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Hematology > Malignant Hematology - Myeloid Disorders > Flashcards

Malignant Hematology - Myeloid Disorders Flashcards

1
Q

Which blood cells are considered granulocytes?

A

Eosinophils
Basophils
Neutrophils

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2
Q

What are the myeloproliferative neoplasms?

A

Polycythemia Vera
Essential Thrombocytosis
Chronic Myelogenous Leukemia (CML)

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3
Q

What are the 4 types of myeloid disorders?

A

(1) Acute Myeloid Leukemia (AML)
(2) Myeloproliferative Neoplasms (MPNs)
(3) Myelodysplastic Syndrome (MDS)
(4) Chronic Myelomonocytic Leukemia (CMML)

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4
Q

What are the 3 types of lymphoid disorders?

A

(1) Acute Lymphocytic Leukemia (ALL)
(2) Lymphoproliferative Disorders (Chronic Lymphoid Leukemia & Lymphoma)
(3) Plasma Cell Dyscrasias (MGUS, MM, primary amyloidosis, Waldenstrom Macroglobulinemia/Lymphoplasmacytic lymphoma)

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5
Q

What should you be most worried about if you see Auer rods on a peripheral blood smear?

A

Acute Myeloid Leukemia

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6
Q

How is a diagnosis of AML made?

A

At least 20% blasts in peripheral blood smear or on bone marrow biopsy.

*Excepts exists, such as in APL, where a PML-RARA mutation is diagnostic of leukemia.

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7
Q

What causes the early mortality in acute promyelocytic leukemia?

A

Due to disseminated intravascular coagulation, resulting in:

(1) Intracranial Hemorrhage
(2) Blood Clots
(3) GI Bleed

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8
Q

What is the urgent treatment needed in acute promyelocytic leukemia?

A

ATRA (all-trans retinoic acid)

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9
Q

What is the mutation in acute promyelocytic leukemia?

A

T(15;17) PML-RARA

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10
Q

How do you treat leukostasis in leukemia (4)?

A

(1) Intravenous fluids
(2) Cytoreduction
(3) TLS prophylaxis
(4) AVOID transfusion

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11
Q

What is the management of tumor lysis syndrome?

A

(1) Correct hyperK and extended lytes
(2) IVF to target high urine output
EITHER:
(A) Allopurinol
(B) Rasburicase (if AKI, uric acid > 535, or no response to allopurinol)

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12
Q

What is the target transfusion threshold for thrombocytopenia in acute leukemia who are not bleeding?

A

Non-APL —> Platelets < 10

APL — Platelets < 30

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13
Q

When would you transfuse cryoprecipitate or fibrinogen in acute leukemia?

A

(A) Non-APL — If bleeding and fibrinogen < 1.5

(B) APL — If fibrinogen < 1.5

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14
Q

When would you transfuse FFP in acute leukemia?

A

If bleeding and PT or PTT > 1.5 ULN

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15
Q

How is APL treated?

A

ATRA + arsenic +/- chemotherapy

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16
Q

How is ALL treated (broad terms)?

A

(1) Induction
(2) Consolidation/Intensification
(3) Maintenance

*High risk of CNS involvement so intrathecal chemotherapy +/- radiotherapy used

17
Q

In which myeloproliferative neoplasms do you get a JAK2 mutation?

A

(1) Polycythemia Vera (> 95%)
(2) Essential Thrombocytosis (~50%)
(3) Primary Myelofibrosis

18
Q

What is the mutation in chronic myeloid leukemia (CML)?

A

T(9;22) BCR-ABL tyrosine kinase +

AKA Philadelphia-chromosome +

19
Q

What are the features of chronic phase chronic myeloid leukemia (5)?

A

(1) Elevated WBCs (mostly neutrophils and precursors)
(2) Splenomegaly
(3) Constitutional Symptoms
(4) Bone marrow + peripheral blasts < 10%
(5) Left shift + basophils + eosinophils in the peripheral blood.

20
Q

What it’s the treatment for chronic myeloid leukemia?

A

Tyrosine Kinase Inhibitors (Imantinib)

21
Q

What are the phases of chronic myeloid leukemia?

A

(1) Chronic Phase (< 10% blasts on peripheral smear or bone marrow)
(2) Accelerated Phase (10-19% blasts on peripheral smear or bone marrow)
(3) Blast Phase (>20% blasts on peripheral smear or bone marrrow)

22
Q

What are the features of accelerated phase chronic myeloid leukemia?

