Hematology/Oncology Flashcards
Heparin to Warfarin bridge
Prot C and S w/ shorter t1/2
II, VII, IX, X longer t1/2
Transient paradoxic hypercoag before anticoag
Heparin vs LMWH
Heparin: activ antithromb; ↑PTT
Antidote: protamine sulfate
LMWH: esp inhib Xa; No eff on PTT
No eff of prot sulf
Warfarin
Inhib synth vitK-dep coag fact
↑PT
Reversal: rapid w/ FFP; slow vitK
! teratogen
tPA
Help conv plasminog to plasmin (breaks fibrin)
↑PT; ↑PTT
If toxicity, give aminocap acid
Factor Xa inhibitors
Factor IIa inhibitors
No monitoring (PT, PTT) No reversal agent
Hemophilia (PE)
X-linked; A=VIII; B=IX; C=XI Rare acquir (autoimm, post-part)
Young boy; spont hge tissue/joint (intracereb/GI/…)
Hge after surg, trauma, dental proced
If not ttt, arthropathy/joint destruction
Hemophilia (dg, ttt)
Dg: ↑PTT; normal PT/BT
Mixing study (#1): correct PTT
Specific fact assay (accur)
ttt: if sever bld or fact ≤1% (immediat transfu missing fact; cryoprecip)
If not sev and >1% (DDAVP releases VIII from endoth C)
von Willebrand disease (PE)
AD; #1 inherit bld ds
Defect or deficiency in vWF + ↓VIII
Types: 1 (mild-mod defic); 2 (qualit defect); 3 (No funct)
Child; recurr + prolong mucosal bld + bld after proced
⊕ fam Hx; sympt worse w/ ASA
von Willebrand disease (dg, ttt)
Dg: ↑BT; may ↑PTT (by ↓VIII); normal PT/PC
Accur: Ristocetin cofact assay + vWF Ag level
ttt: DDAVP (mild-mod); OCPs (if menorrhg); Avoid ASA and plt fct inhib
Bernard-Soulier syndrome
Glanzmann thrombasthenia
B-S: def GpIb (binds plt to endoth vWF)
G-T: def GpIIb/IIIa (binds 2 plts by fibrinogen)
ADP receptor inhibitors
GpIIb/IIIa inhibitors
ADP-R inh: clopidog/prasug/ticlopidine
No externaliz of GpIIb/IIIa
GpIIb/IIIa inh: abciximab/eptifibatide/tirofiban
Hypercoagulable states (etiologies)
Thrombophilias or prothrombotic states
Genet, acquir, physiol
3 main causes but same PE
- F.V Leiden: f.V resist to inactiv by activ prot C (#1 hered thromboemb ds)
- HIT: immuno react to heparin; Ab activates plt; bld clots + rapid ↓50% PC
- APS: ass w/ SLE/RA; art+ven thrombi; spont abort
Hypercoagulable states (PE, dg)
Recurr thromb (DVT, PE, art thromb, MI, stroke, abort)
HIT: hospit ptt on anticoag then ↓PC
F.V.L: young caucas + pers/fam Hx thromb
APS: middle-age women + recurr miscarr/thromb
Dg: r/o acqu causes before hered workup
Hered: 2 abNl values while asympt/unttt + similar values in 2 fam memb
HIT: PF4 Ab + serotonin release assay
APS: lupus anticoag + anticardiol Ab
Hypercoagulable states (ttt)
DVT/PE: heparin then 3-6mo warfarin (1st epis); 6-12mo (2nd); lifelong (next)
If anticoag CI (hge, sev HTN, recent trauma) or refract: IVC filter
HIT: !d/c hep; start argatroban/lepirudin
F.V.L: avoid OCPs
Protein C or S deficiency (PE)
Skin or tissue necrosis after Warfarin
Disseminates intravascular coagulation (RF)
Acquir; deposit of fibrin in Vx → thromb + end-org dam
Deplet coag fact + plts → bleed
Ass w/ obstetr complic; sepsis; neopl; ApML; pancreatitis; massiv trauma; drug react; ARDS; …
Disseminates intravascular coagulation (PE, dg, ttt)
Acute: bld from venipunct/in org; ecchym, petech
Chronic: bruis, mucos bld; thrombophleb; renal dysfct; neuro sympt
Dg: clinic+lab; #dg liv ds (but DIC w/ VIII is depressed)
ttt: underl cause; transfuse RBCs/plts/FFP; manage shock
Thrombotic thrombocytopenic purpura (PE)
Inherit or acquir (after inf)
vWF-cleav enz def (ADAMTS-13 def) → large vWF → plts microthrombi → end-org dam
RBCs fragm by microT → hemolysis
TTP if 3/5⊕: ↓PC; microang hemol anemia; neuro chang; impair renal fct; fever
