Myelofibrosis Flashcards
What is the average age of presentation of patients with Idiopathic Myelofibrosis?
> 60
What is the risk of males vs females?
Equal risk
What is Myelofibrosis?
Neoplastic proliferation of mature myeloid cells especially megakaryocyes which produce excess PDGF which causes marrow fibrosis.
What are some of the physical findings?
- Splenomegaly due to extramedullary haematopoiesis.
- Portal hypertension
- Hepatomegaly
- Bone pain and tenderness
- Muscle wasting
- Purpura
- Neutrophilic Dermatosis
- Thrombosis
- Tear Drop shaped RBCs on PB smear
What type of collagen is most prominent in the marrow in Myelofibrosis?
Type III collagen
What is the main cause of the fibrosis?
Release of cytokines by aberrant megakaryocytes.
What are some other complications of Myelofibrosis?
- Fibrohematopoetic Extramedullary Tumors
- Portal Hypertension Resulting in ascites, esophageal and gastric varices
- Osteosclerosis in Proximal femur and humerus pelvis, vertebral bodies, ribs and skull
What are some of the therapies for Primary myelofibrosis?
- Androgens
- Procrit
- Hydroxyurea
- Thalidomide
- Lenalidamide
What sub-type of Primary Myelofibrosis may achieve remission?
del 5(q31)
What is the purpose of radiation therapy in PMF?
- Splenic pain
- Massive Splenic enlargenment
- Focal bone pain
- Extramedullary tumors
What would require a splenectomy in PMF?
- Painful enlargement
- Excessive transfision requirement
- Severe Thrombocytopenia
- Portal hypertension
What is the JAK2 inhibitor currently used for PMF?
Ruxolitinib
What effect does Ruxolitinib have?
- Decreases the cytokine production
- Decreases the size of the Spleen
- Does not improve the fibrosis
What is the only possible cure for PMF?
Bone Marrow transplant.
What is the main issue with Bine marrow transplants in PMF patients?
The age of the patients and mortality risk
