output HF v)aplastic crisis 2/2 to Parvo B19: reduces ability of BM to compensate; tx w/xfusion
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What are findings of Sickle cell 2/2 to vaso-occlusion? x5 (part 1)
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i)painful crises due to bone ii)hand foot syndrome: swelling of hands and feet 2/2 to avascular necrosis iii)acute chest syndrome: presents like pneumonia (CP, resp distress, pulm infiltrates) due to pulm infarcts iv)repeated splenic infarcts v)avascular necrosis of joints: hip and shoulder
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What are findings of Sickle 2/2 to vasoocclusion x6 (pt 2)
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i)priapism: vaso-occluded erection; hydralazine or nifedipine or antiandrogen. ii)CVAs in kids iii)ophtho: retinopathy, retinal infarct, retinal detach iii)renal papillary necrosis w/hematuria (usually painless) iv)leg ulcers, usually over lat malleoli v)ab crisis: like acute abdomen
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Which bugs in Sickle cell and why?
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i)functional asplenia->infections ii)H flu, Strep pneum, salm OM
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How to dx Sickle cell? x2
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i)peripheral smear ii)Hb electrophoresis required for DX
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What is splenic sequestration crisis?
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SCD: potentially fatal in kids (b/c intact spleen): sudden pooling of blood into spleen->splenomegaly and hypovolemic shock
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What advice for Sickle cell Dz pts? x3
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i)avoid high altitudes (low O2 precipitates) ii)maintain fluids (dehydration precipitates) iii)treat infections promptly (infections precipitate)
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How to treat sickle cell DZ? x5
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i)early vaccination for S pneum, H flu, N meningitis ii)prophylaxis PCN kids from <4 mo->6 y/o iii)folic acid supplement: b/c chronic hemolysis iv)hydroxyurea to incr HbF levels to interfere w/sickling: decr painful crises and faster leg ulcer healing v)xfusions: not based on Hb level but if acute chest, stroke, refract priapism, cardiac decompensation
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How to manage painful crises? x4
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i)hydration: oral if mild episode, NS IV for severe ii)morphine for pain control iii)keep pt warm iv)supplemental O2 if hypoxic
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What are causes of spherocytosis? x5
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i)hereditary spherocytosis ii)G6PD def iii)ABO incompatibility (not Rh) iv)hyperthermia v)AIHA
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Genetics of spherocytosis? pathophys?
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i)AD ii)loss of RBC membrane SA w/o reduction in RBCV->trapped in spleen by macros->extravascular hemolysis
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What are 3 features of spherocytosis? How to treat?
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i)hemolytic anemia ii)splenomegaly iii)hemolytic crises iv)splenectomy
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How to DX spherocytosis? x4
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i)RBC osmotic fragility to hypotonic saline: undergo lysis at a higher (earlier) oncotic pressure ii)spherocytes on smear iii)negative direct coombs: distinguishes from AIHA
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What is genetics of G6PD def? What are precipitants x5?
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i)X link ii)a)nitrofurantoin b)sulfonamide c)primaquine d)fava e)infection
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What is diff b/w Afr am men form vs mediterranean form? x3
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i)Afr: a)self limited b/c only old RBCs w/insuff G6PD b)trigger: infection or drugs (primaquine or sulfurs) ii)a)young RBCs affected also b)fava beans c)may need xfusions until drug eliminated
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What are clinical features of G6PD def? x2
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i)episodic hemolytic anemia usually drug induced ii)dark urine and jaundice on PE
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What is pathophys of G6PD def?
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excess H2O2 denatures Hb giving Heinz bodies inside RBC->attach to RBC membrane and reduce flexibility so sequestered in spleen
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How to DX G6PD x2?
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i)Smear: bite cells (from macros); Heinz bodies ii)Deficient NADPH formation on G6PD assay. iii)G6PD level measurement is diagnostic, but will be normal during hemolytic episode b/c G6PD deficient RBCs already have been destroyed.
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How to treat G6PD? x3
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i)avoid drugs ii)maintain hydration iii)perform RBC xfusion if needed
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What is AIHA? What does type of Ig determine? What is the course (who is it worse in?)
