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Pathology - Blood Module > Primary Myelofibrosis (MPD) > Flashcards

Primary Myelofibrosis (MPD) Flashcards

1
Q

What happens in PMF?

A

Obliterative marrow fibrosis
Cytopenias
Extramedullary hematopoiesis

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2
Q

How do you define marrow fibrosis?

A

Collagen deposition + Angiogenesis

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3
Q

Why does anemia occurs in PMF if extramedullary hematopoiesis is occuring?

A

Extramedullary hematopoiesis is also disordered so that’s why anemia occurs

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4
Q

What is the pathogenesis of PMF?

A

Inappropriate release of fibrogenic factors from platelets leads to fibrosis

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5
Q

What are the fibrogenic factors released by platelets in MPD?

A

PDGF, TGF-B

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6
Q

What are the mutations involved in PMF?

A

JAK2, MPL, calreticulin

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7
Q

PMF occurs more in persons ______ (older/younger) than 60 years

A

older

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8
Q

What are the clinical features of PMF?

A 
⦁	Progressive anemia 
⦁	Sensation of fullness
⦁	Fatigue
⦁	Weight loss
⦁	Night sweats
⦁	Hyperuricemia and gout
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9
Q

Sensation of fullness is due to __________ (liver failure/splenomegaly/hepatomegaly) in PMF

A

splenomegaly

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10
Q

What is the reason of night sweats in PML?

A

increased metabolism ass. w/ expanding mass of hematopoietic cells

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11
Q

What are the stages of bone marrow in PMF?

A

Early
Late
Very late

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12
Q

What happens in early stage of PMF in bone marrow?

A

Hypercellularity
Normal erythroid and granulocytic precursors
Large megakaryocytes

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13
Q

What happens in late stage of PMF in bone marrow?

A

Hypocellularity
Fibrosis
Megakaryocytes with cloud like nucleus
Hematopoietic elements move to dilated sinusoids

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14
Q

What happens in very late stage of PMF in bone marrow?

A

osteosclerosis

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15
Q

What are the changes in spleen in PMF?

A

Color changed (red to gray)
Size changed (4000 gm)
Subcapsular infarcts occur
EMH move from sinusoids to cords

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16
Q

What are the findings in peripheral blood smear?

A

Tear drop RBCs
Leukoerythroid precursors
Basophils
Large platelets

17
Q

What is the type of anemia in PMF?

A

normocytic normochromic anemia

18
Q

What does the CBC report says in PMF?

A

Anemia
Leukoerythroblastosis
Early - High WBC and platelet count
Late - Low WBC and platelet count

19
Q

What is the median survival rate of PMF?

A

3-5 years

20
Q

What is the treatment of PMF?

A

JAK2 inhibitos

Hematopoietic stem cell transplantation in young

21
Q

What threats develop when someone is suffering from PMF?

A

Intercurrent infections
Thrombotic episodes
Bleeding
Transformation to AML (even where there is EMH)

Pathology - Blood Module (20 decks)

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