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USMLE Hematology Leukemmia and Lymphoma > Myeloproliferative Disorders > Flashcards

Myeloproliferative Disorders Flashcards

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USMLE® Step 1 flashcards
1
Q

What is the disease called when too many RBCs are produced?

A

Polycethemia Vera

- Type of Myeloproliferative disorder (granulocytes and platelets are also increased)

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2
Q

What is a proliferation of mature lymphoid cells (e.g Naive B cells, CD8or4 T cells) called?

A

CLL

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3
Q

What is a neoplastic proliferation of mature myeloid cells called?

A

Myeloproliferative disorders

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4
Q

What is a proliferation of granulocytes called? (type of myeloproliferative disorder)

A

CML (chronic myelogenous leukemia)

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5
Q

What is a proliferation of the megakaryocytes called?

A

Essential thrombocytopenia

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6
Q

What is a myeodysplastic syndrome result in?

A
  • High WBC count with hypercellular bone marrow

- All cells of myeloid lineage are increased but classified based on dominant myeloid cell produced

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7
Q

What are the complications of myelodysplastic syndrome?

A
  • Increased risk of hyperuricemia (gout)
  • Progression to marrow fibrosis
  • Transformation to acute leukemia
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8
Q

What is seen on blood smear in CML?

A

Increase in granulocytes

- Possible early precursor granulocytes

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9
Q

What does 9;22 translocation result in?

A
  • BCR-ABL fusion resulting in increased tyrosin kinase activity
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10
Q

What is the first line treatment of CML?

A

Imatinib

- Blocks tyrosine kinase activity

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11
Q

What are the 3 phases of CML?

A

Chronic, accelerated and transformation phase

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12
Q

What does splenomegaly suggest in CML?

A

Accelerated phase of disease

- Transformation to acute leukemia follows therafter

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13
Q

What does transformation of CML result in?

A

AML (2/3) or ALL (1/3)

- Mutation is in a pluripotent stem cell

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14
Q

How do you differentiate between CML and leukemoid reaction (e.g infection)?

A
  • CML granulocytes are LAP negative
  • CML is associated with increased basophils
  • CML granulocytes exhibit t(9;22)
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15
Q

What mutation is associated with polycythemia Vera?

A

JAK2 kinase mutation

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16
Q

What are the clinical symptoms of polycythemia Vera?

A
  • Blurry vision and headache
  • Increased risk of venous thrombosis
  • FLushed face due to congestion
  • Itching after bathing (Mast cells release hsitamine)
17
Q

What is the most common cause of Budd-chiari syndrome?

A

Polycythemia Vera

18
Q

What is the treatment of Polycythemia Vera?

A
  • Phlebotomy
  • 2nd line treatment is hydroxyurea
  • W/o treatment death occurs usually within 1 year
19
Q

How do you differentiate between Polycythemia Vera (PV) and reactive polycythemia?

A
  • In PV SaO2 is normal (unlike in lung disease where it is decreased)
  • PV has decreased EPO (unlike in ectopic EPO production)
20
Q

Essential Thrombocytopenia

A
  • Neoplastic proliferation of mature myeloid cells, especiallt platelets (many on blood smear)
  • RBCs and granulocytes also increased
  • Associated with JAK2 kinase mutation
21
Q

What other disease may have a lot of platelets on blood smear (not essential thrombocytopenia)?

A

Iron deficiency anemia

22
Q

What are the symptoms of essential thrombocytopenia?

A
  • Increased risk of bleeding (defective) and/or thrombosis
  • Rarely progress to marrow fibrosis or acute leukemia
  • No significant risk for hyperuricemia or gout
23
Q

What is myelofibrosis?

A
  • Neoplastic proliferation of mature myeloid cells, especially megakaryocytes
  • Associated ith JAK2 kinase mutation
  • Results in marrow fibrosis (due to excess PDGF)
24
Q

What does marrow fibrosis look like on hsitology?

A

Pink collagen deposited in marrow space

- due to excess PDGF

25
Q

What are the clinical features of myelofibrosis?

A
  • Splenomegaly due to extramedullary hematpoiesis
  • Leukoerythroblastic smear
  • Increased risk of infection, thrombosis and bleeding
26
Q

What are reticulin gates?

A 
Prohibit immature (larger) cells from exiting into blood 
- Defective in myelofibrosis (leukoerythroblastic)
27
Q

What kind of cells may be seen on blood smear in myelofibrosis?

A

Teardrop cells

USMLE Hematology Leukemmia and Lymphoma (14 decks)

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