Hematology

Question 1

Diagnostic procedure to quickly distinguishing AML from ALL and for identifying subtypes of AML

Answer 1

Multiparametric flow cytometry

Question 2

Most important prognostic information in cytogenetically normal AML (3)

Answer 2

NPM1 mutation
FLT3-ITD
biallelic CEBPA mutations

Question 3

Poor prognostic indicator of AML

Answer 3

Advancing age

• 1) survive induction therapy d/t coexisting medical comorbidities
• 2) older = greater proportion have intrinsically more resistant disease

Question 4

Poor outcome in AML (3)

Answer 4

1. Hyperleukocytosis (>100000)
2. Early CNS bleed
3. Pulmonary leukostasis

Question 5

Complete remission in AML (6)

Answer 5

1. Neutrophil >/= 1,000mcL
2. Plt count >/= 100,000mcL
3. BM <5% blasts
4. Neg circulating blasts
5. Neg Auer rods
6. Neg Extramedullary leukemia

Question 6

What is the 7 and 3 regimen in AML?

Answer 6

Cytarabine and Anthracycline (Daunorubicin, Idarubicin)
> Cytarabine 100-200mg/m2 IV for 7 days
> Daunorubicin 60-90mg/m2 or Idarubicin (12mg/m2) IV on days 1, 2 and 3

Question 7

It is a CD33-targeting immunoconjugate, added to induction therapy for subsets of patient, especially those with CBF AML

Answer 7

Gemtuzumab Ozogamicin

Question 8

Treatment of patients with AML, >65 years, and those unable to receive intensive therapy due to comorbidities?

Answer 8

1. Low intensity therapy with hypomethylating agent (HMA: Decitabine or Azacitidine), OR
2. Low-dose cytarabine, in combination with daily venetoclax

Question 9

What is used in monitoring APL?

Answer 9

RT PCR for PML-RARA

Question 10

What is the Philadelphia chromosome?

Answer 10

t(9;22)(q34.1;q11.2)

Question 11

What TKI is effective against T315I?

Answer 11

Ponatinib

Question 12

What is accelerated phase CML?

Answer 12

1. Peripheral blasts >15%
2. Peripheral blasts + promyelocyte >30%
3. Peripheral basophils >20%
4. Cytogenetic clonal evaluation: trisomy 8, double Ph, isochromosome 17, 17p deletions, 20q-, others
5. Thrombocytopenia <100

Question 13

Biopsy finding of CML

Answer 13

Marked hypercellularity with replacement of fat spaces, normal elements, and occasionally increased fibrosis

Question 14

What disease causes accumulation of iron in mitochondria appears in necklace fashion around the nucleus of the erythroblast (ring sideroblasts)?

Answer 14

Myelodysplastic Syndrome

Question 15

Indication to give parenteral iron

Answer 15

1. Unable to tolerate oral iron
2. Needs are relatively acute
3. Need iron on an ongoing basis - persistent GI or menstrual loss

Question 16

EPO requirement of patients with CKD

Answer 16

EPO 50-150U/kg 3x a week

Question 17

EPO requirement of patients with cancer

Answer 17

EPO 300U/kg 3x a week

* Darbepoetin alfa → weekly or every other week dosing
* EPO mimetics (Roxadustat) → 50 mg PO thrice weekly

Question 18

What laboratory test reflects an inadequate iron supply to erythroid precursors to support hemoglobin synthesis?

Answer 18

Proporphyrin

* NV : <30mcg/dL
* IDA: >100mcg/dL

D/t increased in absolute or relative iron deficiency and lead poisoning

Question 19

Pretransfusion goal of Beta-thalassemia

Answer 19

9-10.5g/dL+ Oral iron chelation
Every 2- 4 weeks

Question 20

Three drivers of mutations in Polycythemia Vera Syndrome (3)

Answer 20

1. JAK2
2. CAL-R
3. MPL

Question 21

Most frequent infectious cause of HA

Answer 21

Malaria

Question 22

Most frequent infectious cause of HA in non-malarial areas

Answer 22

Shiga toxic-producing E. coli O157:H7

Question 23

What is an acquired intracorpuscular defect type of hemolytic anemia?

Answer 23

Paroxysmal Nocturnal Hemoglobinuria

Question 24

What is an inherited extracorpuscular factors that leads to hemolytic anemia?

Answer 24

Familial hemolytic-uremic syndrome

Question 25

What is the telltale sign if the hemolysis is intravascular?

Answer 25

Hemoglobinuria

Question 26

What is the only condition that will present as an increased mean corpuscular hemoglobin concentration (MCHC > 34)

Answer 26

Hereditary Spherocytosis

Question 27

What is the definitive treatment for paroxysmal nocturnal hemoglobinuria for young patients?

Answer 27

Allogeneic bone marrow transplant

Question 28

Toxin-producing microorganism with lecithinase activity causing life-threatening intravascular hemolysis

Answer 28

Clostridium perfringens

Question 29

Severe disease in Aplastic Anemia

Answer 29

Any of the 2: ANC < 500mcL Platelets < 20,000 Corrected reticulocyte count <1% or absolute reticulocyte count <60,000

Question 30

Conditions associated with microcytic erythrocytosis (3)

Answer 30

1. PV 2. B-thalassemia 3. Hypoxic erythrocytosis

Question 31

Independent prognostic factors in NHL

Answer 31

B2 micro globulin level and serum LDH

Question 32

Indications to do PET scan in NHL

Answer 32

Indolent lymphoma to an aggressive lymphoma is suspected Differentiate treated vs active for CT of residual masses Guideline: > For HL and DLBCL > FL: Used for prognosis - Residual PET-avid disease: Poorer prognosis > Clinical trial > End of tx scan: * not be done before 3 weeks * 6-8 weeks after CHEMO * 8-12 weeks after RAD or CHEMORADIO

Question 33

Major regulator of platelet production

Answer 33

Thrombopoietin Produced in the liver

Question 34

What can be given for patients with refractory ITP?

Answer 34

Rituximab

Question 35

Mainstay treatment of TTP

Answer 35

Plasma exchange

Question 36

Mainstay treatment for type 1 VWD

Answer 36

DDAVP

Question 37

A disorder where abnormal telangiectatic capillaries result in frequent bleeding episodes, primarily from the nose and gastrointestinal tract.

Answer 37

Osler-Weber-Rendu Syndrome

Question 38

Patients with this condition develop painful episodes of perifollicular skin bleeding as well as more systemic bleeding symptoms.

Answer 38

Vitamin C Corkscrew hairs

Question 39

Vitamin K-dependent coagulation

Answer 39

1972, with Protein C and S 🔹 Factor II (Prothrombin) 🔹 Factor VII 🔹 Factor IX 🔹 Factor X

Question 40

Triad of PNH

Answer 40

1. Intravascular hemolysis 2. pancytopenia 3. risk of VTE