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Hematology 4 > MDS and MPN > Flashcards

MDS and MPN Flashcards

1
Q

What is MPN?

A

Proliferation of myeloid cells of abnormal growth.

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2
Q

What is CNL?

A

Elevation in mature neutrophils caused by a mutation of colony-stimulating factor 3 receptor

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3
Q

What are features of CMML?

A

Hypersegmentation of monocytes

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4
Q

What are features in the bone marrow of CNL?

A

hypercellular with increase granulocytic proliferation

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5
Q

What are the features of eosinophilic leukemia?

A

eosinophils with dysplastic changes
- elevated maturation of eosinophils

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6
Q

What is the difference between MPN and MDS.

A

MDS - cytosis
MDS - penia , dyplasia of abnormal development of cells

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7
Q

Which mutation is common in PV PMF and ET?

A

JAK2 V617F

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8
Q

What is the difference between relative polycythemia and absolute polycythemia?

A

Relative is not actually PV, increased RBC due to dehydration, vomiting or sport doping
Absolute PV is divided by primary and secondary, increase in RBC caused by true PV (Mutation, other diseases)

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9
Q

What are some symptoms and treatments for PV?

A

Symptoms:
- hepatosplenomegaly
- painful dilation of blood vessels
- increase blood in tissue
- ulceration and gangrene toes and fingers
Treatment:
- Therapeutic phlebotomy
- aspirin

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9
Q

What causes polycythemia vera (PV)>

A

defect in the pluripotential stem cell.

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9
Q

What are features of PV

A
  • increased RBC, WBC, PLT,
  • hypercellular BM
  • Fibrosis in BM
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10
Q

What is PMF

A

Primary myelofibrosis is replacement of normal BM elements with fibrotic connective tissue

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11
Q

What are key findings of PMF?

A
  • Dacrocytes: Extramedullalry hematopoiesis
  • Leukoerythroblastic
    -megathrombocytes
  • BM : dry tap
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12
Q

What are treatments for PMF?

A
  • Hydroxyurea
  • splenectomy
  • radiation
  • stem cell / BM transplant
  • Support anemia
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13
Q

What is essential thrombocythemia (ET)?

A

Increased proliferation of megakaryocytes in the marrow at the multipotential stem cell

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14
Q

What are symptoms of ET?

A
  • Thrombosis (TIA, stroke DVT)
15
Q

What are findings of ET?

A

Marked thrombocytosis , clusters of PLT in PBF and BM
BM:
- increased cellularity
- Giant PLT
- abnormal megakaryocytes

16
Q

What is the treatment of ET?

A
  • aspirins & anticoagulants
  • Plateletpheresis
  • BM transplant
17
Q

What is mastocytosis?

A

Neoplastic proliferation of mast cells in one or more organs.

18
Q

Which mutation is related to mastocytosis?

A

KIT mutation

19
Q

What are the two types of mastocytosis?

A

-Cutaneous mastocytosis (children)
- Systematic mastocytosis (adults)

20
Q

What is the difference between the two subtypes of mastocytosis?

A

Cutaneous: accumulation of mast cells in the skin
Systemic: accumulation of mast cells in bone marrow, lymph nodes, organs w/o skin involvement

21
Q

What is the difference in Mast cell leukemia and Mast cell sarcoma?

A

leukemia: >10% immature mast cells in PBF and >20% in BM
Sarcoma: solid mast tumor

22
Q

How is mastocytosis diagnosed?

A
  • BM biopsy
  • Skin biopsy
23
Q

What are treatments for mastocytosis?

A
  • antihistamines
  • chemotherapy
  • radiation
  • splenectomy
24
Q

What is myelodyplastic syndrome?

A
  • BM malignancy, poorly formed cells with increased marrow destruction.
25
Q

What are the two subtypes of MDS?

A
  • Primary: new case, unrelated to other treatment
  • Secondary : caused by prior therapy
26
Q

What are symptoms related to myelodysplastic syndrome?

A
  • fatigue
  • SOB
  • Pale
  • bruising / bleeding
  • infections
27
Q

what are features of MDS in terms of diagnosing it?

A
  • Leukoerythroblastic
  • megaloblastic
  • may have ringed sideroblast
    BM:
  • hypercellular + immature - pancytopenia
28
Q

How is MDS categorized?

A
  • how many immature and dysplastic cells are in BM
  • What cell line have low counts
  • ringed sideroblasts
  • quantity of blast cells
  • chromosome changes
29
Q

what terms are used in classification of MDS?

A
  • refractory anemia (RA)
  • refractory neutropenia (RN)
  • Refractory thrombocytopenia (RT)
30
Q

What dysplastic changes can be seen in the nucleus of cell in MDS?

A
  • multinuclear
  • disintegration of nucleus
  • hyper-segmentation
  • hypo-segmentation
  • nuclear budding
  • ringed sideroblast
31
Q

What cytoplasmic dysplastic changes can be seen in MDS?

A
  • abnormal vacuolation
  • Poor granulation (Hypo/hyper)
  • poor staining (Harlequin)
  • hypogranular thrombocytes

Hematology 4 (4 decks)

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