Megakaryocyte dysplasia
and increase in PLT
production
Presents with vasomotor Sx / bleeding
/ thrombotic / viscocity
Treatment: Aspirin
Blood smear shows
increased PLT count
and Bone marrow
shows megakaryocytes
Exclude secondary causes
e.g. reactive
thrombocythaemia
Primary Myelofibrosis
Nota:
PATHOPHYS
Replacement of bone marrow tissue with fibrous tissue from fibroblasts / Reduction in production of normal haematopoesis - leads to extramed haematopoeisis.
CAUSE
Primary or secondary to malignancyPRESENTATIONAnaemiaHepatosplenomegalyINVESTIGATIONTear drop RBC poikilocytes in circulation / bone marrow aspiration shows fibrous tissue (dry)TREATMENTTreat symptoms e.g. anaemia with EPO / transfusions
Splenectomy
Chemotherapy
Bone marrow replaced
by fibrous tissue
Presents with
anaemia and
hepatosplenomegaly
Tear drop poikilocyte
RBCs and fibrous
tissue in bone marrow
Treat symptoms e.g.
anaemia / splenectomy /
chemotherapy
Often secondary to
other cancers e.g.
Lymphoma
Plasma Cell
Malignancies
Multiple Myeloma
High protein levels in the
blood and urine (Bence
Jones Proteinuria) -
nephrotic syndrome
X-ray: Lytic lesions in
bones due to bone
infiltration (leads to
hypercalcaemia