Heme

Question 1

Why are blood smears not effective for microcytosis

Answer 1

All are hypo chromic and can have target cells

Question 2

What patients with anemia are transfused?

Answer 2

Symptomatic or low hct in elderly or heart diseased

Question 3

Low ferritin microcytosis?

Answer 3

Iron deficiency

Question 4

High iron microcytosis?

Answer 4

Sideroblastic

Question 5

Normal iron microcytosis?

Answer 5

Thalassemia

Question 6

Iron deficiency anemia unique lab features?

Answer 6

Increased rdw and increased platelet count

Question 7

Sideroblastic unique lab features

Answer 7

Ringed blasts with Prussian blue

Question 8

Thalessamia diagnosis test

Answer 8

Electrophoresis

Question 9

First step in distinguishing macrocytic anemia

Answer 9

Peripheral blood smear to detect hypersegmented neutrophils

Question 10

Classic neuropathy of b12 deficiency

Answer 10

Posterior column damage to position and vibratory sensation possibly causing ataxia

Question 11

What test distinguishes b12 and folate deficiency

Answer 11

Mma is elevated in b12 only

Question 12

What is a complication of b12 or folate deficiency

Answer 12

Hypokalemia

Question 13

Sickle cell mutation?

Answer 13

Point mutation at position 6 of beta globin changes glutamic acid to valine

Question 14

Diagnostic tests in scd

Answer 14

Initial: peripheral smear
Accurate: hb electrophoresis

Question 15

Where do you see Howell jolly bodies

Answer 15

Scd

Question 16

What lowers mortality in scd

Answer 16

Hydroxyurea + Abx with fever

Question 17

Vaccine for asplenia in scd?

Answer 17

Pneumococcal

Question 18

Test for aplastic crisis in scd? And tx?

Answer 18

Initial: reticulocyte count
Accurate: pcr for parvo b19 dna
Treat: IVIG

Question 19

Treat hereditary spherocytosis

Answer 19

Chronic folic acid + splenectomy

Question 20

Treating AIHA

Answer 20

Warm: steroids –> splenectomy –> IVIG –> Rituximab
Cold: stay warm –> Rituximab –> other immunosuppressive

Question 21

G6pd presentation

Answer 21

African or Mediteranean male with sudden anemia after infection or dapsone/quinidine/sulfa/primaquine/nitrofurantoin/fava beans
Initial: Heinz bodies
Accurate: test g6pd level 1 to 2 months after hemolysis

Question 22

What disease is worsened by platelet transfusion

Answer 22

Ttp and hus

Question 23

PNH cause of death and treatment

Answer 23

Thrombosis (mesentaric or hepatic V)

Tx: steroids –> BM transplant (cure) –> Eculizumab to inactivate C5

Question 24

Inhibitor of JAK2

Answer 24

Ruxolitinib

Question 25

AML (4 things)

Answer 25

M3 gene (causes DIC, treat with ATRA) Auer Rods cr1517 myeloperoxidase

Question 26

Persistently elevated WBC count of all neutrophils

Answer 26

CML

Question 27

CML test + initial tx + cure

Answer 27

Accurate test: BCR-ABL (922) on PCR or FISH Tx: TK inhibitor (imatinib) Cure: BMT = never 1st therapy!

Question 28

Leukostasis rxn 1st step

Answer 28

Leukapheresis

Question 29

Classic abnormality of MDS

Answer 29

5q deletion

Question 30

Distinct lab abnormality of MDS

Answer 30

Pelger Huet cells (bilobed nucleus)

Question 31

Treating MDS

Answer 31

Lenalidomide for 5q deletion to decrease transfusion dependence Azacitidine also decreases transfusions and increases survival

Question 32

Treating CLL

Answer 32

``` Stage 0 (elevated WBC): None Stage 1 (lymphadenopathy): none Stage 2 (hepatosplenomegaly): none Stage 3 (anemia), stage 4 (thrombocytopenia): fludarabine + cyclophosphamide + Rituximab Fludarabine failure: Alemtuzumab Refractory cases: Cyclophosphamide Mild cases in elderly: Chlorambucil Severe infection: IVIG AIHA/thrombocytopenia: prednisone ```

Question 33

Accurate test for hairy cell leukemia

Answer 33

Immunotyping by flow cytometry

Question 34

Treat hairy cell leukemia

Answer 34

Cladrabine or Pentostatin

Question 35

NHL initial test and severity correlation

Answer 35

``` excisional biopsy (NOT needle aspiration) High LDH indicates worsening severity ```

Question 36

Staging NHL

Answer 36

1: lymph node group 2: 2 or more nodes on 1 side of diaphragm 3: both sides of diaphragm 4: widespread disease

Question 37

NHL tx

Answer 37

Local (1a/2a): local radiation and small chemo dose | 3+: CHOP (Cyclophosphamide/Adriamycin/Vincristine/Prednisone) + Rituximab

Question 38

HL

Answer 38

RS cells | Lymphocyte predominant is good for prognosis

Question 39

Treating stage 3 or 4 HL

Answer 39

ABVD: Adrimycin (doxorubicin)/Bleomycin/Vinblastine/Dacarbazine

Question 40

Radiation complications

Answer 40

Cancer (screen 8 years later), CAD

Question 41

What does the EF need to be for Adrimycin and how do you screen

Answer 41

>50% | MUGA or nuclear vetriculogram

Question 42

4 presenting factors of Multiple Myeloma

Answer 42

Bone pain Hyperuricemia (high turnover) Anemia (bony infiltration) Renal Failure (accumulated IG and Bence Jones)

Question 43

Diagnostics of MM

Answer 43

``` Punched out lyric lesions SPEP shows IgG or IgA spike HyperCa Bence JOnes Rouloux formation decreased AG due to IgG (cationic) increasing Cl and HCO3 ```

Question 44

Why does urinary level of protein stay low in MM

Answer 44

Dipstick only measures albumin

Question 45

SPEP results

Answer 45

99% of those with an M spike do NOT have MM --> must do BM biopsy

Question 46

Waldenstrom Macroglobinuria (pathophys/sx/tx)

Answer 46

Increased IgM causes hyper viscosity Lethargy/engorged eye vessels/mucosal bleeding Plasmapheresis initially

Question 47

Bleeding disorders (platelet vs factor)

Answer 47

Superficial vs deep

Question 48

Initial management of mild bleeding

Answer 48

Prednisone

Question 49

Vaccines before splenectomy

Answer 49

Meningitis H flu Pneumococcus

Question 50

VWD dx and tx

Answer 50

Dx: normal platelet count, worsened by ASA, Ristocetin + Tx: DDAVP

Question 51

Hemophilia dx

Answer 51

Delayed joint or muscle bleeding in a male child, prolonged PTT

Question 52

Confirming HIT

Answer 52

ELISA for platelet factor 4 (PF4) ABs or the serotonin release assay

Question 53

Tx for HIT after stopping heparin containing products

Answer 53

Administer direct thrombin inhibitors (Argatroban, Lepirudin, Biivalirudin, Fondaprinux) --> administer Warfarin after

Question 54

Most common cause of hyper coagulation

Answer 54

Factor V Leiden

Question 55

Tx for Anti Phospholipid Syndrome

Answer 55

Possibly lifetime anticoagulation

Question 56

Initial evaluation of anemia

Answer 56

CNBC