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71 Cards in this Set

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1. key words for anemia (4)


2. key words for severe anemia


3. dz similar to anemia



1. fatigue, loss of energy, malaise, pica


2. short of breath, lightheadedness, confusion


3. hypoxia, CO poisoning, methemoglobiemia, ischemic heart dz

Anemia


- physical exam

- general: pallor


- HEENT: pale conjunctiva, scleral icterus (hemolysis)


- Chest: for sob


- Cardio: flow murmur (systolic)


- extremitites

Anemia


1. dx testing

- initial: CBC w/ peripheral smear


- others: retic count, LDH, haptoglobin, bilirubin, iron studies, B12/folate, TSH/T4


- UA w/ microscopic analysis

Categorizing anemia


1. use of MCV


2. use of MCHC

1. MCV: microcytic, macrocytic, normocytic


2. problem with Hb syn: hypo-, hyper-, normo-chromic

Microcytic anemia: list hx clues for


1. Fe-def anemia


2. chronic dz


3. thalassemia


4. sideroblastic anemia

1. blood loss, platelet elevated


2. Rheumatoid arthritis, ESRD


3. target cells, very small MCV w/ min sx


4. alcoholic, isoniazid, lead exposure

Iron deficiency


1. dx testing & values


2. most accurate test


3. intial tx (caveat)

1. iron studies: low ferritin, high TIBC, low Fe and sat, elevated RDW [cells smaller and smaller]


2. bone marrow bx


3. ferrous sulfate oral


- can make stool black = get guaiac testing (only Fe in Hb make this positive)

Anemia of chronic dz


1. dx testing


2. mos accurate


3. initial tx

1. iron studies: high ferrtin, low TIBC, low Fe, nl or low Fe sat


2. none


3. correct undlying condition

Thalassemia


1. dx testing


2. most accurate test


3. initial tx

1. normal iron studies


2. Hb electrophoresis


- alpha: normal (DNA sequencing most accurate)


- beta: elevated HgA2, HgF


3. no tx available

Sideroblastic anemia


1. dx testing


2. most accurate


3. initial tx


4. pathology

1. iron studies: high Fe


2. prussian blue stain


3. major: remove toxin


- minor: pyridoxine replacement


4. iron build up in mitochondria

1. when to use capsule endoscopy


2. which is the only microcytic anemia w/ high retic count

1. upper and lower endoscopy are normal


2. HgH = 2 gene deleted alpha thalaseemia

Macrocytic anemia


1. sx for each type


2. dx test

1. B12 def: peripheral neuropathy + smooth tongue (glossitis) + diarrhea


- folate: no neuro


2. initial CBC w/ peripheral smear. if >5% with >5 lobes = megaloblastic (hyperseg) anemia + macrocytosis.

Macrocytic anemia


1. lab values (caveat)


2. best est to confirm B12 def

1. low retic ct, incr iBili, incr LDH


- get B12 or folate level


- 30% B12 def have nl B12 b/c transcobalamin is acute phase reactant => get methylmalonic acid (elevated).


- homocyteine elevated for both


2. antiparietal cell and anti-intrinsic factor Ig = pernicious anemia


- metformin decr B12 abs

Hemolytic anemia


1. sx


2. dx testing for any hemolysis


3. intravascular hemolysis sx

1. sudden onset weakness adn fatigue


2. elevated indirect bili


- elevated retic count


- elevated LDH (w/ any tissue breakdown)


- decr haptoglobin (transport of ibili)


- spherocytes on smear (also in AI hemolysis)


3. hemoglobinuria, hemosiderinuria, peripheral smear (schistocytes, helmet cells)



W/ tx what comes back first in:


1. B12 def (caveat)


2. folate


3. hemolytic anemia

1. retic improves => neuro sign if short period


- watch for low K


2. retic


3. retic, LDH

Sickle cell anemia


1. classic compliant


2. PE fx

1. pain in chest, back adn thighs


2. HEENT: retinal infaction


- CV: flow murmur from anemia


- Chest: rales or consolidation from infxn


- Abd: splenomegaly in kid; absence in adult


- extrem: skin ulcer, aspetic necrosis of hip (MRI)


- neuro: stroke

Sickle cell anemia


1. inital tx


2. if fever present

1. O2, hydration w/ nl saline, pain meds


2. ceftraixone, levoflaxacin, moxifloxacin


- in CCS, ans bld cx, UA, retic count, CBC, CXY, but do not wait for results before antibx

Sickle cell anemia


- when to give exchange transfusion


- purpose of transfusion (4)

