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125 Cards in this Set
- Front
- Back
- 3rd side (hint)
poikilocytosis
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varying shapes
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RBC membrane contains ________ antiport that allows RBC to transport CO2
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chloride-bicarb
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platelet contains
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dense granules (ADP, Ca) and alpha granules (vWF, fibrinogen)
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PMN
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multilobed, azurophilic granules with hydrolytic enzymes, lysozyme, myeloperoxidase, lactoferrin (binds iron during ifxn)
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monocyte
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large kidney shaped nucleus, extensive frosted glass cytoplasm
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marcophage activated by
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gamma interferon
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eosinophil
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bilobate, major basic protein, phagocytic for Ag-Ab complexes, produces histaminase and arylsulfatase to help limit mast cell degran
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neoplasms can elevate
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basophil
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allergy. bilobate. contain heparin (anticoag), histamine (vasodil), vasoactive amines, LTD4
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mast cell
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contains histamine, heparin, eosinophil chemotactic factors
in tissue |
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langerhans
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DC
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extrinsic coag factor
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VII
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antithrombin inhibis
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VIIa, IXa, Va, XIa, XIIa
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epoxide reductase
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activates VitK, warfarin inhibits
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tPA
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activates plasmin
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thrombomodulin
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activates Protein C
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protein S
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req by Protein C to cleave Va and VIIIa
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Factor V Leiden
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factor V resistant to APC inhibition
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after platelets bind vWF...
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they release ADP and Ca. ADP helps platelets adhere to endothelium and ADP binding to receptor induces GpIIb/IIIa expression
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fibrinogen binds GpIIb/IIIa and links platelets
pro-agg: TXA2 from platelets anti-agg: PGI2 and NO from endothel |
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ticlopidine and clopidogrel MoA
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inhibit ADP-induced expression of GpIIb/IIIa
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abciximab MOA
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inhibits GpIIb/IIIa directly
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acanthocyte, spur cell
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liver dz, abetalipoproteinemia
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basophilic stippling
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thalassemias, anemia of chronic dz, iron def, lead poison
BASte the ox TAIL |
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schistocyte
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DIC, TTP, HUS, traumatic hemolysis
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teardrop cell
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myelofibrosis
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heinz bodies
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alpha thal, G6PD
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alpha thal
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Asian and African
1-2 gene deletion, no anemia 3 is HbH dz (beta4) 4 is hydrops fetalis, death |
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beta thal
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minor is hetero, asym, incr HbA2 on electrophoresis
major is homo, req transfusion. no beta chain. marrow expansion shows crew cut on xray, chipmunk face |
point mutations in splice sites and promoter seq. Mediterranean. aniso, poikilo, micro, hypo, targets, schistos. both major and minor incr HbF, HbS/beta thal hetero is milder than SS.
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lead poison
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inhibits ferrochelatase and ALA dehydratase. lead lines on gingivae (burtons) and epiphyses of long bones on xray. Encephalopathy and Erythrocyte basophilic stippling (aggreg of ribosomes) abdominal colic and sideroblastic anemia. wrist and foot drop
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tx: dimercaprol and EDTA
succimer for kids |
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sideroblastic anemia
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defect in heme sunth. XL defect in ALA synthase. reversible if from alcohol or lead
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ringed sideroblasts with iron-laden mitochondria. incr iron, normal TIBC, incr ferritin.
tx: B6 |
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cause of B12, neuro sx
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fish tapeworm Diphyllobothrium latum
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periph neurop with sensorimotor dysfxn
posterior columns (vibration and proprioception) lateral corticospinal (spasticity) dementia |
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nonblastic macrocytic anemia
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liver dz, alcohol, reticulocytosis
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blastic macrocytic anemia (not B12 or folate)
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orotic aciduria
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intravascular hemolysis findings
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decr haptoglobin, incr LDH, Hb in urine
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extravasculat hemolysis findings
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incr LDH, incr UCB, jaundice
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ACD
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incr hepcidin
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aplastic anemia
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failure or destruction of myeloid stem cells
sx: fatigue, malaise, pallor, purpura, mucosal bleeding, petechiae, ifxn |
tx: immunosupp, allogeneic bone marrow transplant, RBC and platelet transfusion, GCSF, GMCSF
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hereditary spherocytosis
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no central pallor
positive osmotic fragility test |
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pyruvate kinase def
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AR. leads to decr ATP and rigid RBCs
hemolytic anemia in a newborn |
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HbC defect
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glutamic acid to lysine mutation at position 6
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paroxysmal nocturnal hemoglobinuria
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impaired synth of GPI anchor of decay accelerating factor in RBC mem
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labs: incr urine hemosiderin
complication: thrombosis |
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sickle cell
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glutamic acid becomes valine at position 6