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i)Igs to RBC membrane Ag causing destruction ii)IgG vs IgM determines a)prognosis, site of RBC destruction and response to treatment iii)kids is worse than parents
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What type of Ig in warm AIHA? What is the type of hemolysis? where is RBC sequestration and therefore what happens? What are causes x4
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i)IgG binds at 37 C ii)extravascular ii)spleen->splenomegaly iii)a)leukemia b)lymphoma c)other malig d)CVD like SLE e)a-methyldopa
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What type of Ig is cold AIHA? What is type of hemolysis? What are 2 causes?
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i)IgM ii)complement activation leads to intravasc hemolysis->sequester in liver iii)a)idiopathic b)infection (Myco pneumonia, infectious mono)
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How to DX AIHA? x3
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i)Direct Coombs: a)If RBCs coated w/IgG, then warm AIHA b)if coated w/complement alone, then cold AIHA ii)spherocytes may be in warm AIHA iii)pos cold agglutinin titer=cold AIHA
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What is treatment of each type of AIHA if severe? (usually not severe hemolysis)
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i)warm: a)steroids b)splenectomy if not responsive to steroids c)folic acid d)immunosuppression might help ii)Cold: a)avoid cold exposure b)xfusions if needed c)chemotherapeutics d)NO STEROIDS
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What is PNH? What is it caused by?
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i)acquired disorder caused by deficiency of anchor proteins linking complement inactivating proteins to blood cell membranes. Get susceptibility to complement mediated lysis of RBCs, WBCs, plts
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What are clinical features of PNH? x5
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i)intravasc hemolysis: get paroxysmal hburia and elevated LDH ii)normochromic normocytic anemia iii)pancytopenia iv)thrombosis of venous systems (ie Budd chiari) v)can get aplastic anemia, myelodysplasia, myelofibrosis, acute leukemia
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How to DX PNH? x3. Which is best test?
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i)Ham test: pt cells acidified->alt complement pthway triggered->lysis of PNH but not nl cells. ii)sugar water test: pts serum mixed w/sucrose->hemolysis ensues in PNH iii)flow cytometry of anchored cell surface proteins (CD55, 59); best test
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What is TX of PNH? x2
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i)steroids are initial, but many don't respond ii)BM xplant
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What are 5 causes of thrombocytopenia?
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i)decr production ii)incr destruction iii)sequestration from splenomegaly iv)dilutional after xfusion or hemorrhage v)pregnant: nl or part of HELLP
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What are 3 causes of decr production of plts and 3 examples of each
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i)BM failure: a)acquired (aplastic anemia) b)congenital (fanconi's) c)congenital intrauterine rubella. ii)BM invasion: a)tumor b)leukemia c)fibrosis. iii)BM injury: a)drugs (ethanol, gold, cancer chemo) b)chemicals c)infection d)radiation
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What are the 2 types of thrombocytopenia 2/2 to increased destruction, and 4 exs of each?
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i)immune: a)infection, drug induced, ITP, SLE, HIT type 2, HIV ass'd b)nonimmune: DIC, TTP, HIT type 1
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What are clinical features in thrombocytopenia x4. What isn't seen?
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i)cutaneous bleeding: petechiae; ecchymoses at sites of minor trauma ii)mucosal bleeding: epistaxis, hemoptysis, bleed in GI and GU iii)excessive bleed after procedures iv)intracranial hemorrhage and heavy GI bleed when plts very low v)no hematomas or hemarthroses
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How to TX thrombocytopenia? x3
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i)treat underlying cause ii)xfusion: depends on cause and severity iii)d/c NSAIDs, other antiplt agents, anticoagulants
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What is ITP due to?
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i)autoimmune antibody formation against host plts. Antiplt IgG coat and damage plts, which are then removed by splenic macros
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What are the 2 forms of ITP?
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i)acute form: kids, preceded by viral infection, self limited: 80% resolve w/in 6 mths. ii)Chronic: adults, mostly women b/w 20-40; rare spontaneous remissions
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What are clinical features of ITP? x3
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i)petechiae and ecchymoses on skin ii)bleeding of mucus membranes iii)no splenomegaly
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How to DX ITP? x4
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i)plt count <20K w/nl H/H unless significant bleeding happened. ii)peripheral smear->decr plts iii)BM aspiration=incr megakaryocytes iv)incr amt of plt ass'd IgG
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What is TX of ITP? x4
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i)adrenal steroids ii)IV Ig: saturates RES bind sites for plt bound self Ig-> less plt uptake and destruction by spleen iii)splenectomy: induces remission iv)plt xfusion in life threatening and severe hemorrhage
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What is cause of TTP? What is pathogenesis?
