Haematolgy summary 15

Haematolgy summary 15

Chapter 15 in Haematology (myeloproliferative neoplasms) summary     

The increase in blood viscosity leads to headaches,plethoric appearance and splenomegaly.

-Treatment aims to maintain the haematocrit around 0.45. useful apporaches include venesection or hydroxyurea and aspirin is also given.JAK2 inhibitors are being assessed in clinical trials.survival is usually over 10 years but there may be progression to leukaemia or myelofibrosis.

-Secondary polycythaemia can arise from rare congential causes or acquired disorder such as lung disease or tumours that secrete erythropoietin,venesection may be needed.

-Essential thrombocythaemia is diagnosed by persistent raied platelet count in the absence of other causes.JKA2 is mutated in appromixtaley 50% of cases.

-The predominant feature of primary myelofibrosis is a progressive generalized reactive fibrosis of the bone marrow in association with the developement of haemopoiesis in the spleen and kiver.Symptoms usually result from anaemia and a grossly enlarged spleen.

-Diagnosis is made on blood film, which shows a leucoerythroblastic appearance,together with bone marrow biopsy and JKA2 mutation screen .Treatment is with red cell tranfusion .splenectomy is sometimes used and JKA inhibitors appear encouraging.

-Systemic mastocytosis is a clonal proliferation of masr cella with involvement of bone marrow,skin(as uticaria pigmentosa)and other organs .

 

From book: Essentail Haematology A.V.HOFFBRAND,P.A.H.MOSS.6EDITION