Myeloproliferative Disorders Flashcards
What causes polycythemia Vera?
JAK2 mutation in 95% of cases, leading to activist icon of a tyrosine kinase causing extra RBC proliferation.
Features of polycythemia Vera,?
Headache, dizziness, tinnitus, visual disturbance, angina, cladication, pruritis (uric acid), and venous thrombosis.
Red complexion and hepatosplenomegaly
What do blood tests show in polycythemia Vera?
High Hb >18.5g/l, High wcc, high platelets, low serum epo,
Treatments for polycythemia Vera?
RBC - venesection
Platelets - chemo/ hydroxyurea (hydroxycarbimide)/ busulfan
Clots - aspirin
Radio active phosphorus (only in over 70s)
Uric acid - allopurinol
What is myelofibrosis?
Heamopoetic stem cell proliferation and marrow fibrosis.
Features: weight loss, weakness, lethargy, hepatosplenomegaly
Whats seen in investigations for myelofibrosis?
Bloods - anemia, initially high wcc and plts then fall as marrow fails,
Film- immature red cells and tear drop shaped red cells
Bone marrow - dry tap, biopsy shows fibrosis
No Philadelphia chromosome (to distinguish from CML)
What’s the presentation of myeloma?
Calcium - high!
Renal failure
Anemia - BM failure, and infections
Bone - lesions, back ache, fractures
What do investigative tests show in myeloma?
Blood- Low Hb (normocytic), low WCC, low plt,
High ESR, high Ca2+, high ALP
Blood film- roulleax formation
Marrow film - ⬆️ plasma cells
Serum and urine electrophoresis- monoclonal bands
X-ray - osteolytic lesions eg pepper pop skull
Treatment of multiple myeloma?
1) supportive - analgesia, bisphosphonates, transfusion, EPO, Rx for infections and renal failure
2) chemo
Which is the most common plasma cell involved in myeloma?
IgG (2/5)
IgA (1/5)