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i)rare dz w/unknown origin ii)hyaline microthrombi (mostly plts) occlude small vessels in any organ. Get mechanical damage to RBCs w/schistos. life threatening!
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What are clinical features of HUS vs TTP?
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i)HUS=MAHA; Thrombocytopenia; renal failure. ii)TTP=HUS + fever + altered mental status
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What is TX of TTP? x2 What is contraindicated
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i)plasapheresis (large vol): monitor plts which will increase. ii)steroids and splenectomy: sometimes beneficial iii)plt xfusions!
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What is genetics of Bernard Soulier? What is it 2/2 to? What are clinical features? x2
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i)AR ii)d/o of plt adhesion due to def of plt glycoprotein GpIb-IX. ii)a)plts abnormally large b)plt count is mildly low
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What is genetics of Glanzmann's thrombasthenia? What is it due to? What is BT like? What is plt count like?
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i)AR ii)d/o of plt aggregation 2/2 to deficiency in plt glycoprotein GP2b-3a. iii)BT is prolonged; plt count is normal
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What is genetics of vWD? What is it? What does vwF do? Where synthed?
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i)AD ii)decreased vWF (type 1) to absent vWF (type 3). iii)vWF's fcn a)enhance plt aggregation and adhesion (1st steps in clot formation). b)carrier for factor 8. iv)synth'd in endo cells and megakaryocytes
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What does factor 8 do? What are the 2 portions of the molecule?
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i)fibrin clot formation. ii)F 8 has coagulant portion (F 8 coag protein) and antigenic portion (ass'd w/vWF)
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What are 3 clinical features of vWD?
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i)cutaneous/mucosal bleeds: epistaxis, easy bruising, gingival bleeding ii)GI bleed iii)menorrhagia
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How to DX vWD? x3
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i)prolonged BT w/normal plt count: can have long PTT. ii)decr plasma vWF, decr factor 8 activity iii)decr ristocetin induced plt aggregation
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What is treatment of vWD x2? What is not recommended? x2
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i)DDAVP: induces endothel cells to secrete vWF; esp for type 1>2>3 ii)F 8 concentrates: give to all w/vWD after surgey or major procedures; recmmend for type 3 iii)no cryoprecipitate b/c of viral xmission risk; no ASA/NSAIDs
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What is Hemophilia A's genetics and which sex? What protein is deficient?
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i)X-linked ii)male iii)F VIII
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What are 5 clinical features of hemophilia A?
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i)hemarthrosis: get blood and then joint destruction (knee). maintain F VIII levels; if recurrent->synovectomy ii)intracranial bleed: watch out for head trauma iii)IM hematoma iv)retroperitoneal hematoma v)hematuria or hemospermia
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What dz is common in hemophilia A? How to detect F VIII inhibitor (test)?
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i)HIV: donors ii)mix hemophiliac blood w/normal->normal PTT. If not normal->presence of F VIII inhibitor
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How much F VIII do you need b/4 it becomes clinical? How much is there if severe case?
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i)10% ii)<1%; get spontaneous hemarthrosis, severe hemorrhage s/p sx or trauma.
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How to treat hemarthrosis in hemophilia A? How to treat hemophilia A? x2
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i)acute hemarthrosis: analgesia (codeine w/w/o tylenol). No NSAID and ASA; immobilize joint, ice packs. ii)Clot factor replacement: F VIII concentrate, give for acute episode or pre-sx/dental. iii)DDAVP: pts w/mild dz->can incr F VIII 4x
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What is deficient in hemophilia B? What is tx and how diff from A?
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i)F 9 ii)F 9 replacement; no DDAVP used.
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What is is pathogenesis of DIC?
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Abnl activation of coag cascade by release of TF (7) or endothel cell injury->forms microthrombi thruout microcirculation->consume plts, fibrin, coag factors. Then get fibrinolytic mech's activated (via 12)->hemorrhage. Get thrombosis and bleeding simultaneously.
