Hematology

Question 1

Iron deficiency is the only anemia with elevated______

Answer 1

RDW

Question 2

Only microcytic anemia with high reticulocyte count _____

Answer 2

HgH (hemoglobin H, variant of alpha thalassemia)

Question 3

B12 deficiency can be tested with a b12 level, and ____

Answer 3

methylmalonic acid, homocysteine (both elevated)

B12 deficiency can also look hemolytic (LDH, haptoglobin)

Question 4

___ electrolyte deficiency can occur after treating B12 deficiency

Answer 4

Low potassium

Question 5

Treatment for severe sickle cell disease______

Answer 5

Exchange transfusion

• Pulm infarction
• priapism (try aspirating first)
• Stroke
• Retinal Infarction

*start fluids, O2, possibly Abx, hydroxyurea, maybe IVIG

Target sickle cell percent = 15%

Question 6

Sickle cell patient with sudden drop hemoglobin ______, _____

Answer 6

Folate deficiency, parvovirus B12

- Reticulocyte count will be abnormally low-normal

Question 7

Only signs of sickle cell trait ____, ____

Answer 7

Dilute urine, hematuria/renal defect

Question 8

Autoimmune hemolytic anemia (warm antibody) associated with lymphoma, other auto-immune (SLE, RA), drugs (quinine, sulfa, penicillin), will show ______ on the peripheral smear

Answer 8

Spherocytes

Tx: Steroids, rituximab—> IVIG —> Splenectomy

Question 9

Cold antibody hemolytic anemia.
Coombs ____
Complement ____

Only responds to Tx______

Answer 9

negative

positive

Tx: Rituximab

Question 10

Heinz body, bite cells is a sign of ______

Drug most correlated

Answer 10

G6PD deficiency

Dx: G6PD level 2 months after episode

Dapsone, rasburicase

Question 11

Only condition causing elevated MCHC____

Caused by defect in _____

Diagnosed with ____ assay

Answer 11

Hereditary spherocytosis
- splenomegaly, hemolysis, bilirubin gallstones

Ankrin/spectrin cytoskeletal proteins

EMA (eosin-5′-maleimide (EMA) binding test) , or osmotic fragility

Question 12

Treatment of severe HUS or TTP______

Answer 12

Plasmapharesis , not steroids

Eculizumab only used for non-infectious HUS

ADAMS TS 13 breaks down Vwf, to break down platelet strands, plasmapheresis replenishes VwF

Question 13

_____ characterized by pancytopenia, dark urine episodes early morning, diagnosed with ____

Answer 13

PNH

CD55, 59

Tx: Eculizumab, vaccinate with menningococal vaccine before

Cx: AML

Question 14

_____ during pregnancy results in hemolysis, liver injury, distinguished from DIC since no change to PT/PTT

Answer 14

HELLP syndrome

Question 15

Anaphylaxis from blood transfusion caused by ______

Answer 15

IgA deficiency

Question 16

Type of leukemia most associated with DIC_____

Answer 16

Promyelocytic leukemia, treated with all trans retinoid acid or arsenic trioxide

Question 17

_____ smoldering, pancytopenia disease with pelger huet cells on smear, treated with linalidomide

Answer 17

MDS

Another treatment: azacitidine

Question 18

How to distinguish reactive vs CML neutrophil count____. CML cytogenetic hallmark_____

Answer 18

LAP score (Low score)

Philadelphia chromosome (BCR/Abl)

Question 19

____ characterized by elevated lympocytes, and smudge cells on smear

Answer 19

CLL

Tx: Rituximab, fludarabine, –> Venetoclax

Question 20

Massive splenomegaly, age = 50, panctopenia ______

Answer 20

Hairy cell leukemia

Tx: Cladribine

Dx: Smear + Flow Cytometry

Question 21

Pancytopenia, splenomegaly with tear drop cells_____ treated with _____

Answer 21

Myelofibrosis, Ruxolitnib (Anti Jak2)

Question 22

Treatment of polycythemia vera_____

Answer 22

aspirin, phlebotomy, hydroxyurea, —> ruxolitnib/anagrelid

Dx: LAP, B12 level

Tx: Hydroxyurea

Question 23

Treatment of polycythemia vera_____

Answer 23

aspirin, phlebotomy, hydroxyurea, —> ruxolitnib/anagrelid

Dx: LAP, B12 level (elevated)

Tx: Hydroxyurea

Question 24

Multiple myeloma workup

1) Skeletal Survey
2) SPEP: IgG monoclonal
3) UPEP: Bence jones proteins
4) Free light chain : Kappa/lambda

Most effective therapy:

Answer 24

Rouleux formations

Autologous bone marrow transplant, others: lenalidomide/thalidomide/bortezomib

Question 25

Treatment of Waldenstrom_____

Answer 25

Plasmapheresis Rituximab/Fludarabine

Question 26

Reed stern burg, owl eyes cells associated with ____

Answer 26

Hodgkins Lymphoma

Question 27

Best diagnostic step for Hodgkins and Non-Hodgkins_____

Answer 27

Excisions lymph node biopsy Tx: CHOP, R-CHOP, ABVD

Question 28

______ caused by lack of platelet stabilization and function, diagnosed with ____

Answer 28

Von willibrand, ristocetein assay or Vwf assay Hack: elevated PTT, Plt levels are normal Tx: ddAVP, Factor 8 replacement

Question 29

disease characterized by low Plt, no splenomegaly, megakaryocytes in bone marrow with large platelet chunks on peripheral smear____

Answer 29

ITP

Question 30

Treatment of ITP_____

Answer 30

Only treat if Plt<50,000 Acute: Steroids, IVIG (Plt<20,000 + bleeding) Chronic: Romiplostim, Splenectomy

Question 31

How to tell if clotting factor deficiency and inhibitor_______

Answer 31

Mixing study | - If deficiency, mixing will correct

Question 32

glanzman and bernard soulier act like VwF, ristocetein assay will show no binding, ddAVP can be used to release sub endothelial stores , will show giant platelets on smear

Answer 32

Glanzman (low glycoprotein IIb, IIIa) for fibrinogen bridging, like being on abxiximab Bernard soulier caused by deficiency in a VwF receptor (Giant platelets) Tx: ddAVP, also only works for hemophilia A (Factor 8)

Question 33

How is a factor 8 inhibitor treated______

Answer 33

Factor 7, bypasses to Factor 10

Question 34

HIT 1) Diagnosis: 2) Treatment:

Answer 34

Plt Factor 4 Antibody Fondaparinaux, Argatroban, Bivalirudin

Question 35

Test for antiphospholipid antibody syndrome____

Answer 35

Russel viper venom