Myelodysplastic Syndrome & Myeloproliferative Neoplasms Flashcards Preview

Lindsey's Blood and Lymph Unit III > Myelodysplastic Syndrome & Myeloproliferative Neoplasms > Flashcards

Flashcards in Myelodysplastic Syndrome & Myeloproliferative Neoplasms Deck (93)
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51

What does RC-UD stand for?

refractory cytopenia with unilineage dysplasia

53

Who gets MPNs?

adults 50-70yo

54

What is the later stage of PV called, and what characterizes it?

spent phase; prominent fibrosis of the marrow and falling peripheral blood cell counts

56

What does high grade MDS mean?

myeloblasts account for >5% of marrow cells and/or more than 2% of peripheral blood cells

56

What is the prognosis of RC-MD? Survival rate? Transformation?

poor- median survival 2.5 years- 10% transformation to AML

58

What is RC-MD?

low grade MDS with dysplasia in 2 or more lineages

59

In the ____ stage of PMF, the marrow is hypercellular, showing a granulocytic and megakaryocytic proliferation.

prefibrotic

61

What is the prognosis for refractory anemia with excess blasts-2 (RAEB-2)? Median survival? Transformation?

poor; 9 mos; 33% of patients transform to AML

62

What are the treatments of PV?

serial phlebotomy; aspirin; mild chemo

64

What is MDS with isolated deletion 5q?

low grade MDS assoc. with anemia, increased platelets, and megakaryocytes with small, round, non-lobated nuclei

65

What is refractory anemia with excess blasts-1 (RAEB-1)?

5-9% blasts in the marrow or 2-4% blasts in the peripheral blood

67

Nearly all cases of PV have a mutation of the _____ gene that encodes a _____.

JAK2; JAK2 cell signaling protein

69

What is the intermediate phase of CML?

the accelerated phase

70

What are the 4 main types of MPNs?

1. chronic myelogenous leukemia (CML) 2. polycythemia vera (PV) 3. primary myelofibrois (PMF) 4. essential thrombocytopenia (ET)

71

What are the 3 types of low grade MDS?

1. refractory cytopenia with unilineage dysplasia (RC-UD) 2. refactory cytopenia with multilineage dysplasia (RC-MD) 3. MDS with isolated deletion 5q

73

What does ET stand for?

essential thrombocytopenia

74

What does RC-MD stand for?

refactory cytopenia with multilineage dysplasia

75

What does MPN stand for?

myeloproliferative neoplasms

76

What does dyserythropoeisis look like?

RBC precursors with nuclear budding, irregular shaped nuclei, lack of coordinated nuclear/cytoplasmic maturity, increased ring sideroblasts

77

What is refractory anemia with excess blasts-2 (RAEB-2)?

10-19% blasts in the marrow or 5-19% blasts in the peripheral blood

78

What are dacrocytes?

tear drop RBCs

80

What are the s/s of CML?

fatigue, weight loss, night sweats, splenomegaly, anemia

81

What is the prognosis of RC-UD?

relatively good

82

What are the s/s of ET?

TIAs; digital ischemia with parasthesias; thrombosis of major BVs

84

What does low grade MDS mean?

myeloblasts account for

86

What is the treatment for MDS?

stem cell transplant, supportive care, transfusions

87

What is the prognosis of PV? Survival?

very good- 10-20 years

88

What is MDS?

hematopoietic stem cell disorder characterized by ineffective hematopoiesis and increased risk of transformation to acute myeloid leukemia (AML)

89

____ should always be considered in a pt with a thrombosis of the mesenteric, portal, or splenic vein.

PV

90

MPNs are usually associated with _____ that encode cytoplasmic or receptor protein tyrosine kinase (PTKs).

abnormalities of genes (translocations, point mutations)