Thrombotic thrombocytopenic purpura (dg, ttt)
Dg: schistoc + ↓PC + ↑creat
HUS: simil to TTP; child w/ EcoliO157H7; renal fail, hemol anemia, ↓PC, No neuro sympt
ttt: #1 plasma exchange; steroids to ↓thromb
CI for plt transfu
3 causes of microangiopathic hemolytic anemia
HUS
TTP
DIC
Idiopathic thrombocytopenic purpura (PE, etiologies)
IgG against plts → plt/Ab complex destr by spleen → ↑plt prod by BM
#1 immuno ds in middle-age women
Ass w/ leukemia/lymphoma/SLE/HIV/HCV
Acute (aft viral inf; 2-6yo) or chronic (20-40yo, esp women)
Asympt; minor mucocut bld, easy bruis, petech, hematuria, melena
Idiopathic thrombocytopenic purpura (dg, ttt)
Dg: of exclusion; r/o causes of thrombocytop then labs (Nl RBC morpho) BM biopsy (only if atyp or >60yo): ↑megakaryoc
ttt: No ttt (if plt >30K + no bld)
CS or IVIG: if plt <30K or bleed/sympt
If refract: splenectomy +/- rituximab +/- TPO recept agonist
9 #dg of thrombocytopenia
HIT HUS TTP ITP Medications Splenomegaly Hereditary (Wiskott-Aldrich sd) Chemotherapy Other (malignancy)
Iron deficiency anemia (etiologies, PE)
Normocytic then microcytic An
↑demand (pgncy, growth) or ↓iron (malnutr/malabs, bleed)
Esp toddlers, ado girls, women of childB age
Fatig, dyspn, tachycard, pica; asympt if slowly
Glossitis; conj pallor; cheilosis; koilonychia
Iron deficiency anemia (dg, ttt)
Dg: CBC; ↓iron, ↑TIBC/transferrin, ↓ferritin, ↑RDW; periph bld smear (hypochromic+microc w/ ↓reticulocyte count)
ttt: oral iron x4-6mo (!N, abdo pain, const/diarr)
CI antacids w/ iron
IV iron dextran (! anaphylaxis)
Anemia of chronic inflammation/disease
Chronic infl, inf, malign, RA, SLE
Body hides iron (limit bact prolif)
Normocytic then microcytic An
Dg: ↓iron, ↓TIBC/transf, ↑ferritin
Sideroblastic anemia
Microcytic An
Def in heme metab (hered; alcoh, lead poison, chloramph, INH, malign)
Dg: ↑iron; basoph stippl (periph smear); ringed sideroblasts (BM)
Megaloblastic anemia (etiologies, PE)
Macrocytic anemia
Impaired DNA synth (vitB9 def, vitB12 def, MTX, 6-MP)
-B12: intest malabs (Crohn/celiac/gastrect/panc insuff), pernicious An, vegetarian, Diph latum
-B9: alcoh, ↓diet folate, malabs, phenytoin
Fatig, pallor, diarr, ↓appet, headac
B12: demyelin (subacute comb degen of cord)
Megaloblastic anemia (dg, ttt)
Dg: CBC, smear w/ hypersegm neutroph (≥6 lobes)
MMA+homocysteine (↑both in B12 but MMA Nl in B9)
BM (giant+hyperseg neutro); Schilling test (for B12)
ttt: underl cause
Hemolytic anemia (etiologies)
Normocytic An
BM unable to compens for ↑destruc
- Intrinsic: memb def (spheroc), enz def (G6PD def), HbC, SCD, parox noct hemoglobinuria
- Extrinsic: autoimm, microangiop (HUS/TTP/DIC), macroangiop (mechan heart valv), inf (malaria), hypersplenism
Hemolytic anemia (PE, dg, ttt)
Pallor, fatig, tachycard, tachypn
Dg: jaund, ↑ind bili, ↑reticuloc, ↑LDH, ↓haptogl
ttt: underl cause; CS in immuno; iron to replace loss
If severe, splenectomy or transfusions
G6PD deficiency
X-linked; hemolyt An after oxidant stress
Episod dark urin + jaund
Trigger: sulfa drug, antimalar, inf, fava beans, metab acid
Dg: CBC, smear (bite cell, Heinz); G6PD level after 1mo
ttt: avoid trigg
Paroxysmal nocturnal hemoglobinuria
Def in GPI-anchor molec → No CD55/CD59 on RBCs → No protec ag complem-med hemolysis
Dg: An, dark urin, venous thromb, abdo pain; flow cytom w/o CD55/59 (accur)
Hereditary spherocytosis
AD def in spectrin/ankyrin
Spherical RBCs destroyed by spleen (extravasc hemol)
Dg: CBC + smear; osmotic fragil test (accur)
ttt: splenectomy (+pneumo/meningo/haemo vacc before)