Hematology

Question 1

Tumor lysis syndrome risk

• high
• intermediate
• low

Answer 1

High:

• AML, ALL
• Lymphomas
• intermediate risk with elevated electrolytes

Intermediate:

• solid tumors
• lower grade lymphomas

Low risk:
- Solid tumors

Question 2

Treatment of TLS

Answer 2

Rasiburicase- causes breakdown of the urate therefore use if kidney disease and hyperuricemia already. Do no use in G6PD deficiency

Hydration with UOP 80-100cc/hr

Allopurinol: use if no evidence of hyperuricemia. Increases the excretion of urate. Do no use in kidney disease

Question 3

Myelofibrosis

• Symptoms
• Diagnosis - peripheral smear
• bone marrow biopsy

Answer 3

Myeloproliferative disorder

clonal proliferation of abnormal stem cells produce cytokines that promote fibrosis of the bone marrow resulting in:

• splenomegaly/hepatomegaly (from extra-medullary hematopoiesis)
• — may develop portal hypertension
• anemia (normocytic) - may have initial leukocytosis
• thrombocytopenia - may have initial thrombocytosis
• normal MCV

On peripheral Smear:

• Tear drop cells
• circulating erythroblasts and myeloid precursors
• giant platelets

Bone Marrow:
- Dry tap

Commonly JAK2 mutation

treatment:
- Hydroxyurea and ruxolitinib
- Allogenic HSCT if <60yo

Question 4

Myelofibrosis Treatment

Answer 4

Hydroxyurea
Ruxolitinib (JAK 2 inhibitor)
HSCT <60yo

** PV at risk for developing “post pv myelofibrosis”

Question 5

Myelodysplastic Syndromes

• Symptoms
• Diagnosis
• bone marrow biopsy
• Flow cytometry

Answer 5

Due to Ineffective hematopoiesis

symptoms:
- associated with cytopenias

Diagnosis

• at least 2 lines of cytopenias
• Anemia with Elevated MCV (normal b12, folate, no alcoholism)
• NO hepatosplenomegaly

Bone Marrow:

• Hypercellular (intra-medullary apoptosis; therefore cytopenias)
• Flow cytometry: look for 5q - specific for MDS

Question 6

Myelodysplastic Syndrome Treatment

Answer 6

IPSS score to determine risk and subsequently treatment:

• low risk: no treatment
• If <60yo and High risk: HSCT
• if >60yo and High risk: 5-azacytidine

if 5q- lenalidomide

Question 7

Graft Versus Host Disease- Acute

Answer 7

Graft T cells attack the Gut, Skin, Liver, Renal system- (all over)

Acute: Within 100 days of transplant 
Skin: bullous rash, TENS 
Liver: Elevated LFTs 
Gut: Dyspepsia, N/V 
Renal: SLE like Nephritis 

Treatment:

• High dose steroids
• Cyclosporine and MTX

Question 8

Graft Versus Host Disease- Chronic

Answer 8

Can happen at any time.
* fibrotic changes more than inflammatory as in acute

Skin:  Hypo/hyperpigmentation, lichen planus, fibrotic changes 
Liver: Elevated LFTs
Gut: Esophageal webs and strictures 
Lung: BOOP 
Joints: Stiffness, fascitis 

Treatment:
Localized: topical steroids
Diffuse: Steroids, cyclosporine

Question 9

Essential Thrombocythemia

• symptoms
• Diagnosis

Answer 9

Myeloproliferative disorder

Symptoms:

• Headaches, dizziness, erythromelalgia, blood clots (arterial and venous.
• IDA anemia that corrects with Iron, but platelets are still increased
• Splenomegaly
• At risk for acquired vWD due to consumption

Diagnosis:

• elevated platelets >600k on 2 separate occasions, 1 month apart.
• JAK 2 mutation may be present

Treatment:

• Low risk: ASA
• High Risk: HU
• plateletpharesis if stroke, MI, TIA, GI bleeding

Question 10

Hairy Cell Leukemia

Answer 10

B cell accumulate in the bone marrow- cytopenias and splenomegaly.

Sx:

• associated with cytopenias
• Abd pain due to splenomegaly

Diagnosis

• Peripheral smear: hairy Cells
• Bone marrow biopsy with Flow cytometry

Treatment:
- Chemo

Question 11

Leukoreduced

Answer 11

When the RBCs separated from whole blood, WBCs can remain with the RBCs.

• Leukoreduction further removes WBCs but not ALL the components of WBCs
• CMV resides in WBCs therefore reduces the risk of transmission in CMV negative patients.

Indicated for

• frequent transfusions
• CMV negative at risk (AIDs, transplant)
• potential transplant candidates
• Previous febrile non-hemolytic reactions

Question 12

Washed

Answer 12

When RBCs separated from whole blood, plasma can remain. Washing removes the plasma (proteins)

Indicated for

• IgA deficiency
• Complement related autoimmune hemolytic anemia
• Continued allergic reactions to transfusion despite pre-treatment

Question 13

Irradiated

Answer 13

When RBCs separated WBC components remain
- Irradiation removes all WBC components

Indicated for

• BMT patients
• Acquired or genetic Cellular immunodeficiency
• blood donated by 1st or 2nd degree relatives

Question 14

Polycythemia Vera

Answer 14

Myeloproliferative disorder

Jak2 mutation
Suspect with Hemoglobin 18.5 (m), 16.5(w) and after secondary causes have been eliminated

sx: plethora, erythromelgia, pruritis, splenomegaly, thrombosis or bleeding. May have stroke, DVT, or budd chiari syndrome.

tx: therapeutic phlebotomy to lower to hemocrit <45.
Low dose ASA