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Haematology > Myeloproliferative disorders > Flashcards

Myeloproliferative disorders Flashcards

1
Q

What is polycythaemia?

A

abnormally high haematocrit + Hb conc

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2
Q

What is relative polycythaemia

A

reduced plasma volume + normal rbc mass

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3
Q

What are the causes of relative polycythaemia?

A

dehydration

chronic: obesity, HTN, high alcohol/tobacco)

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4
Q

What is absolute polycythaemia

A

increase in total number of rbcs in circulation (↑ rbc mass)

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5
Q

what is primary polycythaemia?

A

polycythaemia rubra vera

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6
Q

What are secondary causes of absolute polycythaemia?

A

hypoxia - COPD, OSA, high altitude

↑ EPO secretion - renal carcinoma, hepatocellular carcinoma

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7
Q

What is the cause of polycythaemia vera?

A

neoplastic proliferation of haematopoietic cells in the BM

JAK2 V617F mutation

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8
Q

what is polycythaemia vera?

A

neoplastic proliferation of haematopoietic cells in the BM
these cells are capable of developing into: platelets, rbcs + neutrophils so there may be thrombocytosis and neutrophil leucocytosis

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9
Q

How does polycythaemia vera present?

A
  1. hyperviscosity sx: headaches, dizziness, tinnitus, visual disturbance
  2. Itch after hot bath
  3. erythromelalgia - burning sensation in fingers and toes
  4. facial plethora
  5. splenomegaly
  6. Thrombosis - stroke, MI, PE, DVT
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10
Q

What investigations would u do>

A
  1. Haematocrit - >49% men, >48% women
  2. WBC ↑
  3. ↑ platelets
  4. BM biopsy - hypercellularity + erythroid hyperplasia
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11
Q

What is the treatment of polycythaemia vera?

A

venesection
aspirin 75mg daily
hydroxyurea
alpha interferon

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12
Q

What is essential thrombocytosis?

A

megakaryocytic proliferation resulting in overproduction of platelets

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13
Q

What are the features of essential thrombocytosis?

A

platelet ≥450 x 10⁹/L
Arterial + venous thrombosis
Microvascular occlusion

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14
Q

What are the sx of essential thrombocytosis?

A

headache
atypical chest pain
lightheadedness
erythromelalgia

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15
Q

What is the treatment of essential thrombocytosis?

A

aspirin 75mg OD

Hydroxycarbamide

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16
Q

What is myelofibrosis?

A

hyperplasia of megakaryocytic that produce platelet derived GF -> intense marrow fibrosis + haematopoiesis in spleen and liver

17
Q

How may myelofibrosis present?

A

hypermetabolic sx - WL, night sweats etc
Abdo discomfort - massive hepatosplenomegaly
Bone marrow failure - anaemia, infections, bleeding

18
Q

What is seen on blood film in myelofibrosis?

A 
leukoerythroblastic cells (nucleated rbcs)
teardrop rbcs
19
Q

What is the Rx of asymptomatic myelofibrosis?

A

folic acid + pyridoxine

allopurinol if hyperuricaemic

20
Q

What is the rx of symptomatic myelofibrosis

A

myeloablative stem cell transplant

Haematology (18 decks)

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