low O2 or dehydration ppts sickling crew cut on xray |
complic: aplastic crisis d/t parvoB19
renal papillary necrosis d/t low O2 in papilla and microhematuria d/t medullary infarcts |
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AI hemolytic anemia
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warm agglutinin, IgG in SLE, CLL, alpha methyldopa
cold agglutinin, IgM, in CLL, mycoplasma, mono |
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direct coombs
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pt RBC, anti-IgG added
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indirect coombs
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pt serum, RBC added
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microangiopathic anemia
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schistocytes. DIC, TTP HUS, SLE, malig HTN
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macroangiopathic anemia
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prosthetic heart valves and aortic stenosis
schistocytes |
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lead poisoning accumulates
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protoporphyrin in blood
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acute intermittent porphyria
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porphobilinogen deaminase (aka uroporphyrinogen I synthase) defective
porphobilinogen, ALA, uroporphyrin acc in urine |
sx: painful abdomen, red wine colored urine, polyneurop, psych disturb, can be from drugs
tx: glucose and heme, which inhibit ALA synthas |
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porphyria cutanea tarda
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uroporphyrinogen decarboxylase defect
uroporphyrin acc in urine (tea color) |
blistering cutaneous photosens. most common
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substrates for ALA synthase, rate limiting and first step of heme synthesis
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glucine and succinyl CoA and B6
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PT tests
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I, II, V, VII, X (extrinsic)
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PTT tests
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all except VII and XIII (intrinsic)
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hemophilia A or B
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intrinsic path defect, incr PTT only
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platelet abnormalities
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microhemorrhage: mucus mem bleeding, epistaxis, petechiae, purpura, incr bleed time, possible decr platelet count
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bernard soulier
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decr platelet count
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glanzmann's thrombasthenia labs
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blood smear shows not platelet clumping
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ITP
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decr PC, incr BT
defect: anti-GpIIb/IIIa Abs lab: incr megakaryocytes |
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TTP
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dec PC, incr BT
decr platelet survival def of ADAMTS13 (vWF metalloprotease) leads to decr degradation of vWF multimers |
incr large vWF multimers leads to incr platelet aggreg and thrombosis
labs: schistocytes, incr LDH sx: neuro and renal sx, fever, thrombocytopenia, microangiopathic hemolytic anemia |
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von willebrands dz
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PTT may be incr. intrinsic defect. vWF carry/protects VIII. AD.
tx: DDAVP (desmopressin), which releases vWF stored in endothelium |
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DIC
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decr PC, incr BT/PT/PTT
schistos, incr D-dimers (fibrin split products), decr fibrinogen, decr V & VIII widespread activation of clotting leads to def in clotting factors, which creates bleeding |
Causes - STOP Making New Thrombi: Sepsis (GN), Trauma, OB probs, acute Pancreatitis, Malignancy, Nephrotic Syn, Transfusion
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factor V leiden
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most common cause of inherited hypercoag
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prothrombin gene mutation
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mutation in 3' UTR assoc with venous clots
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antithrombin def
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inherited, reduced incr in PTT after heparin
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protein C/S def
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decr inability to inactivate V and VIII. incr risk of thrombotic skin necrosis with hemorrhage after warfarin
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FFP for
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DIC, cirrhosis, warfarin OD
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cryoprecipitate
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contains fibrinogen, VIII, XIII
treats coag factor defs |
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leukemoid reaction
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incr wbc count with left shift (80% bands) and incr leuk alk phos usu d/t ifxn
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Hodgkins
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50% EBV
bimodal young/old good prog when incr Ls decr RS |
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Non Hodgkins
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assoc with HIV and IS
extranodal, noncontig fewer constitutional sx peak incidence 20-40yo |
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Reed-Sternberg
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CD30, CD15
variant include lacunar cells in nodular sclerosis variant |
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nodular sclerosing hodgkins
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65-75%, lots of Ls. most common. collagen banding, lacunar cells. women, young adults
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mixed cellularity hodgkins
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25%, lots of RS and Ls, intermed prog
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lymphocyte predominant hodgkins
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6%. less then 35yo men
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lymphocyte depleted
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rare. older men with disseminated dz. bad prog
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t(8;14)
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burkitts. c-myc moves next to HC Ig (14)
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diffuse large B cell lymphoma
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usually older adults, but 20% kids
most common adult NHL 20% T cell |
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t(11;14)
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mantle cell lymphoma. older men. transloc of cyclin D regulatory gene. CD5. poor prog.
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t(14;18)
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bcl-2. difficult to cure, indolent course. follicular lymphomas. adults.
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adult T cell lymphoma
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cutaneous lesions, japan, W Africa, Caribbean. aggressive
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mycosis fungoides/sezary syndrome
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cutaneous patches/nodules. indolent CD4+
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multiple myeloma
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CRAB: hyperCa, renal insuff, anemia, bone lytic lesions/pain
rouleaux |
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ALL
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TdT+, CALLA+, can spread to CNS and testes. t(12;21) is a better prog
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SLL/CLL
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>60, asym, smudge cells. warm agglutinins.
