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Year 2 Hematology > Myelodysplasia > Flashcards

Myelodysplasia Flashcards

1
Q

what is the clinical phenotype of MDS?

A

peripheral cytopenias in the setting of a normocellular or hypercellular bone marrow

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2
Q

the vast majority of MDS cases are caused by what?

A

de novo - exact cause is unclear

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3
Q

what are the symptoms of MDS?

A
  1. anemia
  2. infections
  3. bleeding problems
  4. organomegaly / lymphadenopathy
  5. sweet’s syndrome
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4
Q

what is sweet’s syndrome?

A
  1. acute neutrophilic dermatosis and pyoderma gangrenosum
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5
Q

what is the gold standard test for MDS?

A

bone marrow biopsy

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6
Q

which has a shorter latency period for development of MDS / AML - aklylating agents or topoisomerase inhibitors?

A

topoisomerase inhibitors

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7
Q

what is the typical demise for a patient with high risk MDS?

A

infection

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8
Q

what are the features of 5q syndrome?

A
  1. female predominance
  2. median age 68 years
  3. macrocytic anemia, leukopenia, platelets increased
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9
Q

what is the treatment for 5q syndrome?

A

lenalidomide

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10
Q

what is CMML?

A

chronic myelomonocytic leukemia - a form of MDS

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11
Q

what are the features of CMML?

A
  1. increase in monocytes in BM and peripheral blood
  2. variable degrees of dysplasia
  3. can have response to imatinib - otherwise use azacytidine
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12
Q

hypocellular MDS resembles what other condition?

A

aplastic anemia

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13
Q

what is the low intensity treatment for MDS?

A
  1. supportive
  2. transfusion
  3. growth factors
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14
Q

what is the high intensity treatment for MDS?

A
  1. stem cell transplant
  2. chemotherapy
  3. clinical trials of novel therapy
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15
Q

what is the treatment for MDS outside of 5q and CMML?

A

azacytidine

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16
Q

what is the only chance at a cure for MDS?

A

allogeneic transplant

17
Q

what are the classic myeloproliferative disorders?

A
  1. CML
  2. polycythemia vera
  3. essential thrombocytosis
  4. primary myelofibrosis
18
Q

the majority of myeloproliferative disorders will have what mutation?

A

JAK2

19
Q

what are the symptoms of CML?

A
  1. fatigue, night sweats, weight loss
  2. splenomegaly
  3. anemia / platelet dysfunction
  4. hyperleukocytosis (over 100,000)
20
Q

what are the lab findings of CML?

A
  1. neutrophilia and immature circulating myeloid cells
  2. absolute basophilia
  3. elevated LDH, uric acid
21
Q

what are the three phases of CML?

A
  1. chronic
  2. accelerated
  3. blast
22
Q

what are the features of chronic phase CML?

A
  1. blood and bone marrow blasts less than 10%

2. BCR-ABL rearrangement

23
Q

what are the features of accelerated phase CML?

A
  1. blood or bone marrow blasts over 10%

2. progressive splenomegaly, weight loss, fevers, bone pain

24
Q

what are the features of blast phase CML?

A

blood or bone marrow blasts over 20%

25
Q

what is the diagnosis for CML?

A
  1. BCR-ABL mutation

2. confirm with FISH or RT-PCR

26
Q

what is the treatment for CML?

A

imatinib

27
Q

definition: polycythemia vera

A

elevated RBC mass in absence of secondary conditions

28
Q

what is the difference between polycythemia vera and essential thrombocytosis in terms of diagnosis?

A

polycythemia vera will have an elevation in all of the cell lines

29
Q

what are the symptoms of polycythemia vera?

A

itching, erythtomelagia, venous / arterial thrombosis

30
Q

what are the signs of polycythemia vera?

A
  1. hypertension
  2. splenomegaly
  3. hepatomegaly
31
Q

what are the major and minor diagnostic criteria for polycythemia vera? what constitutes an affirmative case?

A

major - Hb over 18.5 male, 16.5 female plus JAK2
minor - bone marrow with hypercellularity and trilineage growth, low serum EPO

REQUIREMENTS: both major and 1 minor OR 1st major with 2 minor

32
Q

what is the first line therapy for polycythemia vera?

A

phlebotomy

33
Q

what is the first line therapy for essential thrombocytosis?

A

hydrea, cytoreductive therapy

34
Q

what needs to be ruled out before making a final diagnosis of ET?

A

reactive thrombocytosis

35
Q

what are the hallmarks of primary myelofibrosis?

A

marrow fibrosis and extramedullary hematopoiesis

Year 2 Hematology (34 decks)

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