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Heme Path > Myeloproliferative Disorders > Flashcards

Myeloproliferative Disorders Flashcards

1
Q

Hypercellular marrow with abnormal megakaryocytes early on

A

Primary Myelofibrosis

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2
Q

Appearance of a “spent” phase with fibrosis and cytopenias

A

myeloproliferative disorders

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3
Q

Progress to a “spent” phase‐later in the disease course • Extreme marrow fibrosis • Extramedullary hematopoiesis (liver , spleen )‐ organomegaly

A

Polycythemia vera

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4
Q

Tyrosine kinase induces signaling via (1) pathways

A

RAS and JAK/STAT

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5
Q

Bone marrow cells mature via growth factors that bind receptors on progenitor cells which activate (1) which activate pathways for cell growth and survival

A
  1. tyrosine kinases
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6
Q

What are the clinical signs of polycythemia vera?

A

Plethoric, cyanosis, headache, dizziness, HTN, pruritis at night, hyperuricemia

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7
Q

Symptomatic hyperuricemia‐ increased cell turnover

A

Polycythemia vera and primary myelofibrosis

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8
Q

What is the most characteristic symptom of polycythemia vera? What causes it?

A

Pruritus at night due to basophils releasing histamine

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9
Q

Inappropriate release of fibrogenic factors from neoplastic megakaryocytes

A

Primary Myelofibrosis Platelet derived growth factor and TGF‐beta

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10
Q

CML has markedly hypercellular bone marrow containing hyperplasia of what cell types?

A

myelocytes metamyelocytes bands eosinophils basophils megakaryocytes

but not erythrocytes

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11
Q

How is CML diagnosed?

A

High WBC, peripheral smear and bone marrow findings BCR/ABL fusion detected –chromosome analysis or by PCR

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12
Q

Death within months if untreated due to bleeding or thrombosis

A

Polycythemia vera

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13
Q

70% of blast crises are (1) leukemia

A

acute myeloid

refers to CML

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14
Q

How do you distinguish polycythemia vera from secondary polycythemia?

A

P. vera high oxygen saturation and low erythropoetin Secondary polycythemia low oxygen saturation and high erythropoetin

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15
Q

participates in JAK.STAT pathway downstream from hematopoietic growth factors

A

JAK2

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16
Q

Mutated (1) lead to growth factor independent proliferation and survival of (2)

A
  1. tyrosine kinases 2. marrow progenitors refers to myeloproliferative disorders
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17
Q

Major bleeding and thrombotic events occur

A

Polycythemia vera

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18
Q

Absence of fibrosis

A

essential thrombocytosis

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19
Q

mutations in essential thrombocytosis

A

JAK2 activating mutations MPL mutation thrombopoietin activated protein kinase Calreticulin mutation‐functions in the endoplasmic reticulum

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20
Q

Features of Primary Myelofibrosis

A

Pancytopenia • Extramedullary hematopoiesis

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21
Q

presence of a chimeric BCR‐ABL fusion gene coding an active BCR‐ABL tyrosine kinase resulting in?

A

growth factor independent proliferation and survival of marrow progenitors refers to CML (Chronic myelogenous leukemia)

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22
Q

Iron deficiency from bleeds has a beneficial effect

A

Polycythemia vera

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23
Q

CML peripheral blood findings

A

Leukocytosis (high WBC count) Entire spectrum of marrow cells present Basophilia Blast count < 10%

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24
Q

Osteosclerosis replaces the marrow spaces

A

Primary Myelofibrosis

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25
Q

Mutations originate in (1) level or (2) level

A
  1. multipotential myeloid progenitor 2. pluri‐potential stem cell refers to myeloproliferative disorders
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26
Q

Transforms to AML‐up to 20% of cases

A

Primary Myelofibrosis

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27
Q

Increased HCT, blood viscosity,abnormal platelet function

A

Polycythemia vera

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28
Q

How is CML treated?

A

Hematopoietic stem cell transplant for young stable patients Therapy with BCR/ABL tyrosine kinase inhibitors

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29
Q

Obliterative bone marrow fibrosis in primary myelofibrosis leads to?

A

pancytopenia and extra medullary hematopoesis

30
Q

6mos or so‐enters “blast crisis” acute leukemia; 50% enter blast crisis without the accelerated period

A

CML

31
Q

In myeloproliferative disorders, (1) is not impaired and increased numbers of (2) are therefore produced

A
  1. differentiation 2. mature blood elements
32
Q

Identical to “spent “phase of other myeloproliferative disorders

A

Primary Myelofibrosis

33
Q

In essential thrombocytosis progenitors undergo (1) independent‐proliferation

A

thrombopoietin

34
Q

Pancytopenia

A

Primary Myelofibrosis

35
Q

BCR gene –chromosome ?

