Skip to main content

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       

Comments

Post a Comment

Popular posts from this blog

Haematology summary 18

  Chapter 18 in haematology (chroinc lymphoid leukaemias)summary -Chronic lymphocytic leukaemiaes are characterized by the accumulation of mature B or T lymphocytes in the blood. -Individual subtypes are distinguished on the basis of morphology,immunophenotype and cytogenetics. -Chronic lymphocytic leukaemia (CLL,B cell) represents 90% of cases and has a peak incidece between 60 and 80 years of age .There is genetic predisposition to development of the disease. -Most cases are indenified when a routine blood test is performed the patient can develope enlarged lymph nodes ,splenomegaly and hepatomegaly. -Immunosuppression is asignificant problem because of hypogammaglobuilaemia and cellular immune dysfunction. -Anaemia may also develop because of autoimmune haemolysis and bone marrow in filtration. -Diagnosis is usually performed by immuophenotypic analysis of peripheral blood which reveals aclonal population of CD5 + ,CD23 + B cells. -The best guide
2 comments
Read more

Haematology summary 19

Chapter 19 in haematology (Hodgkin lymphoma)summary -Lymphoma are a group of diseases caused by malignant lymphocytes that accumulation in lymph nodes and cause lymphoadenopathy. -The major subdivision of lymphomas ia into hodgkin and this is based on the presence of Reed-strenberg cells in hodgkin lymphoma. -Reed-strenberg cells are neoplastic B cells but most cells i the lymph node are reactive inflammatory cells. -The usual clinical presentation is with painless asymmetrical lymphadenopathy- most commonly in the neck. -Constitutional symptoms of fever,weight loss and sweating are prominent in patients with widespresd disease. -Blood tests may show anaemia, neutophilia and rasied erythrocyte sedmentation rate (ESR) or lactic dehydrogenase (LDH). -Diagnosis is made by histological examination of an excised lymph node and there are four subtype of disease . -Staging of the disease is important for determining treatment and prognosis history, examiantion
Post a Comment
Read more

Haematology summary 17

Chapter 17 in haematology (Acute lymphoblastic leukaemia)summary -Acute lymphoblastic leukaemia (ALL) is casued by an accumulation of lymphoblasts in the bone marrow. It isthe most common malignant disease of clidhood-75% of cases occur before the age of 6 years. Eighlty-five per cent of cases are of B-cell lineage with the rest being of T-cell lineage. -The first genetic mutation ocuurs in amy cases in utero , with a secondary genetic event occuring later inclidhood,possibly as a reaction to an infection. -The clinical presentation is with the features of bone marrow failure (anaemia,infection and bleeding) together with symptoms of tissue infiltration by tumour cells, leading to bone pain or swollen lymph nodes. -Diagnosis is by examination of blood and bone marrow.important tests include microscopic examination of the tumour cells, immunophenotyping and genetic analysis. -ALL is subclassified according to the underlying genetic defect and a wide varity of genet
Post a Comment
Read more