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What are clinical features in DIC? 2
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i)bleeding tendency (acute cases): a)SF hemorrhage (ecchymoses, petechiae, purpura). b)bleeding from GIT, Urinary T, gingival or oral mucosa. c)oozing from sites of procedures, incision ii)thrombosis: (chronic cases). End organ infarct, esp CNS and kidney. Can get shock from blood loss
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What are causes of DIC? x5
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i)Infection: G-- sepsis usually ii)obstetric complications (placenta and uterus w/incr TF) iii)major tissue injury: trauma, sx, burn iv)malignancy: APL, lung (and ARDS), pancreas,iv)rattlesnake venom. v)shock, circulatory collapse
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How to diagnose DIC? (what is incr x6? vs what is decr?). What about peripheral smear?
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i)BT, TT (low fibrinogen levels), PT/PTT, fibrin split products, D-dimer. ii)fibrinogen level, plt count. iii)shistocytes
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How to treat DIC? What are supportive measures and when indicated? x5
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i)manage condition that precipitated DIC ii)indicated if severe hemorrhage present. a)FFP replaces all clots b)plt xfusions c)cryoprecipitate replaces clot factors and fibrinogen d)low dose heparin if thrombosis dominates e)O2 and IV, maintain BP/renal perfusion
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What is most common cause of death in DIC? what other complications occur? 5
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i)intracranial hemorrhage ii)thromboembolism: stroke, PE, bowel infarct, ARF, arterial occlusion.
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If coagulopathy--Vit K and liver dz in ddx. How can you distinguish?
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i)Vit K: PT prolonged, but PTT, TT, plt count, fibrinogen levels normal. ii)Liver dz: PT/PTT elevated, but TT, fibrinogen, plts normal.
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What are sources of Vit K? x2 What are 3 causes of Vit K def?
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i)diet (leafy greens) ii)intestinal flora synthesis iii)a)Broad spectrum Abx w/NPO pt. b)Pts on TPN (unless vit K added) c)malabsorption of fat soluble vits (Small bowel dz/IBD; obstructive jaundice)
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What is course of PT and PTT in vit k def?
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PT initially prolonged (F 7 w/shortest 1/2 life); PTT prolongation follows (as other factors decr)
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Which factors are made in liver and which aren't? what is correlation w/liver dz and coagulopathy for px? What are 3 reasons for coagulopathy to develop in liver failure?
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i)all except vWF ii)worse prognosis b/c liver dz must be very severe for coagulopathy to develop. iii)a)cholestatis leads to decr Vit K absorption->vit K def. b)decr synth of factors c)hypersplenism 2/2 to portal htn: get thrombocytopenia.
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What are 2 clinical features of coagulopathy 2/2 to liver dz?
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i)abnl bleeding, esp GI: varices 2/2 to portal htn. ii)prolonged PT and PTT (esp PT)
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What is TX of coagulopathy of liver dz? x4
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i)FFP: all clot factors; used if PT or PTT prolonged or if bleed is present ii)Vit K if 2/2 to cholestasis iii)plt xfusion: if thrombocytopenic iv)cryoprecipitate if deficiency of fibrinogen
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What are the 7 inherited hypercoag states? (and thus get thrombosis)
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i)AT3 def (incr thrombosis) ii)APLS: acquired; arterial/venous thrombosis iii)Protein C def: inhibits F V and VIII->unreg fibrin synth. iv)Protein S def: cofactor of Protein C v)F. V leiden: Protein C can't inactivate F V->unreg prothrombin activation vi)prothrombin gene mutation vii)hyperhomocysteine
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How to DX inherited hypercoag states?
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functional assays for all of these
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how to treat hypercoag state? x2
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i)same as DVT/PE: heparin, tPA, IVC filter. ii)permanent anticoag w/warfarin if >2 thromboembolic events
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What are some 2/2 hypercoag states or risk factors? x5
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i)myeloproliferative d/o ii)trauma iii)nephrotic syndrome iv)HIT or DIC v)PNH vi)heart failure: get stasis of blood
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How does heparin work? What is 1/2 life of normal vs LMWH?
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i)potentiates ATIII->inhibits factor 2 and 10. ii)1 hour vs 3 hrs->24 hrs
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What are indictations for heparin use (and also warfarin)? 4
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i)venous thromboembolism: DVT, PE ii)acute coronary syndromes: USA, MI iii)Afib in acute setting iv)after vascular bypass grafting
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how long does therapeutic PTT take in heparin? Is PTT monitoring necessary w/SC dosing? How is LMWH dosed therapeutically vs prophylactically?
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i)60-90 s ii)no iii)based on weight; ppx=depends on product
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what are 5 adverse effects and which 2 are less so w/LMWH?
