Myeloproliferative disorders Flashcards
What are the different myeloproliferative disorders and which cells do they involve?
Multiple myeloma - plasma cells
Polycythemia vera - RBC
Essential thrombocythaemia - Platelets
Fibroblasts - myelofibrosis
How is polycythaemia vera treated?
treated with regular venesection and hydroxyurea
How is essential thrombocythaemia treated?
With aspirin and in high risk patients hydroxycarbamide
How is myelofibrosis treated?
With bone marrow support
Stem cell transplant may be curative in young people
What is the treatment for multiple myeloma?
Supportive:
-Bisphosphonates to reduce fractures and bone pain
-Orthopaedic procedures for symptomatic relief e.g. vertebroplasty
-Treat anaemia with transfusion
-Renal failure may require rehydration or dialysis
-Treat infections with broard spectrum
Chemo:
-In suitable fit patients chemo may be followed by stem cell transplant
What is used to monitor multiple myeloma activity?
Ig levels that are being secreted by the plasma cells
What are the presenting features of myelofibrosis?
Remember with the pneumonic CRABBI
Calcium - Increased osteoclast activity causes hypercalcaemia, this causes constipation, anorexia, nausea and confusion
Renal - monoclonal immunoglobulin production causes light chain deposition in the renal tubules
Anaemia - erythropoiesis suppressed leading to anaemia causing pallor and fatigue
Bleeding - thrombocytopenia puts patients at increased risk of bleeding and bruising
Bones - Bone marrow infiltration and increased osteoclast activity causes lytic lesions and pain especially in the back
Infection - increased susceptibility to infections due to reduced production of normal immunoglobulins
What are the investigation findings for multiple myeloma?
Bloods will show anaemia, thrombocytopenia, raised calcium and raised U and Es
Serum or Urine electrophoresis - shows increased IgA and IgG - known as bence jones proteins in the urine
Whole body MRI is useful to survey skeleton for lesions - rain drop skull is common finding
