Path 1

Question 1

Anisocytosis vs poikilocytosis. Iron can be stored in; marker? Iron hemostasis?

Answer 1

Abnl RBC size vs Abnl RBC shape; schist, target cells, echino/burr (PKD), acantho/spur (abetalipoproteinemia). Kupfer, splenic red pulp Mac’s, bone marrow; ferritin. Hepcidin blocks ferroportin -> no iron in/out; inc in iron overload, dec in anemia & inc erythropoiesis

Question 2

HbH vs hydrops fetalis. Complications vs tx of beta thal

Answer 2

Beta tetramers, high affinity to O2 -> hypoxia, HbH [O] -> RBC inclusions -> cleared by spleen, severe anemia vs zeta chains, HbPortland, 2o hemochromatosis & cardiac, tx w/ intrauterine & lifelong blood transfusion. Crewcut/chipmunk, erode bony cortex, cachexia, 2o hemochromatosis & cardiac vs blood transfusion +/- iron chelators

Question 3

Key features vs triggers vs labs/dx G6PD defic

Answer 3

Heinz/bite, inc fragmentation & rigidity, extravasc hemolysis, hemolytic anemia vs fava, nitrofurantoin, prima/chloroquine, oxidants, sulfa, free radicals vs bone marrow bx shows hypercellular bone marrow + erythroid hyperplasia, neg Coombs, spherocytosis, violet Giemsa; dx by Buetler fluorescent spot test

Question 4

Causes of relative polycythemia. Tx? Labs vs sxs of polycythemia Vera?

Answer 4

Dehydration, burns, diuretics, V/D. Replenish fluids & tx underlying cause. Inc normochromic/cytic RBC, PLT, granulocytes, splenomegaly till spent phase -> anemia but still thrombo/granulocytosis, teardrop vs joint pain, pruritus, epigastric pain

Question 5

Posthem anemia: acute vs chronic

Answer 5

Hypovol/perfusion -> renal failure, ECF to ICF -> dec RBC & HCT -> normocytic/chromic anemia, inc EPO -> transient macrocytosis vs inc EPO, deplete iron stores -> iron defic anemia

Question 6

Chloramphenicol, phenylbutazone, gold vs sulfa, sz meds, degreasing agents, benzene. CD8, anti-diazepam, anti-kinectin. Bone marrow bx shows pancytopenia, adipose, hypocellular; NO FIB; peripheral blood smear shows normocytic/chromic anemia. Blood transfusion, PLT; immunosuppression, HSC transplant

Answer 6

Dose dep vs idiosyncratic (4) aplastic anemia. Mechanism? Dx? Tx?

Question 7

Types of cells seen in ACD/ACKD? Assoc conditions?

Answer 7

Echino & acantho. RA, osteomyelitis, IBD, lưng/br/hodgkin, endocarditis, CHF, DM, obesity

Question 8

Causes for macrocytosis? Complications of B12 deficiency?

Answer 8

Cirrhosis/alc , reticulocytosis, myelodisplastic syndrome, hypothyroidism, hydroxyurea/MTX/cap/clad/AZ-6MP. SCD -> dx by Romberg test, bright T2W in posterior column, Schilling test; pernicious anemia -> shiny tongue, lemon skin, shuffling gait, mentally sluggish

Question 9

General idea of hemolytic anemia. Mechanism vs dx vs tx of spherocytosis?

Answer 9

Intravasc: dec haptoglobin, inc Hb -> inc unconj bili; NO splenomegaly
Extravasc: RES -> splenomegaly
General sxs: anemia, jaundice/cholelith, reticulocytosis, erythroid hyperplasia
AD>AR frame shift/premature stop -> bud/shed vesicles from mềm -> dec SA:V ratio x-> spherocytes -> can’t pass thru capillaries-> cleaned by macs-> splenomegaly vs Eosin binding test to band 3, osmotic fragility test inc vs blood transfusion & EPO, splenectomy

Question 10

Histo vs complications vs tx sickle

Answer 10

Target cells, sickle, Howell Jolly vs AVN, dactylitis, renal medulla, crewcut/chipmunk, ACP syndrome, ischemic stroke, aplastic crisis, encapsulated bacteria (H flu, S pneumonia, salmonella -> osteomyelitis)

Question 11

Acute vs delayed hemolytic transfusion rxn

Answer 11

Back/flank pain, fever/chills, DIC sxs; inc LDH, hgburia, unconj bili; +Coombs vs ab exposed -> ab -> hemolytic response; same labs + spherocytosis

Question 12

Paroxysmal nocturnal hburia

Answer 12

Somatic mutation PIGA Xp22.1 -> no GPI anchor -> no CD55/59 (MIRL) + C3b/4b -> overactive C’ -> hemolysis, depleted NO -> vasoconstrict, smooth mm ctx, eso spasm, no GI Perfusion; pos Coombs, hburia + hemosiderin; dx flow cytom -> low GPI anchor. Tx with ecluzimab

Question 13

Bernard (3) vs Glanzmann (1). Sx and dx for hemophilia A (3) vs B (mild/moderate/severe)

Answer 13

AR GPIb. Thrombocytopenia, giant PLT, severe bleed vs AR ITGA2B/B3. lifelong hemorrhagic syndrome -> sport mucocutaneous bleed
Joint/hemarthroses, CNS, mm. Dx by measuring FVIII lvls vs mild -> bleed from surg/tooth; mod -> bleed from surg/tooth + minor injuries; severe -> bleed from same + spont bleed. Dx by measuring FIX lvls

Question 14

DIC mechanism (4) vs sx vs dx

Answer 14

TF, OB, malig, sepsis; use up all clotting factors -> anticoagulant factors vs microangiopathic hemolytic anemia, coag then bleeding diathesis or thrombosis vs inc PT/PTT, FDP/D dimers

Question 15

LOF anticoag: AT vs C vs S defic

Answer 15

AD SERPINC1. no degrading 2/9/10, no interaction w/ heparin -> DVT/PE. Dx w/ AT-heparin assay or DNA Analysis vs AD PROC. No degrading 5/8 -> neonatal or DVT, warfarin induced. dx w/ C activity assay or DNA analysis; bx of microthrombi vs AD PROS1. No cofactor for C -> no degrading 5/8 -> neonatal or DVT. Dx w/ S activity assay or DNA analysis

Question 16

GOF procoagulant: Factor V Leiden vs prothrombin G20210A mutation

Answer 16

AD F5 G1691A -> resistant to C -> activate prothrombin -> DVT/PE. Dx by fxnal coag test or DNA analysis vs AD F2, Ca2+ dep & reversible complex -> inc prothrombin to get activated -> venous thrombosis. Dx by PCR

Question 17

Acquired coag: ALS vs ca vs preg

Answer 17

aPL bind to GPIb or endothelial receptors -> NO (toxic) -> venous > arterial thrombosis. 2 hit hypothesis. Dx by aPL, anticardiolipin, antiGPIb assays. No tx needed vs TF, ca procoag activation FX, fibrinolytics dissolving clots, TNFa & IL1B vs Virchows 3ad from gravid ut, placental insuff, inc common and 9/8 coag pathway while dec S