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i)bleeding ii)HIT (lower w/LMWH) iii)osteoporosis (less w/LMWH) iv)alopecia v)rebound hypercoag after removal b/c depression of AT3
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What are 4 contraindications to heparin?
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i)Bleed: actively, GI, intracranial ii)hemophilia, thrombocytopenia iii)severe HTN iv)recent sx on eyes, spine, brain
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What should you give if severe bleeding in warfarin or heparin?
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FFP
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What is diff in MOA of LMWH vs regular heparin? Why no monitoring?
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i)inhibits X as much as regular, but less effect on 2 and plt aggregation. ii)don't affect either
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Why is LMWH good in oupt?
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easier to use b/c no monitoring: can continue LMWH until level of long term anticoag is therapeutic
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What clotting factors are affected by warfarin besides 2,7,9,10
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Protein C and S
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how long do you continue heparin after starting warfarin?
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At least 4 days s/p starting warfarin, w/INR 2-3
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What is a rare but serious complication of warfarin? why does it happen? Who should warfarin not be given to?
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i)skin necrosis: rapid decrease in protein C ii)drunks; peeps w/ frequent falls: intracranial bleed is catastrophic
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How long does it take for Vit K to reverse warfarin?
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4-10 hours
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What are the diagnostic criteria for P vera: 3 major and 4 minor? (either 3 major or 2 major, 2 minor)
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i)Major: a)incr RBC mass; b)O2 sat>92% c)splenomegaly ii)minor: a)leukoctye alk phos >100 (no fever/infect) b)thrombocytosis (>400) c)leukocytosis d)High Vit B12
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What are 2 main symptoms of P vera? What are other features x4?
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i)Thrombotic phenomena: DVT, CVA, MI ii)bleeding: GI/GU bleed; ecchymoses; epitaxis iii)a)HTN b)hyperviscosity: pruritus, dizzy, h/a, weak c)splenomegaly, hepatomegaly
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What accounts for most of sxs of P vera? x2
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i)hyperviscosity ii)elevated total blood volume
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How to DX P vera? x6
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i)r/o other causes of 2ndary polycythemia (CO poison, hypoxemia) ii)elevated RBC count, Hb; possible thrombocytosis and leukocytosis iii)decr EPO iv)elevated B12 v)hyperuricemia. vi)DX by BM Bx
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How to treat P vera? x2
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i)repeated phlebotomy ii)myelosuppress w/hydroxyurea or IFN-a
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What are myelodysplastic syndromes? What are they characterized by? x2
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i)acquired clonal blood d/o's. ii)a)ineffective hematopoiesis w/apoptosis of myeloid precursors
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What are causes of MDS? x3 What is px and what is end result usually?
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i)radiation ii)immunosuppressive agents iii)toxins iv)poor->acute leukemia
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How does MDS present? What disease does it mimic?
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i)manifestations of anemia, thrombocytopenia, or neutropenia ii)asympto in early stages; pancytopenia may be incidental on routine blood test
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What does MDS BM Bx usually show? What does CBC and blood smear show? Cytogenetics?
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i)dysplastic cells w/blasts or ringed sideroblasts ii)normal or mildly elevated MCV; low retic count. iii)H-J bodies; basophilic stippling; nucleated RBCs; large, agranular plts. iv)chromo abnormalities or mutated oncogenes
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How to treat MDS? x3
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i)supportive: a)RBC and plt xfusions b)EPO c)GCSF for neutropenic pts d)Vit supplements: B6, 12, folate b/c large turnover ii)pharm: a)immunosuppressives b)chemo c)androgenic steroids iii)BM xplant is only cure
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What is defn of ET? what must be r/o? How does it manifest? x2
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i)plts >600k ii)reactive thrombocytosis (infection, inflamm, bleed) and other myleoprolif d/o's. iii)thrombosis (CVA) or bleeding (defective plt fcn)
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What are 3 findings in ET?
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i)splenomegaly ii)elevated BT iii)erythromelalgia: burning pain and erythema of extremities b/c of microvascular occlusion
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What does smear show for ET? BM BX? treatment for ET?
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i)hypogranular, abnormally shaped plts ii)incr megakaryocytes iii)antiplts like anagrelide and low dose ASA
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What is agnogenic myeloid metaplasia w/myelofibrosis? What are 2 manifestations?
