MYELOPROLIFERATIVE NEOPLASMS

Question 1

Caused by genetic mutations in the stem cell that causes excessive production and accumulation of RBCs, granulocytes and platelets in the Bone Marrow, Blood and tissues

Answer 1

MYELOPROLIFERATIVE NEOPLASMS

Question 2

Philadelphia chromosome t(9;22)

Answer 2

CML

Question 3

JAK2 gene mutation (90-95% of cases)

Answer 3

PV

Question 4

JAK2 gene mutation (50% of cases)

Answer 4

ET

Question 5

JAK2 gene mutation (50% of cases)

Answer 5

PMF

Question 6

Arises from a single genetic translocation in a pluripotential HSC producing a clonal overproduction of the myeloid cell line

Answer 6

CHRONIC MYELOGENOUS LEUKEMIA

Question 7

Associated with Philadelphia Chromosome

Answer 7

CHRONIC MYELOGENOUS LEUKEMIA

Question 8

Translocation between the long arms of Chromosomes 9;22 (_______)

Answer 8

Philadelphia Chromosome

Question 9

Lab findings:
Increased Total WBC Count (>50x10^9/L)
- increased in mature forms of granulocytes – Neutrophils, Eosinophils, Basophils
- Normal or increased platelet count
- Normal or decreased RBCs

Answer 9

CHRONIC MYELOGENOUS LEUKEMIA

Question 10

Differential Diagnosis:
Must be differentiated from Leukemoid reactions thru NAP cytochemical staining

Answer 10

CHRONIC MYELOGENOUS LEUKEMIA

Question 11

NAP score for CML

Answer 11

Lower

Question 12

Increased or Normal NAP score

Answer 12

Leukomoid reaction

Question 13

Differential Diagnosis:
________ transplantation has been successful, and ________ ( tyrosine kinase inhibitor) produces remission in most cases of CML

Answer 13

Bone Marrow
imatinib mesylate

Question 14

The neutrophil Alkaline phosphatase (NAP) is greatly ________ or ________ in >90% of patients with CML

Answer 14

reduced
absent

Question 15

NAP
Spx:

Answer 15

Blood film

Question 16

NAP
Dye:

Answer 16

Diazo (alkaline pH)

Question 17

NAP
Product:

Answer 17

insoluble blue compound

Question 18

NAP
Evaluated 100 neutrophils
Grading: 0

Answer 18

no staining

Question 19

NAP
Evaluated 100 neutrophils
Grading: 1+

Answer 19

faint/diffuse

Question 20

NAP
Evaluated 100 neutrophils
Grading: 2+

Answer 20

pale/moderate

Question 21

NAP
Evaluated 100 neutrophils
Grading: 3+

Answer 21

strong blue ppt

Question 22

NAP
Evaluated 100 neutrophils
Grading: 4+

Answer 22

deep blue without visible cytoplasm

Question 23

Reference Interval for NAP:

Answer 23

15-170

Question 24

• Characterized by excessive proliferation of erythroid, granulocytic and megakaryocytic elements in the bone marrow (panmyelosis).
• Splenomegaly is usually present

Answer 24

POLYCYTHEMIA VERA

Question 25

WHO criteria for diagnosis for PV: - _______ Hemoglobin, Hematocrit, or red cell mass - ________ Bone Marrow with trilineage growth (________) - Identification of the________ mutation or the ________ mutation - _____ EPO levels

Answer 25

Increased Hypercellular panmyelosis JAK2 V167F JAK2 exon 12 Low

Question 26

WHO criteria for diagnosis for PV: (Ref. range) Men: Hgb > ____ g/dL and Hct > % Women: Hgb > ____ g/dL and Hct > ____ %

Answer 26

16.5 and 49 16 and 48

Question 27

Treatment for PV:

Answer 27

- Therapeutic phlebotomy - Alkylating agent hydroxyurea - JAK inhibitor (eg. Ruxolitinib)

Question 28

Clonal MPN with increased megakaryopoiesis and thrombocytosis

Answer 28

ESSENTIAL THROMBOCYTHEMIA

Question 29

ESSENTIAL THROMBOCYTHEMIA Clinical Manifestations: - ________ (thromboses in major arteries and veins) - _______ resulting to atrophy - Pulmonary ______ and neurologic complications - Bleeding in mucous membranes

Answer 29

Vascular Occlusions Splenic infarcts emboli

Question 30

ESSENTIAL THROMBOCYTHEMIA Lab Findings: - Thrombocytosis (>400 x 109/L) - Platelets often appear normal, but there could be presence of _____ bizarre platelets, platelet aggregates, micro megakaryocytes, and megakaryocyte fragments - _______ is usually present and ranges from 22-40x10^9/L - Segmented neutrophils may be _______

Answer 30

giant Leukocytosis increased

Question 31

Treatment for ET:

Answer 31

Plateletpheresis Hydroxyurea therapy Low-dose aspirin JAK2 inhibitors

Question 32

It is the least common but most aggressive form of MPN

Answer 32

PRIMARY MYELOFIBROSIS

Question 33

Chronic, progressive clonal panmyelosis characterized by megakaryocytic and often granulocytic hyperplasia with varying degrees of reactive fibrosis of the marrow & extramedullary hematopoiesis

Answer 33

PRIMARY MYELOFIBROSIS

Question 34

Splenomegaly is present in 90% of patients with PMF and 50% have hepatomegaly

Answer 34

PRIMARY MYELOFIBROSIS

Question 35

PRIMARY MYELOFIBROSIS Bone Marrow Findings: - ______ (megakaryocytic and often granulocytic hyperplasia) - Varying degrees of ________ - Collagen _________ disrupting the normal architecture of the Bone Marrow - It is usually impossible to aspirate marrow, and ______ is necessary for examination

Answer 35

Panmelosis Reactive fibrosis overproduction biopsy

Question 36

PRIMARY MYELOFIBROSIS CBC Findings: - ________ anemia - anisocytosis - poikilocytosis - _______ resulting from fibrosis of Bone Marrow

Answer 36

Normocytic Pancytopenia

Question 37

Treatment for PM:

Answer 37

transfusions hydroxyurea interferon-y busulfan androgens erythropoietin JAK2 inhibitors

Question 38

Historically referred to as refractory anemia, smoldering leukemia, oligoblastic leukemia, preleukemia

Answer 38

MYELODYSPLASTIC SYNDROME (MDS)

Question 39

MDS The median age of diagnosis is ___ years old

Answer 39

76

Question 40

MYELODYSPLASTIC SYNDROME (MDS) Group of acquired clonal hematologic disorder characterized by: → Progressive _______ → (DDD)

Answer 40

Cytopenia Dyserythropoiesis Dysmyelopoiesis Dysmegakaryopoiesis

Question 41

FAB classification of MDS: Refractory anemia Refractory anemia with ______ Refractory anemia with _____ Chronic __________ leukemia Refractory anemia with excess _____ in transformation (RAEB-t)

Answer 41

Ring Sideroblasts Excess Blasts myelomonocytic blasts