A

22

36
Q

Slow progression (3 yr. survival even if untreated) Followed by “accelerated phase”‐increasing anemia and thrombocytopenia

A

CML

37
Q

major clinical manifestations of essential thrombocytosis

A

Thrombosis and hemorrhage from defective platelet function • DVT • Portal and hepatic vein thrombosis • MI; throbbing/burning sensation of hands and feet

38
Q

Leukoerythroblastosis (myelocytes, metamyelocytes and early erythroid forms in peripheral blood)

A

Primary Myelofibrosis AND infiltrative marrow processes such as metastatic tumor

39
Q

How does CML present?

A

Hypermetabolism resulting in fatigue, weakness, weight loss, anorexia Dragging sensation or pain from splenic infarcts

40
Q

Hyper cellular marrow of all cell lines but mostly red cell series; basophilia

A

Polycythemia vera

41
Q

Reciprocal translocation Philadelphia chromosome (9;22)(q34;q11)

A

CML (Chronic myelogenous leukemia)

42
Q

Marked megakaryocyte increase with bizarre abnormal forms

A

essential thrombocytosis

43
Q

Infections; Bleeding and thrombotic episodes

A

Primary Myelofibrosis

44
Q

Mild hepatosplenomegaly‐extramedullary hematopoiesis‐50%

A

essential thrombocytosis

45
Q

throbbing/burning sensation of hands and feet

A

Erythromelalgia; due to platelet aggregates occurs in essential thrombocytosis

46
Q

Treatment for polycythemia vera

A

Simple regular phlebotomy and low dose aspirin

47
Q

(1) to (2) substitution at residue 617

A
  1. Valine 2. phenylalanine refers to Polycythemia vera
48
Q

Large platelets in peripheral blood

A

essential thrombocytosis

49
Q

thrombopoietin activated protein kinase

A

MPL - mutated in essential thrombocytosis and primary myelofibrosis

50
Q

Primary Myelofibrosis mutations

A

Activating JAK2 mutations‐50% • MPL mutations

51
Q

Median survival‐up to 15 yrs with long asymptomatic periods

A

essential thrombocytosis

52
Q

Variable transformation to (1) in myeloproliferative disorders

A

acute leukemias

53
Q

Progressive replacement of marrow with (1)

A
  1. collagen Primary Myelofibrosis
54
Q

Primary Myelofibrosis treatment

A

JAK2 inhibitors can decrease the splenomegaly somewhat Hematopoietic stem cell transplant for younger pts

55
Q

essential thrombocytosis elevated (1) without (2)

A
  1. platelet counts 2. polycythemia or marrow fibrosis
56
Q

panmyelosis of proliferation red cells, white cells, and platelets

A

Polycythemia vera

57
Q

functions to promote cell proliferation and manufacturing in the ER

A

Calreticulin - mutated in essential thrombocytosis

58
Q

S/S of Primary Myelofibrosis

A

Dragging sensation –massively enlarged spleen Fatigue, weight loss, night sweats Hyperuricemia‐increased cell turnover

59
Q

Strong association with activating point mutations in JAK/2 TYROSINE KINASE

A

Polycythemia vera, essential thrombocytosis, primary myelofibrosis

60
Q

obliterative marrow fibrosis

A

Primary Myelofibrosis

61
Q

ABL gene‐chromosome ?

A

9

62
Q

1% transform to AML

A

Polycythemia vera

63
Q

Dacrocytes (Tear‐drop Rbc’s)

A

Primary Myelofibrosis

64
Q

Progressive marrow fibrosis and hypo cellularity and atypical megakaryocytes

A

Primary Myelofibrosis

65
Q

Mild organomegaly; Minimal extramedullary hematopoiesis

A

Polycythemia vera

66
Q

Other CML findings

A

splenomegaly and hepatomegaly, splenic infarcts

67
Q

mild organomegaly is seen in which conditions?

A

polycthemia vera and essential thrombocytosis

68
Q

dragging sensation or pain from splenic infarcts is seen in which myeloproliferative disorders?

A

CML and primary myelofibrosis

69
Q

High WBC count

A

CML

70
Q

Very high hematocrit

A

polycythemia vera

71
Q

elevated platelet counts

A

essential thrombocytosis

Heme Path (21 decks)

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