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i)fibrosis of BM leading to pancytopenia and extramedullary hematopoiesis ii)a)massive splenomegaly b)2ndary to pancytopenia: fatigue, bleed, infection
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What is hallmark feature of AMM w/myelofibrosis? What does BM aspirate show? What is px? What is treatment? x3
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i)teardrop cells on peripheral smear; sometimes abnl, large plts and immature myeloid cells ii)BM aspirate=marrow fibrosis iii)PX is poor: pts progress to AML or die of bleed or infection iv)tx: xfusions, EPO, splenectomy
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What is multiple myeloma? Who is it most common in? What heme problems are present in advanced disease? x3
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i)plasma cell malignancy making monoclonal Ig: excess IgG or IgA ii)African Am iii)Plasmas replace BM: anemia, leukopenia, and thrombocytopenia may be present
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What 4 things suggest MM?
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i)Low Hb ii)high Ca iii)high serum protein iv)poor renal function
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What are the 5 clinical manifestations for MM?
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i)skeletal manifestations: bone pain b/c of osteolytic lesions, fractures, vertebral collapse--esp in low back or chest (ribs) and jaw (mandible) ii)normocytic normochromic anemia: BM infiltration and RF. iii)RF: tubular casts of BJ protein--Ig precipitation in renal tubules; Hyper Ca causes renal decomp iv)recurrent infections: most common cause of death (UTI or lungs) v)amyloidosis
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what are DX criteria for MM? 1+ 1 of 3
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i)10% abnl plasmas in BM +1 of following: a)M protein in serum (SPEP)b)M protein in urine (UPEP)c)lytic bone lesions: skull and axial skeleton
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What other lab findings in DX of MM? x6
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i)hyperCa ii)incr total protein in serum b/c of paraproteins in blood (hyperglobulinemia) iii)peripheral smear: rouleaux formation b/c of hyperglobulinemia causing RBCs to stick together iv)elevated ESR v)lots of BJ protein in urine vi)leukopenia, thrombocytopenia, anemia
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Why do pts get osteolytic lesions in MM?
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release of osteoclast activating factor by neoplastic plasma cells
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What are indications for tx in MM? What is given?
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i)hyperCa, bone pain, spinal cord compression. Otherwise treat sxs. ii)systemic chemotherapy is initial (alkylating agents); 2nd is radiation if no response to chemo; iii)xplant: autologous peripheral blood stem cell xplant, not BM xplant
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What is waldenstroms macroglobulinemia? How to DX?x2
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i)malig prolif of plasmacytoid lymphocytes: they make IgM paraprotein, which causes hyperviscosity of blood ii)DX: IgM>5g/dL; no bone lesions! Can have BJ protein
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What are clinical features of waldon macroglobulin? x5 What is treatment? x2
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i)a)lymphadenopathy b)splenomegaly c)anemia d)abnl bleeding e)hyperviscosity syndrome (elevated IgM) ii)no cure; give chemo and plasmapheresis for hyperviscosity syndromes
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What can hyperviscosity syndrome lead to in waldenstroms?
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retinal dilation and thus hemorrhage
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What is MGUS dx'd by? What is tx? What is course?
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i)IgG spike <3.5 g; less than 10% plasmas in BM; BJ proteinuria <1g/24 hrs. ii)no tx, just observe iii)less than 20% go to MM
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What is age distribution for Hodgkin's disease? x2
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Bimodal: 15-30; >50
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What are the 4 types of lymph node histology for Hodgkins?
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i)lymphocyte predominance: many B cells; few reed sternberg ii)nodular sclerosis: women; bands of collagen envelope RS cells. iii)mixed cellularity: Lots of RS cells iv)lymphocyte predominant: no RS; worst prognosis
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What is staging based on for HD? x3 What are the diff stages? (applies to HD and NHL). What does A and B suffixes mean?
