heme-onc Flashcards
what is the classic smear finding for myelofibrosis?
teardrop cells
what might you think of in young person with diarrhea and iron deficiency anemia?
celiac dz
how do you use methlymalonic acid and homocysteine in megaloblastic anemia workup?
If B12 is borderline (200-400) you can order
in folate def HC is up
in B12 def both are up
what biliary complication do patients with hereditary spherocytosis get?
cholelithiasis from bilirubin stones
how do you test for PNH? what are the major consequences of the disease?
flow for CD55 & CD 59
can p/w hemolysis, hemoglobinuria, Fe deficienc, arterial or venous thrombosis
(mechanism is genetic change that affects a surface protein that when missing makes cells susceptible to complement-mediated lysis)
true/false: steroids are effective for both warm (IgG) and cold( IgM) antibody-mediated autoimmune hemolytic anemia.
false – used for IgG only. for IgM, just keep person warm and tx underlying cause (neoplas or infxn (mycoplasma, EBV))
what might you suspect in someone who appears cyantotic with a normal O2 sat? causes? tx?
methemoglobinemia – o2 sat falsely high but PaO2 will be low
exposure to nitrites/nitrats, tylenol, dapsone, sulfas, ‘caines
tx with methylene blue
what is tx for methemoglobinemia?
methylene blue
give levels of transferrin saturation and ferritin for which people should be tested for hemochromatosis?
transferrin sat >45% (women) or >50% (men) or ferritin >1000
what bleeding problems do people with primary hemostasis problems (platelets) present with? what about secondary hemostasis (factor deficiencies)?
platelets - gingival bleeding, petechiae, echymoses
factors - hemarthrosis, hematomas (more deep tissue)
PT monitors which coag cascade? PTT?
PT – extrinsic
PTT - intrinsic
(PT is like physical therapy so it measure the “exercise” pathway)
what disorders might you think of in someone with a normal PT, prolonged PTT and bleeding problems?
what about the same pt but with clotting problems?
bleeding – Factor VII, Factor IX, Factor IX or Factor XII deficiency
clotting – antiphospholipid Ab syndrome
what is diagnosis in somene with clotting and prolonged PTT?
antiphospholipid Ab syndrome
compare/contrast ITP and TTP.
ITP: autoimmune, can be idiopathic or from drugs or infxn, tx with steroids or IVIG; splenectomy is 2nd line, rarely bleed, NO schistocytes
TTP: FAT-RN (fever, anemia, thrombocytop, ARF, neuro sx), get thrombosis, atx with plasmaphresis or steroids, + schistocytes
why do we avoid abx in kids who might have EHEC diarrhea?
giving abx increases the cahnce that they get HUS!
which two disorders that present with thrombocytopenia have a high clotting risk?
HIT and TTP – so never give platelets
T/F: THere are schistocytes in TTP but not in ITP.
true
ITP: autoimmune, no schistos, tx wtih steorids/IVIG
TTP: FAT-RN, get clots, +schistos
what are tx options for bleeding in factor VIII deficiency?
DDAVP (causes release of vWF and Factor VIII) or recombinant Factor VIII
how are factor VIII and Factor IX deficiences inherited? VWD?
factor VIII and IX are recessive
VMD is dominant
which factor deficiency results in a long PTT but NO clinical bleeding, even with surgeries.
Factor XII
what bleeding disorders can present with nl plt, PT, PTT and bleeding time?
mild VWD – can check levels of VWF and F VIII
mild hemophlia – can chek levels o F VIII and F IX
F XIII deficiency - can do “clot lysis” test (these peopl present with late post-surgical bleeding and poor wound healing)
why is warfarin initially pro-thrombotic?
proteins C and S decrease before factors bc they have shortest half-life
what is a common complication of patients iwth protein C deficiency who are started on warfarin?
warfarin skin necrosis
what should you test for in pts with warfarin-related skin necrosis?
protein C deficieny (predisposes)
what 3 antibpodies make up the antiphospholipid abs?
lupus anticoagulant Ab
anticardiolipin Ab
anti-beta2-glycoprotein 1 Ab
how does Factor V Leiden cause thrombosis? Do you have to be homozygote or heretero?
it causes resistance to activated protein C which normally acts to inactivate Factor V and VIII so with them unchecked you clot more
homozygotes have 20x risk of clotting, heteros 7x
what is tx for follicular lymphoma (usually diffuse LAD in middle-aged with +CD20) in asymptomatic pts? symptomatic?
asymptomatic – watchful waiting
symptomatic – rituximab, chemo and prednisone
what type of RBC transfusion should you give someone who has a h/o severe anaphylaxis with trasnfusion? what disease should you suspect in someone with a h/o severe anaphylaxis with transfusion?
washed (b/c they are reacting against hte plasma proteins?
IgA deficiency can p/w severe anaphylaxis to transfusion (bc people have anti-IgA Abs)
what immunoglobulin deficiency can p/w severe anaphylaxis to blood transfusion?
IgA deficiency
compare and contrast when you use tamoxifen, raloxifene and aromatase inhibitors in tx of hormone-receptor positive breast cancer.
premenopausal – use tamoxifen
postmenopausal – use aromatase inhib
raloxifene – only used for tx of osteoporosis
true/false asymptomatic pts with hairy cell leukemia should be treated.
yes – 1 round of cladribine cures >80%
what is treatment for hairy cell leukemia
cladribine
what leukemia might you suspect in an elderly man with pancytopenia, splenomegaly without lympadenopathy and a dry aspirate when BM is attempted? what is tx?
hairy cell leukemia
tx with cladribine even if asymptomatic b/c cures >80% of pts
what can you do tx hromone receptor positive breast cancer in a pre-menopausal pt with h/o DVT?
ovarian ablation (tamoxifen CI 2/2 clot history)
contrast/compare the electrophoresis findings for thalasemmic pts with alpha-thal trai and beta-thal minor.
alpha-thal trait: have nl electrophoresis results
beta-thal: elevated heglobni A2 band.
what is diagnosis of african-american woman with MCV 70, low RDW, hgb 11 wiht normal hgb electrophoresis?
alpha-thal trait
beta-thal would show elevated hgb A2 band
what is diagnosis of african-american woman with MCV 70, low RDW, hgb 11 with elevated hgb A2 band on electrophoresis?
beta-thal
alpha-thal trait would have nl hgb electrophoresis
give the three cytogenetic changes assoc with favorable prognosis in AML.
t(8;21) (M2)
t(15;17)(M3–APML)
inv16(M4)
give thee cytogenetic changes assoc with negative prognosis in AML.
inv(3)
del(5q)
del7
for each of the following cytogenetic characteristics of AML, say if they give a favorable or unfavorable prognosis: inv(3) t(8;21) t(15;17) FLT3 mutation del(5q) del7 inv16 NPMI1 mutation
good: (t8;21),t(15;17), inv16, NPMI mutation
bad: del5q, del7, inv(3), FLT3 mutation
which AML is assoc with DIC?
APML (M3)
what is major SE of ATRA given to pts with APML?
ATRA sydnrome – fever, volume overload, effusions, respiratory distress and hypotension
tx by holding ATRA, giving steroids
what is standard induction for AML? who should be offered BMT?
ara-c x 7 days with daunorubicin x3 days
can tx if t achieve remission after induction or those with poor cytogenetics (inv3, del5q, del7)
what % of blasta on bone marrow defines acute leukemia?
> 20%
how is AML classified? ALL?
AML: M0 through M7 (M3 is APML)
ALL: precursor B, precursor T, mature B (Burkitt’s lyhphoma)