1. transfuse = decr Hb S to 30-40%


2. give when eye: retinal infarction


- lung: pulm infarction => pleuritic pain


- penis: priapsim


- brain: stroke

Sudden drop in hematocrit in sickle cell pts


1. diagnosis


2. most accurate test


3. tx

1. folate def or parvovirus B19


2. PCR for parvoB19 (r/o folate if supplemented)


3. transfuse + IVIG

long term mgmt of sickle cell anemia

- pneumococcal vaccine


- folate replacement daily


- hydroxyurea if crises >4 /year (incr Hb F)

Sickle cell disease


1. sx


2. vs sickle cell trait

1. milder version w/ freq visual disturbance


- renal: hematuria, isosthenuria (can't [] or dilute urine), UTI


2. trait only has renal sx (hematuria, isosthenuria). splenic vein thrombosis when scuba

Autoimmune hemolysis


1. hx clues


2. dx testing

1. hx of SLE or RA, CLL


- meds: penicillin, methyldopa, quinine, sulfa


2. smear = spherocytosis (Ig remove membrane)


- accuate = pos Coombs (warm agglutinin, IgG)


- LDH, iBili, retic ct elevated; hepatoglobin decr

Autoimmune hemolysis


1. tx (4)

- initial: prednisone


- if not work, try IVIG (response predicts response to splenectomy)


- rituximab: agaisnt CD20 recepotr on Ig-producing lymphocytes (IgM, IgG)


Note: only warm IgG respond to steriods, splenectomy

Cold-induced hemolysis


1. causes (3)


2. dx testing


3. tx

1. syphilis with IVIG, hx of mycoplasma or Epstein-Barr virus


2. neg Coombs, complement positive


3. rituximab. (no steriods, splenectomy, IVIG)

Glucose-6-phosphate dehydrogenase def


1. genetics


2. sx


3. causes

1. X-linked, in males


2. sudden onset of hemolysis (severe)


3. infxn, oxidizing drugs (sulfa, primaquine, dapsone), fava bean

G6PD Deficiency


1. dx testing


2. tx

1. initial smear: heinz body (oxidized Hb embeded in RBC), bite cells


- accurate: G6PD level 2mths after episode


2. test G6PD before giving drugs

Pyruvate kinase deficiency


1. sx





- similar to G6PD Def = sudden hemolysis. but precipitates not clear

Hereditary spherocytosis


1. sx


2. pathology


2. dx testing


3. tx

1. recurrent episodes of heolysis, splenomegaly, bilirubin gallstones


2. loss of ankyrin and spectrin that make bicave shape


3. incr MCHC (hb []), osmotic fragility test


4. splenectomy

Hemolytic uremic syn


1. common cause


2. sx



1. E.coli 157:H7 hx


2. traid = ART [Autoimmune hemolysis, Renal failure (incr BUN/Cr), Thromboctopenia]

Thrombotic thrombocytopenic purpura (ttp)


1. cause


2. sx

1. ticlopidine


2. pentad (FAT RN): Fever, AI hemolysis, thrombocytopenia, renal failure, neuro abnl

HUS/TTP


1. lab value


2. tx

1. get ADAMTS-13 level (down in TTP)


2. resolve itself. plasmapheresis. no steriods, antibx for E.coli or platelet transfusion


- ADAMTS13 break down vWF to release platelets from each other. when lacking, platelets form ong strands = obstruct RBCs


- giving platelets worsen = lengthen strands

Paroxysma nocturna hemoglobinuria


1. sx


2. complication (3)


3. dx testing


4. tx

1. dark urine in morning, pancytopenia


2. death by large vessel venous thrombosis (portal vein), aplastic anemia, AML


3. CD55/59 Ig (decay accelerating factor)


4. glucocorticoids (prednisone) initial


- severe: eculizumab

differentiation of HELLP from DIC

HELLP has nl coagulation studies vs DIC

Methemoglobinemia


1. cause


2. pathology


3. sx


4. tx

1. nitroglycerin, amyl nitrate, nitroprusside, dpasone, anesthetic ending in -caine


2. blood locke din oxidized state that cannot pick up O2


3. brown blood, SOB w/ clear ung son exam and nl CXR


4. methylene blue

Transfusion rxn


1. transfusion-related acute lung injury (TRALI)


2. IgA deficiency


3. ABO incompatibility


4. minor blod group incompatibility (Kel, Duffy, ewis, Kdd)


5. febrile nonhemolytic rxn

1. SOB, infitrates on CXR. resolves spont


2. hypotensive, SOB, tachy. LDH/bili nl


3. hypotensive, tachy, dark urine, chest pain


- LDH/Bili elevated, heptogobin low


4. days after transfusion. jaundiced


5. febrile w/ no hemolysis

Acute leukemia


1. sx


2. dx testing


3. tx

1. pancytopenia: fatigue, beeding, infections


2. periphera smear showing blasts


- auer rods = AML


- cytogenetic abnl tell replase. if high risk, get bone marrow transplantation after chemo

