Myelofibrosis

Question 1

Define Myelofibrosis

Answer 1

Myelofibrosis is a reactive and reversible process of fibrotic scarring of the bone marrow
commonly seen in many bone marrow disorders and malignancies

causes abnormal production of Myeloid cells

denovo scarring (-primary Myelofibrosis (PMF) -scarring from fribroblast  multipotent heamotopoetic stem cell
if fibrosis related to a known cause (leukemia, drugs, etc)-secondary/reactive

Question 2

Aetiology and risk factors of Myelofibrosis

Answer 2

Unkown, but lots of genes have been found to be weird after analysis-but unclear if causative -over 50 mutations
Increase risks after radiation
Pathophysio-fibroblasts are polyclonal, identicating a reactive origin-they then proliferate and block out other cells

risk factors 
age-65
in younger pt, girls>boy
radiation exposure, 
benzene exposure

Question 3

Epidiemology of Myelofibrosis

Answer 3

about 1.4 in 100 000

Question 4

Signs and Sx of Myelofibrosis

Answer 4

an initial pre-fibrotic stage exists which presents with thrombocytopenia, undistinguishable from essential thrombo

usually PMF is a diagnosis of exclusion because there is no diagnostic marker

Constitutional Sx:
Fever, malaise, weight loss, night sweats, fatigue
Pruritus/bleeding

features of extra medullary heamatopoesis (heamotopoesis outside of bone marrow)-heamorrghage (GI (Malena, chzesia, heamotpsis, heamaturia)

Features of portal hypertension-

osteosclerosis-bone pain
recurrent infections

Splenomegaly is ALWAYS present
Hepatomegaly can be

Question 5

Investigations of Myelofibrosis

Answer 5

FBC-aneamia, thrombocytopenia,
leukopenia

Blood smear-nothing to specific to PMF
but always-tear shaped RBC, lots of myelocytes, nucleated RBC in circulation

Bone marrow aspiration-cannot absord-DRY TAP-as its fibrosed

Core biopsy-fibrosis