What is Myelofibrosis?

A bone marrow disorder in which excessive scar tissue forms in the bone marrow and disrupts the body’s normal production of blood cells

What are the main clinical features of Myelofibrosis?

• anaemia
• massive splenomegaly
• hypermetabolic symptoms: weight loss, night sweats etc

What are the blood findings in Myelofibrosis?

• anaemia
• high WBC and platelet count early in the disease
• high urate and LDH (reflect increased cell turnover)

What is the pathophysiology of Myelofibrosis?

• thought to be caused by hyperplasia of abnormal megakaryocytes
• the resultant release of platelet derived growth factor is thought to stimulate fibroblasts
• haematopoiesis develops in the liver and spleen

What is the bone biopsy finding in Myelofibrosis?

unobtainable bone marrow biopsy - 'dry tap' therefore trephine biopsy needed