Acute leukemia


1. AML tx


2. ALL tx



1. AML: idarubicin, daunorubicin or cytosin arabinoside = initial


- all trans retinoic acid (ATRA) to M3 (acute promyeocytic) lukemia [assoc w/ DIC]


2. ALL: add intrathecal methotrexate

Leukostatsis


1. sx


2. assoc dz


3. tx

1. sludging of bld vessels of brain, eyes, lungs


2. acute leukemia. rare in CLL as lymphocytes smaller


3. leukapheresis. hydroxyurea to lower WBC

myelodysplasia


1. definition


2. sx


3. dx testing (labs)

1, mild, slowly progressive preleukemia syn


2. elderly w/ pancytopenia


3. smear: macroovalocytes, Pelger-Huet cell (2-lobe neutrophil)


- elevated MCV, nl B12, low retic, small # blasts



Myelodysplasia


1. causes of death


2. tx

1. infection, bleeding


2. transfusion


- azacitadine: incr survival


- lenalidomide [decr # transf] for 5qminus syn (deletion of long arm of chormosme 5)

Myeoproliferative d/o


- name them

CML


CLL


hairy cell leukemia


myelofibrosis


polycythema vera


essential thrombocytopenia



CML


1. sx


2. lab


3. dx testing



1. early satiety = spleenomegaly compress stomach


2. elevated WBC w/ prodominant neutrophil


3. elevate neutrophil w/ low LAP score (high = infection)


- PCR for philadelphia chromosome


- FISH for BCR/ABL

CML


1. complication


2. tx

1. untreated = highest risk of acute leukemia


2. imatinib first


- bone marrow transplantation only curative method

CLL


1. classic ppx


2. pathology


2. dx testing



1. >50yo asx and elevated WBC on routine exam


2. susceptible to infxn b/c lymphocytes produce abnl or insuf Ig


3. peripheral blood smear: smudge cell (ruptured nucei)


- elevated WBC w/ nl appearing lymphocytes



CLL stages

0: elevated WBC aone


1: enlarged LN


2: spleen enlarged


3. anemia


4: low platelets

CLL


- tx

- do no tx stage 0 and 1


- advanced stage: fludarabine + ritaximab + cyclophosphamide


- if fludarabine, alemtuzumab

Hairy cell leukemia


1. sx


2. dx testing


3. tx

1. middle age (50s) w/ massive splenomegaly, pancytopenia


2. smear showing hariy cells and immunophenotyping (flow cytometry)


3. cladribine

myelofibrosis


1. sx


2. dx testing (3)


3. tx (3)

1. same as hairy cell = pancytopneia + splenomegaly


2. smear: teardrop ces


- marror fibrosis, JAK2 mutation


3. bone marrow transplant currative


- next best: lenalidomide, thalidomide


- ruxolitinib inhbits JAK2

Polycythemia vera


1. sx


2. dx testing

1. headache, blurred vision, dizziness


- pruritus esp after hot shower (histamine from basophil)


- splenomegaly


2. CBC: high hematocrit => order arteria bood gas to exclude hypoxia


- erythropoietin level (low) => hem consult


- JAK2 mutation, high B12 & LAP


Note: in CCS, get CBC, LAP, B12



Polycythemia vera


1. tx

- phlebotomy initial + aspirin


- hydroxyurea: ower cel count


- anagrelide when thrombocythemia

Essential thrombocythemia


1. sx


2. cause of death


3. dx testing


4. tx

1. headache, visua disturbance, pain in hands (erythromelagia)


2. bleeding, thrombosis more common


3. markedy eevated platelet count


4. hydroxyuea + aspirin


- anagrelide athough no as well

Multiple myeoma


1. sx


2. renal pathology (4)


3. most specific dx testing

1. CRAB: calcemia, renal, normal anemia, bone pain cause dby racutre under nl use


- infxn


2. calcemia => nephrocacinosis


- bence-Jones protein cogs gomeruli


- amyoid


- hyperurecemia toxic to kidney tubues


3. bone marrow bx: >10 plasma cels

Multiple myleoma


1. dx testing (7)

- skeleta survey: punched out osteolytic lesion


- serum protein electrophoresis (SPEP): IgG incr


- UPEP: bence-jones


- periphera smear: roueaux. incr MPV


- elevated Ca2_


- beta 2 microglobulin (prognostic factor)


- BUN/Cr

Multiple myeloma


1. tx

- mephalan and steroids


- add bortezomib: reverses renal dysfxn


- advance stage: autologous stem cell bone marrow transplant (up to 70yo; aogenic to 50yo)


- hypercalcemia: hydration, diuresis


- bone fractures: bisphosphonates


- renal failure: hydration


- anemia: erythropoietin


- prophy = vaccines

Monoclonal gammopathy of unkn sig (MGUS)