CLL has incr periph blood lymphocytosis than SLL |
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hairy cell leuk
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mature B cell tumor in elderly. TRAP
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AML
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60yo. Auer rods. myeloblasts circ. adults. t(15;17) is M3 AML which responde to all-trans retinoic acid (vitA), inducing diff of blasts. sx: DIC
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CML
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30-60yo. Philly chromo (t(9;22), bcr-abl. myeloid stem cell prolif. incr PMNs, metamyelocytes, basophils, Smeg, may --> blast criss
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very low leuk alk phos d/t immature granulocytes
imatinib |
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Auer bodies
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peroxidase positive inclusions in granulocytes and myeloblasts. tx can release and lead to DIC
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t(9;22)
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Phily. CML
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t(15;17)
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M3 AML
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t(11;22)
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ewings
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t(11;14)
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mantle cell lymphoma
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langerhans cell histiocytosis
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prolif of immature DCs. S-100 and CD1a. Birbeck granules (tennis rackets)
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polycythemia vera
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incr RBC, WBC, platelets. JAK2 mutation
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RCC, Wilms, cyst, HCC, hydronephrosis can cause ectopic erythropoietin
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JAK2 mutations (GF signaling)
chronic myeloproliferative disorders |
essential thrombocytosis and myelofibrosis (positive 30-50%)
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LMWH
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act on Xa, better bioavail, longer half life
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HIT
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heparin binds platelet factor IV, causing ab production that binds to and activates platelets leading to their clearance and resulting in thrombocytopenic hypercoagulable state
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Lepirudin, bivalirudin
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directly inhibit thrombin. for HIT
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warfarin tox
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skin/tissue necrosis
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thrombolytics labs, tox tx
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incr PT and PTT, no chage in PC
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tx tox with aminocaproic acid, an inhibitor of fibrinolysis
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clopodogrel, ticlopidine moa, use, tox
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irreversibly block ADP receptors
use: acute coronary syn, coronary stent, decr stroke risk |
tox: neutropenia (ticlopidine)
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cilostazol, dipyridamole
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phosphodiesterase III inhib, incr cAMP in platelets inhibits platelet aggreg, vasodilation
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use: intermittent claudication, coronary vasodilation, prevent stroke and angina
tox: N, HA, flush, hypoten, abd pain |
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abciximab use
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acute coronary syn, percutaneous transluminal coronary angioplasty
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Mtx
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leuk, lymph, choriocarcinoma, sarcoma, abortion, ectopic preg
inhibits DHF reductase |
tox: macrovesic fatty change in liver, teratogen, mucositis
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5-FU
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colon cancer and other solid tumors, BCC
inhibits thymidylate synthase |
tox: photosens, leucovorin doesn't work, need thymidine to rescue myelosup
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6-MP
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activated by HGPRTase
leuk, lymph but not CLL or hodg |
tox: BM, GI, liver, incr with allopurinol
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6-TG
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acute lymphoid leukemia
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tox: BM depression, liver, allopurinol ok
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Ara-C
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pyrimidine analog
AML, ALL, high grade NHL |
tox: leukopenia, thrombocytopenia, megaloblastic anemia
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dactinomycin
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intercalates in DNA
Wilms, Ewings, rhabdo, kids' tumors (kids ACT out) |
tox: myelosupp
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doxorubicin
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free radicals, noncov intercalate
hodg, myeloma, sarcoma, breast, ovary, lung |
myelosupp, alopecia
dexrazoxane chelates iron to prevent cardiotox |
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bleomycin
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free radicals
testicular CA, hodg |
skin changes
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etoposide
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SCC, prostate, testic
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myelosupp, GI, alopecia
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cyclophosphamide, ifosfamide
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alkylating. covalently crosslink DNA at guanine. req liver activ
NHL, breast, ovary. IS |
myelosupp, hemorrhagic cystitis (mesna helps)
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nitrosurease (carmustine, lomustine, semustine, streptozocin)
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req bioactiv. CNS. alkylates DNA
brain tumors |
dizzy, ataxia
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busulfan
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Alkylates DNA
CML, ablate BM before transplant |
hyperpigment, pulm fibrosis
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vincristine, vinblastine
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hodg, wilms, chorio, ALL
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tox: vincristine: neurotox and paralytic ileus
vinblastine blasts bone (BM sup) |
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paclitaxel
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ovarian, breast
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tox: myelosupp, hypersens
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cisplatin, carboplatin
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crosslink DNA
testic, bladder, ovary, lung |
tox: nephro, acoustic nerve damage, prevent nephro with amifostine (free radical scavenger) and chloride diuresis
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hydroxyurea
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inhibits ribonucleotide reductase, decr DNA synth
melanoma, CML, sickle cell |
BM supp, GI
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prednisone
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CLL, Hodg (part of MOPP)
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tox: cataracts, PUD, psychosis
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tamoxifen
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may incr risk of endometrial CA bc partial agonist. hot flashes. not raloxifene.
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trastuzumab
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herceptin. tyrosine kinase
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cardiotox
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imatinib
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also GI stromal tumors
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edema
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nephrotoxic, acoustic nerve damage
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cisplatin, carboplatin
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periph neuropathy
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vincristine
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cyclophosphamide
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hemorrhagic cystitis
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