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Boards.CP.Heme > MDS, CMML, aCML, JMML > Flashcards

MDS, CMML, aCML, JMML Flashcards

1
Q

Myelodysplastic Syndromes (MDS) - General Features:

  • Signs/Symptoms (3)
  • Splenomegaly (+/-)
A
  • Fatigue, Infection, and/or Bleeding

- Splenomegaly ABSENT

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2
Q 
Myelodysplastic Syndromes (MDS) - Primary vs. Secondary:
-Population
A 
Primary MDS (Most Common):
-Older adults

Secondary MDS:
-Young adults

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3
Q

Secondary Myelodysplastic Syndrome (MDS):

-Causes (4)

A
  • Chemotherapy (Alkylating Agents)
  • Radiation
  • Benzenes
  • Fanconi Anemia
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4
Q

In Secondary MDS, Alkylating agents (chemotherapy) is often associated with genetic anomalies involving what chromosomes?

A

5q/7q

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5
Q

Myelodysplastic Syndromes (MDS) - Morphology:

  • BM cellularity
  • BM finding (2)
  • Blast %
A
  • Hypercellular BM
  • Abnormal localization of immature precursors
  • Dyspoiesis present in 1 or more cell lines
  • Blasts <20%
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6
Q

MDS - Erythroid Dyspoiesis:

-Peripheral Blood (4)

A
  • Anemia
  • Basophilic stippling
  • Poikilocytosis
  • Macrocytosis
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7
Q

MDS - Erythroid Dyspoiesis:

-Bone Marrow (7)

A
  • Megaloblastoid change/Nuclear Lobation
  • Internuclear Bridging
  • Multinuclearity
  • Karyorrhexis
  • Ringed-Sideroblasts
  • Cytoplasmic PAS+
  • Cytoplasmic Vacuolization
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8
Q

MDS - Erythroid Dyspoiesis:

-Functional (5)

A
  • Increased susceptibility to complement mediated lysis
  • Increased HbF
  • Abnormal RBC Ag expression
  • Enzyme defects (eg, PKD)
  • Thalassemia (acquired)
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9
Q

MDS - Granulocytic (Myeloid) Dyspoiesis:

-Peripheral Blood (3)

A
  • Neutropenia
  • Abnormal Granulation
  • Nuclear Segmentation (psuedo-Pelger-Huet anomaly)
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10
Q

MDS - Granulocytic (Myeloid) Dyspoiesis:

-Bone Marrow (2)

A
  • Megaloblastoid Maturation

- Abnormal Granulation

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11
Q

MDS - Granulocytic (Myeloid) Dyspoiesis:

-Functional (1)

A

-Increased susceptibility to Bacterial Infections

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12
Q

MDS - Megakaryocytic Dyspoiesis:

-Peripheral Blood (3)

A
  • Thrombocytopenia
  • Variable size
  • Variable granulation
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13
Q

MDS - Megakaryocytic Dyspoiesis:

-Bone Marrow (3)

A
  • Micromegakaryocytes
  • “Pawn Ball” (Multinucleated)
  • Hypolobated
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14
Q

MDS - Megakaryocytic Dyspoiesis:

-Functional (1)

A

-Abnormal Platelet Aggregometry

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15
Q

In MDS, dyspoeisis must be present in what percentage of a cell line for a diagnosis of dysplasia?

A

> 10%

*NOTE: Healthy marrow contains dyspoietic cells (<5%)

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16
Q

MDS - Molecular and Cytogenetics:

  • Most Common
  • 2nd
  • 3rd
A

MDS - Molecular and Cytogenetics:

  • Complex Karyotype - 2 or more clonal abnormalities (MC)
  • Isolated monosomy 7 or 7q- (2nd)
  • Isolated 5q (3rd)
17
Q

Which MDS Cytogenic abnormalities are associated with a Favorable prognosis? (4)

A 
-Normal Karyotype
Isolated loss of:
-Y
-5q-
-20q-
18
Q

Which MDS Cytogenic abnormalities are associated with a UNfavorable prognosis? (2)

A
  • Complex Karyotype (3 or more anomalies)

- Chr7 anomalies

19
Q

MDS - 5q- syndrome:

  • Population
  • Clinical course
  • Findings (3)
A

-Elderly Females
-Indolent clinical course
Findings:
-Anemia
-Normal/Elevated Platelets
-Micromegs in BM

20
Q

Myelodysplastic/Myeloproliferative Neoplasms (MD/MPN):

-Common features (2)

A
  • Dyspoiesis

- Increased production of Mature cells

21
Q

Myelodysplastic/Myeloproliferative Neoplasms (MD/MPN):

-Types (3)

A
  • Chronic Myelomonocytic Leukemia (CMML)
  • Atypical CML (aCML)
  • Juvenile Myelomonocytic Leukemia (JMML)
22
Q

Chronic Myelomonocytic Leukemia (CMML):

-Diagnostic criteria (4)

A
  • Persistent Absolute Monocytosis (>1x10^9/L)
  • Marrow dysplasia (Dysgranulopoiesis MC)
  • <20% blasts (includes Promonocytes)
  • Absence of Philadelphia Chromosome (9;22)
23
Q

Chronic Myelomonocytic Leukemia (CMML):

-Other Frequent findings (4)

A
  • Hepatosplenomegaly
  • Anemia
  • Thrombocytopenia
  • Abnormal Monocyte morphology
24
Q

Chronic Myelomonocytic Leukemia (CMML) Type 1 (CMML-1) - Blasts/Promonocytes:

  • Blood
  • Bone Marrow
A

<5% in Blood

<10% in BM

25
Q

Chronic Myelomonocytic Leukemia (CMML) Type 2 (CMML-2) - Blasts/Promonocytes:

  • Blood
  • Bone Marrow
A

5-19% in Blood

10-19% in BM

26
Q

Chronic Myelomonocytic Leukemia (CMML) - Molecular and Cytogenetics:

  • JAK2 mutation
  • If Eosinophilia is present
A

JAK2 mutation present in SMALL proportion of cases

Eosinophilia present:
-Rearrangement of PDGFRA and PDGFRB should be Excluded

27
Q

Atypical Chronic Myelogenous Leukemia (aCML):

-Diagnostic criteria (4)

A
  • Neutrophilia (Spectrum of Neuts/Metas/Myelos/Pros)
  • Marrow Dysplasia
  • <20% Blasts
  • Absence of Phila Chr (9:22)
28
Q

Most Atypical Chronic Myelogenous Leukemias (aCML) have cytogenetic anomalies, which are most common? (2)

A
  • Chr8+
  • del(20q)

*Some cases have JAK2 mutation

29
Q

Juvenile Myelomonocytic Leukemia (JMML):

-Findings (6)

A
  • Monocytosis/Granulocytosis
  • Hepatosplenomegaly
  • Constitutional Sxs
  • Anemia
  • Thrombocytopenia
  • Increased HbF
30
Q

Juvenile Myelomonocytic Leukemia (JMML):

-MC chromosomal abnormality

A

Monosomy 7 (25%)

31
Q

What is the confirmatory finding in Juvenile Myelomonocytic Leukemia (JMML)?

A

Spontaneous formation of granulocyte macrophage colonies (in vitro) that are hypersensitive to GM-CSF.

32
Q

Juvenile Myelomonocytic Leukemia (JMML):

-Nearly 10% of cases are associated with what?

A

Neurofibromatosis type 1 (NF1)

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