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i)single LN ii)2 LN on same side of diaphragm iii)Both sides of diaphragm iv)disseminated dz to extralymphatic sites. v)A: no sxs B: fever, wt loss, night sweats (worsens px)
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What is most common sx of HD? Which nodes are most common x3? How does it spread? What are other sxs besides B sxs? x2
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i)painless lymphadenopathy ii)cervical, supraclaviclar, axillary, mediatinal LNs. iii)spreads from one LN to adjacent ones. iv)pruritus and cough (mediastinal LN involvement)
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How to DX HD? x5
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i)LN BX: need RS cells (B cells) ii)Inflamm cell infiltrates (diff from NHL): reactive to RS cells iii)CXR, CT, (chest/ab) for LN involvement iv)BM BX v)lab findings: leukocytosis, eosinophilia, incr ESR
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How to treat HD? x2
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Stages 1,2,3a: radiotherapy along or + chemo. Stages IIIB and IV need chemo
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What are key epidemiological ass'ns w/NHL? x3
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i)Burkitt's lymphoma in regions of africa ii)pts w/hiv and hiv ass'd lymphomas iii)adult T cell lymphoma in japan and caribbean
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Which lymphoma type has worse presentation usually Which is more common?
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i)NHL w/more advanced disease ii)NHL 2x as common
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What are 5 risk factors for NHL?
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i)immunosuppression ii)HIV/AIDS iii)Hpylori gastritis hx (primary ass'd gastric lymphoma) iv)autoimmune dz: hashi's or sjogren's (MALT). v)h/o certain viral infections (EBV, HTLV-1)
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What are the clinical features of NHL? x5
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i)lymphadenopathy: same nodes as HD, painless firm and mobile ii)HSM, ab pain, or fullness iii)BM involvement sxs (anemic, thrombo, infections) iv)SVC, resp involvement, bone pain v)B sxs less common
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What is definitive dx of NHL? What is indication to bx? Other tests for dx? x4
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i)LN bx ii)>1cm LN for more than 4 weeks not attributable to infection iii)a)CXR: mediastinal or hilar lymphadenopathy b)CT scan (chest, ab, pelvis): extent of dz spread and pts response c)serum LDH and B2microglobulin are indirect indicators of dz burden d)LFT or bili incr=liver involvement
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What does tx of NHL depend on? What are the 3 options to use? How to treat aggressive NHLs? WHat is last resort?
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i)subtype and stage of NHL ii)a)observation b)chemo c)rad therapy iii)CHOP(cyclophos, doxorubicin, vincristine, prednisone) therapy and radiation iv)very hi dose chemo + BM xplant
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What are the low grade or indolent NHLs? x2
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i)small lymphocytic lymphoma ii)follicular: predom small, cleaved cell lymphoma
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What are the key features of SLL? Of follicular?
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i)a)closely related to CLL; in elderly pts b)indolent course ii)a)most common NHL b)can xform into diffuse large cell and ass'd w/t(14,18) c)painless, peripheral lymphadenopathy
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What is the intermediate grade NHL? What is its key feature? How to cure?
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i)DLBL ii)locally invasive; presents as large extranodal mass. iii)CHOP therapy
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What are the 2 hi grade NHLs?
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a)Lymphoblastic lymphoma b)Burkitt's
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What are key features of lymphoblastic lymphoma? What is its px?
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i)T cell lymphoma mostly in kids ii)can progress to T-ALL iii)aggressive w/rapid dissemination but may respond to combo chemo
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What is key feature of Burkitts? x3 What are the 2 kinds and what are the diff? What is px?
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i)a)B cell lymphoma; in kids b)African=EBV infection c)ass'd w/sp xlocation t(8;14). ii)a)African: facial bone and jaw; b)american=abdominal organs iii)poor px so treat aggressive chemo
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What is mycosis fungoides and sezary?
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i)a)Mycosis: T cell lymphoma of skin presenting w/eczematoid skin lesions that become generalized erythroderma. b)Cribriform shape of lymphocytes that go to LN, blood, other organs. ii)sezary=involves skin + blood. iii)curable if limited to skin
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What are the HIV ass'd NHLs?
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DLBL and Burkitt's
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What is diff b/w acute leukemias vs chronic leukemias in type of cells and rapidity of progression?
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i)Acute: rapid progression and affect immature cells (immature cells prolif b/4 maturation) ii)chronic: progress slowly and affect mature cells.
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What are risk factors of AML x4? Which acute leukemia has better response to therapy? Who does AML occur in and who does ALL occur in?
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i)a)radiation b)myeloprolif syndromes c)down's d)chemo (alkylating agents). ii)ALL has better response to chemo iii)AML in adults, ALL in kids
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What are poor px factors for ALL? x3
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i)age <2; >9 ii)wbc >10^5 iii)CNS involvement
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What causes incr risk for CNS involvement? x3
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i)B cell phenotype ii)incr LDH iii)rapid leukemic cell proliferation
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What do you evaluate patients for w/leukemias? x5
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i)evidence of infection ii)evidence of bleeding or easy bruising iii)lymphadenopathy/HSM iv)anemia signs v)fatigue, wt loss
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what is presentation of acute leukemias?