1. sx


2. dx testing

1. asx with high protein


2. hgih IgG on SPEP

Waldenstrom's macrogobulinemia


1. pathoogy


2. sx


3. dx testing


4. tx

1. hyperviscosity from IgM overproduction


2. blurry vision, headache, enarged nodes, spleen


3. inital: serum viscosity + SPEP (high IgM)


4. pasmapheresis if sx


- add tx for CLL: rituximab, fludarabine, chlorambucil

Apastic anemia


1. sx


2. tx

1. pancytopenia


2. bone marrow trnasplant. if not possibe (>50yo), antithymocyte globulin + cyclosporine

Lymphoma


1. sx


2. spread of the types


3. dx testin

1. enlarged (cervical) lymph nodes


- B sx of fever, wt loss, night sweats [dz spread]


2. Hodgkin - centrifugally from center at neck


- NHL = widespread dz


3. initial is excisional lymph node bx. HD has reed-sternberg cells

Staging lymphoma


1. tests to order


2. the stages

1. CXR, CT w/ contrast of head/chest/abd, bone marrow bx


2. I - single LN group


- II - 2 LN groups on one side of diaphragm


- III - LN involvement on both sides


- IV - widespread dz


HD presents w/ stage I-II; NHL with III-IV

Lymphoma


1. tx (HD VS NHL, early vs late stage)

1. stage I/II: radiation + low dose chemo


- stage III/IV: chemo


2. HD: ABVD - adriamycin, bleomycin, vinblastin, dacarbazine


- HDL: CHOP - cyclophos, hydroxyadriamycin, oncovin (vinblastine), predisone


- for HDL test anti-CD20 antigen. if present add rituximab

von Willebrand's disease (VWD)


1. sx


2. dx testing


3. tx

1. skin/mucosal bleeding, epistaxis


- worse w/ aspirin


2. ristocetin cofactor assay: ristocetin = endothelial lining that platelets stick to


- vWF level, nl platelet count


3. desmopressin (DDAVP)


- next step, factor VIII replacement (has vWF & 8)

idiopathic thrombocytopenic purpura (ITP)


1. ppx


2. dx testing

1. platelet-type bleeding (VWD sx) w/ platelets <10-30k


2. tx first. then dx with smear = large platelets


- US: nl spleeen in ITP


- bone marrow: incr # of megakaryocytes


- IG to glycoprotein IIb/IIIa receptor

ITP tx


1. platelet >50k


2. platelt ct <50k w/ minor bleeding


3. ct <20k with serious bleeding


4. recurrent episode


5. no response to splenectomy

1. no tx


2. predinsone


3. IVIG (inhibit macrophages against platelets)


4. splenectomy


5. romiplostim, eltrombopag = thrombopoietin analgos

Uremia induced platelet dysfxn


1. ppx


2. labs


3. tx

1. renal failure px w/ platelet type bleeding


2. nl platelet ct, ristocetin test and VWF level


- uremia prevent platelet from degranulating


3. desmopressin, dialysis, estrogen

Factor VIII / IX def


1. ppx


2. dx testing


3. tx

1. hemarthrosis, hematoma in MALE child


2. mixing study initial => specific factor level


3. severe: factor VIII or IX replacement


- minor: DDAVP (only for factor VIII)

Factor XI def


1. ppx


2. dx testing


3. tx

1. rare bleedingw/ trauma or surgery


2. mixing study first => specific factor


3. FFP w/ bleedign episodes

Factor XII def


1. ppx


2. dx testing


3. tx

1. no bleeding


2. mixing study => specifi factor


3. no tx needed

Heparin-induced thrombocytoopenia (HIT)


1. definition


2. complication


3. dx testing


4. tx

1. at least 50% drop in platelet after heparin


2. venous thrombosis (less likely w/ low MWH)


3. platelet factor 4 Ig or heparin-induced, antiplatelt Ig


4. stop heparin and use direct thrombin inhib


- argatroban, lepirudin, fondaparinux

Lupus antiphospholid syn, anticardiolipin


1. ppx


2. dx testing


3. tx

1. elevated aPTT, nl PT,


- false pos VDRL, spon abortion, thrombosis


2. mixing study 1st. russel viper venom test for lupus anticoagulant


3. heparin followed by warfarin

Protein C def


1. ppx


2. dx testing


3. tx

1. skin necrosis post warfarin, v. thrombosis


2. protein C level


3. heparin f/u warfarin

Factor V leiden mutation


1. ppx


2. dx testing


3. tx


4. pathology

1. v. thrombosis


2. factor V mutation test


3. heparin f/u warfarin


Path: Protein C can't inhibit facotr 5

Antithrombin def


1. ppx


2. dx testing


3. tx

1. v. thrombosis, no change in aPTT w/ IV heparin


2. level of antithrombin III


3. large amount of heparin or direct thrombin inhibitor f/u warfarin