A

(1) 10-19% blasts in the peripheral blood or on bone marrow biopsy.
(2) Worsening counts DESPITE therapy (WBC > 10, platelets > 1000 or < 100)

23
Q

What is the blast phase of chronic myeloid leukemia?

A

Acute leukemia — typically AML, but 20-30% ALL

At least 20% blasts on peripheral smear or bone marrow biopsy.

24
Q

How do you treat the blast phase of chronic myeloid leukemia?

A

Treatment as per acute leukemia + tyrosine kinase inhibitor

25
Q

In what malignant hematologist neoplasm would you classically see erythromelagia and aquagenic pruritis?

A

Polycythemia Vera

26
Q

How should you diagnosis polycythemia vera?

A

Diagnosis requires 3 major or 2 major and 1 minor criteria:

Major:

(1) Hb > 165/160 (M/F) or HVT > 49/48 (M/F)
(2) BM: Hypercellular for age, trilineage growth
(3) JAK2 mutation

Minor:
Low serum EPO

27
Q

How do you risk stratify patient with polycythemia vera and essential thrombocytosis for thrombosis?

A

LOW RISK = Age < 60 & no thrombosis hx

HIGH RISK = Age > or = 60 OR thrombosis hx

High risk get treated with hydroxyurea

28
Q

What do all patients with Polycythemia Vera & Essential Thrombocytosis get treated with?

A

ASA

29
Q

Why do patients with essential thrombocytosis bleed when their platelets are > 1000?

A

Acquired VWD

30
Q

What are the diagnostic criteria for essential thrombocytosis?

A

Diagnosis requires 4 major, or the first 3 major + 1 minor:

Major:

(1) Sustained platelets > or = 450
(2) BMBx: Hyperlobulated mature megakaryocytes.
(3) R/O other WHO diseases
(4) JAK, CALR or MPL mutation.

Minor
No reactive cause

31
Q

What is the JAK-2 inhibitor that can be used in myelofibrosis or polycythemia vera?

A

Ruxolitinib

32
Q

What are the diagnostic criteria for myelofibrosis?

A

Requires ALL 3 Major + 1 Minor:

Major (All 3):

(1) BM: Fibrosis, megakaryocyte proliferation, atypia.
(2) JAK 2, CALR, or MPL mutation
(3) Rule out other MPN/MDS

Minor (1 of):

  1. Leukoerythroblastic
  2. LDH > ULN
  3. Anemia
  4. Leukocytosis > 11
  5. Palpable splenomegaly
33
Q

What are the peripheral blood smear characteristics of myelofibrosis?

A

Cytopenias
Leukoerythroblastic (nucleated RBCs + left shift)
Tear Drop RBCs

34
Q

What are the peripheral blood manifestations of myelodysplastic syndrome?

A

(1) Hypolobulated/hypogranulated neutrophils (Pelgeroid)
(2) Macrocytosis
(3) Decreased reticulocytes

35
Q

How is a diagnosis of myelodysplastic syndrome made?

A

Bone marrow biopsy with aspirate and cytogenetics.

Bone marrow features:

(1) Hypercellular (usually) +/- ring sideroblasts
(2) Dysplasia > 10%
(3) Blasts < 20%

36
Q

What is the treatment for patients with MDS?

A

(A) Low Risk (based on BM workup): Supportive (EPO, transfusions) & lenalidomide

(B) High Risk:

  • Supportive, as above
  • Azacitadine (prolongs survival)
  • Cure: Allogenic HSCT
37
Q

What is the differential diagnosis for MDS?

A

(1) Nutritional Deficiency (Folate/B12/Copper)
(2) HIV
(3) EtOH
(4) Zinc Toxicity
(5) Meds - MTX, Azathioprine

38
Q

What are the general criteria for a diagnosis of chronic myelomonocytic leukemia?

A

(1) Peripheral blood monocytes > 1 & > 10% of total WBCs
(2) Blasts < 20% (if > 20 = acute leukemia)
(3) Dysplasia in 1 or more myeloid lineage
(4) Exclusion of other MPN

39
Q

What is the prognosis of CMML for progression to AML?

A

15-20% get AML

Hematology (9 decks)

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