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i)a)all the other shit: anemia, infections, bleeding, HSM, lymphad. b)bone and joint pain (invades periosteum) c)CNS involvement: diffuse or focal neuro dysfcn
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What is the dz that has testi involvement? Ant mediastinal mass? Skin nodules?
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i)ALL ii)T cell ALL iii)AML
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Which dz is auer rods in?
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AML but not ALL
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What are the lab findings in acute leukemias? x5 What is needed for DX?
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i)WBC varies from low to high. lots of blasts in peripheral blood ii)anemia iii)thrombocytopenia: monitor plts regularly iv)granulocytopenia v)electrolyte disturbance: hyperuricemia, hyperkalemia, hyperphosphatemia. B)Need BM bx for dx
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What is tumor lysis syndrome? What does it cause? x3 How to treat?
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i)complication of chemo in acute leukemia and hi grade NHL due to rapid cell death w/release of intracell contents ii)a)hyperkalemia b)hyperphosphatemia c)hyperuricemia iii)medical emergency
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How to treat emergencies for acute leukemia? x3
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i)blood cx ii)Abx for infection iii)blood xfusion for anemia and plt xfusion for bleed
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How to treat acute leukemia? How is AML treated diff from ALL?
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i)Treat w/aggressive chemotherapy in high dose for several weeks to get remission (absent leukemic cells in BM). Then maintenance therapy. b)BM xplant is other option ii)ALL: most get remission; AML: BM xplant is best option b/c refractory to chemo
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What kind of cells cells are created in CLL?
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monoclonal prolif of lymphocytes that are morph mature but functionally defective. (don't differentiate to Ig making plasmas)
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How is progrnosis of CLL? What do most die of?
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i)least aggressive leukemia; CLL survive longer than CML ii)infections
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What are clinical features of CLL? x5
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i)usually asx; CLL discovered on routine CBC (lymphocytosis). ii)generalized painless lymphadenopathy; splenomegaly iii)frequent respiratory or skin infections due to immune deficiency. iv)advanced: fatigue, wt loss, pallor, rash, easy bruising
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How to DX cLL? x2 What is seen?
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i)lab findings and BM bx. ii)Labs: a)WBC 50K-200K b)anemia, thrombocytopenia, neutropenia c)peripheral blood smear: absolute lymphocytosis->all WBC are mature, small lymphocytes; see smudge cells: leukemic cells that are beaten up in blood. iii)BM bx: infiltrating leukemic cells in BM.
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What is treatment for CLL? x2
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i)chemo has little effect ut given for infection ii)fludarabine and chlorambucil
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What are the cells of myeloid line?
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erythrocytes, granulocytes, platelets
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What is diff b/w benign leukomoid reaction from CML leukomoid rxn?
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i)usually no splenomegaly ii)incr leukocyte alkaline phosphatase iii)h/o precipitating event (ie: infection)
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What is course of CML? What xlocation is it ass'd w/? What do pts w/o philly chromosome have for prognosis?
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i)indolent until xforms into acute leukemia; end pt is an acute phase (blast crisis) where there is accelerated phase of blast and promyelocyte production. ii)t(9;22) (philly) iii)shorter survival times and worse response to treatment.
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What are the clinical features of CML?x4
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i)splenomeg, hepatomeg, lymphad ii)recurrent infections, easy bruise/bleed; sxs of anemia. iii)constitutional sxs iv)usually asx at dx!
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What is seen on labs for CML? x7
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i)marked leukocytosis 50-200; left shift toward granulocytes ii)small number of blasts and promyelocytes iii)eosinophilia iv)peripheral smear: leukemic cells in peripheral blood: myelocytes, metamyelocytes, bands, segmented forms. v)decr LAP activity vi)thrombocytosis. vii)BM BX: leukemic cells
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How to treat CML? what drug for chronic phase? What other procedure during chronic phase?
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i)chemo to control sx b/4 acute phase develops, but progressive dz w/o cure. High dose chemo to return dz to chronic phase when have blast crisis. ii)alkylating agent or antimetabolite iii)BM or SC xplant during chronic phase b/c most go on to die during acute phase
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