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YR3: Haematology > Myleofibrosis > Flashcards

Myleofibrosis Flashcards

1
Q

What is Myleofibrosis?

A

Disorder of haematopoietic stem cells characterised by progressive bone marrow fibrosis associated with extramedullary haematopoiesis + splenomegaly

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2
Q

Describe the pathogenesis of Myleofibrosis

A 
Hyperplasia of atypical megakaryocytes 
Increased TGF-B release 
Increased activity of fibroblasts 
Increased collagen deposition in bone marrow  
BM obliteration due to fibrosis
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3
Q

Describe the aetiology of Myleofibrosis

A

Primary stem cell defect is UNKNOWN

Associated with JAK2 mutation

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4
Q

List 4 risk factors for Myleofibrosis

A

Age >65
Radiation exposure
Hiroshima survivor
Industrial solvent exposure (benzene/ other aromatic solvents)

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5
Q

What do 30% of patients with Myleofibrosis have a PMH of?

A 
Polycythaemia rubra vera
Essential thrombocythaemia (overproduction of platelets by BM)
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6
Q

Describe the epidemiology of Myleofibrosis

A

RARE

Peak onset: 50-70 yrs

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7
Q

What 5 complications of extra medullary haematopoiesis may Myleofibrosis present with?

A 
Hemorrhage
Spinal cord compression
Focal seizures
Ascites
Pleural effusion
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8
Q

List 6 common systemic symptoms of Myleofibrosis

A 
Fever 
Anorexia 
Weight loss  
Night sweats  
Pruritis  
Abdominal discomfort
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9
Q

List 7 uncommon systemic symptoms of Myleofibrosis

A 
LUQ pain  
Indigestion (due to massive splenomegaly)  
Bleeding  
Bone pain  
Hearing loss  
Gout  
Infections
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10
Q

List 6 signs of Myleofibrosis

A 
SPLENOMEGALY 
Hepatomegaly (in 50-60%) 
Pallor
Petechiae
Haemarthrosis
Blood on PR
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11
Q

What features although uncommon, may be seen in Myleofibrosis?

A

Portal HTN features due to increased splenoportal blood flow:
Ascites
Varices + GI bleeding
Hepatic encephalopathy

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12
Q

What is seen in early stages of Myleofibrosis on FBC?

A

Variable Hb, WCC + platelets
Thrombocytosis
LFTs: abnormal

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13
Q

What is seen in later stages of Myleofibrosis on FBC?

A

Anaemia
Leukopaenia
Thrombocytopaenia
LFTs: abnormal

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14
Q

What is seen on blood film in Myleofibrosis?

A 
Dacrocytes: 'Tear drop' poikilocyte red cells
Leucoerythroblastic changes (red + white cell precursors in the peripheral blood): Metamyelocytes, myelocytes, myeloblasts + nucleated RBCs
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15
Q

Why is bone marrow aspiration often unsuccessful in Myleofibrosis?

A

Dry tap due to marrow fibrosis

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16
Q

What investigation is diagnostic for Myleofibrosis and what is seen?

A

Trephine bone marrow biopsy

Shows fibrotic hypercellular marrow, with dense reticulin fibres on silver staining

YR3: Haematology (19 decks)

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