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MHD VI: Pulm > 11.18: Hemo IV > Flashcards

11.18: Hemo IV Flashcards

1
Q

Another name for the chronic myeloid disorders?

A
  • Myeloproliferative disorders
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2
Q

4 major myeloproliferative disorders?

A

“MPDs”

  1. CML: “Chronic myelogenous leukemia”: WBCs
  2. Polycythemia vera: RBCs
  3. Primary myelofibrosis
  4. “ET” Essential thrombocytosis: platelets
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3
Q

Commonalities in myeloproliferative disorders?

A
  1. Target cell is multipotent myeloid progenitor cell
  2. Differ from AML in that cells can continue to differentiate beyond blast stage
  3. Increased marrow cellularity and hematopoiesis w/ increased peripheral blood cell counts
  4. Splenomegaly
  5. Terminate in spent phase with marrow fibrosis and peripheral cytopenias
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4
Q

What is unique about CML?

A
  • Pluripotent stem cell that can give rise to both lymphoid and myeloid cells is target
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5
Q

What can MPDs progress to?

A

Leukemia

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6
Q

Common pathogenic feature in all MPDs?

A
  • Mutated constitutively active tyrosine kinase leading to growth factor independent proliferation
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7
Q

What is the mutation in CML?

A
  • BCR/ABL fusion gene
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8
Q

What is mutation in polycythemia vera?

A

JAK2 point mutations

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9
Q

What is the mutation in ET and primary myelofibrosis?

A

JAK2, CALR and MPL point mutations

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10
Q

What does mutation in BCR/ABL fusion gene cause?

A

CML

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11
Q

What does JAK2 point mutations cause?

A

Polycythemia vera

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12
Q

What does JAK2, CALR and MPL point mutations cause?

A

ET

Primary myelofibrosis

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13
Q

Distinguishing factor in CML?

A
  • Observation of Philadelphia chromosome via karyotyping in 90% of patients
  • Mutation can be seen in myeloid stem cells as well as B and T cells
  • Hints that abnormality occurs in pluripotent stem cells
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14
Q

What is the Philadelphia chromosome?

A
  • Reciprocal translocation between ABL gene on chromosome 9 and BCR gene on chromosome 22
  • Encodes for synthesis of protein with tyrosine kinase activity
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15
Q

How can you treat CML?

A

Tyrosine kinase inhibitors

- Gleevec/imatinib

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16
Q

What is see in CML marrow?

A
  • Marked increase in neoplastic granulocytic precursors in marrow
  • Bone marrow is 100% cellular w/ increased megakaryocytes
17
Q

What is seen in CML periphery?

A
  • Leukocytosis with shift to immaturity
  • Thrombocytosis
  • Basophilia
  • Small number of circulating blasts
18
Q

What is marrow cellularity of 100% characteristic of?

A

CML

19
Q

What is the following indicative of:

  • Leukocytosis with shift to immaturity
  • Basophilia
  • Thrombocytosis
  • Small number of circulating blasts
A

CML

20
Q

How to distinguish CML from leukemoid rxn?

A

CML has the following:

  1. WBC > 50k in all stages of maturation
  2. Reduced LAP score
  3. M:E ratio > 10 in marrow
  4. Splenomegaly
  5. 9:22 BCR/ABL
21
Q

What can original presentation of CML be?

A

Abdominal discomfort from splenomegaly

22
Q

What is accelerated phase of CML?

A
  • Unresponsive to treatment
  • Increased symptoms
  • Becomes blast phase which looks like AML
23
Q

What is relative polycythemia?

A

Occurs from dehydration or decreased plasma volume

24
Q

What is absolute polycythemia?

A

From increase in total red cell mass

25
Q

What occurs in PV?

A
  • Erythroid precursors dominate leading to absolute increase in red cell mass causing most of clinical symptoms
26
Q

Pathology of PV?

A
  • JAK2 mutation making cells independent of EPO
  • EPO levels will be drastically low
  • High hematocrit and increased BV create abnormal flow leading to distension and stasis
27
Q

Bone marrow in PV?

A
  • Hypercellular with proliferation of erythroid forms
28
Q

What will PV cause?

A
  • Thrombotic episodes can cause MI, DVT, stroke
  • Hemorrhages
  • Cyanosis
  • Htn. can occur
  • Pletoric
29
Q

What does pletoric mean?

A

Reddish complexion seen in ptn with PV

30
Q

PV treatment?

A
  • Phlebotomies: blood collected and discarded
31
Q

What occurs in primary myelofibrosis?

A
  • Early progression to obliterating marrow fibrosis
  • Caused by excessive collagen deposition
  • Bones can become thick
  • Spleen and liver conduct extramedullary hematopoiesis
  • Tear drop cells in in peripheral smear
32
Q

What are tear drop cells in peripheral smear indicative of?

A

Primary myelofibrosis

33
Q

How to diagnose primary myelofibrosis?

A

HYPERcellular bone marrow biopsy

- With sclerotic bones

34
Q

What occurs in essential thrombocytosis?

A
  • Increased platelet count from increased megakaryocytes
35
Q

What does smear show in essential thrombocytosis?

A

Abnormally large platelets

36
Q

Treatment of essential thrombocytosis?

A

Aspirin

MHD VI: Pulm (20 decks)

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