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 summray 8

Chapter 8 in Haematology (Granulocytes and monocytes) summary -Granulocytes include neutrophils (polymorphs),eosinophils and basophils.They are made in the bone marrow under the control of a variety of growth factors   and have a short lifspan in the blood stream befor entring tissues. -Phagocytes (neutrophils and monocytes) are the   body's main defence against bacterial infection.Neutrophil leucocytosis occurs in bacterial infection and in other types of inflammation.Neutropenia , if severe, predisposes to infection. It may be caused by bone marrow failure, chemotherapy or radiotherapy drugs. -Eosinophilia is most frequently caused by allergic diseases, including skin diseases,parasitic infection or drugs. Ti can be caused by clonacal increase in eosinophils termed chronic eosinophil leukamia or an idiopathic condtion, often associted with tissue damage. -Detects of function of neutrophils and monocytes may affect their chemotaxis, phagocytesis oe killing. ...
Post a Comment
Read more

Haematology summray 9

Chapter 9 in Haematology (White cells: lymphocytes) summary -Lymophcytes are immunologically comptent white cells that are involved in antibody production   (B cells) and with the body's defence against viral infection or other foreign invasion (T cells). -They arise from haemopoietic stem cells in the marrow, T cells being subseqently processed in the thymus. -B cells secrete gammaglobulin antibodies specific for individual antigens.T lymphocytes are further subdivided into helper (CD4 + ) and cytotoxic (CD8 + )cells .They recognise peptides on HLA atigens.Natural killer cells are cytoxic CD8 + cells that kill target cells with low expression of HLA molecules. -The immune response occurs in the germinal centre of lymph nodes and involves B-cell and T-cell proliferation, somatic mutation, selection od cells by recognition of antigen on antigen-presenting cells and formation of plasma cells (which secrete immunoglobulin)or memory B cells. -Immunoglobulins inc...
Post a Comment
Read more

Heamatology summary 12

Chapter 12 in Haematology (Haematoloical malignancy: managment) summary -Progress in the treatment of haemopoietic malignancies has been the result of improvemnets in both supportive therapy and speicific tumour treatments. -Supportive treatments often include : insertion of a central nevous catheter; Appropriate use of red cell and platelet transfusions; Early administration of drugs to traet infection;Otimiziation of the blood coagulation system Drugs to reduce side effects such as nausea or pain;Psychological support. -Gram-positive skin organisms such as staphyloccus are common infections and often colonize central venous catheters. -Gram-Negative bacteria are usually derived from the gut and and can cause severe septicemia. -The use of air filters,handwashing and antibiotics can reduce infection rates . -Neutropenic patients who developa fever should be treated urgently with broad-spectrum antibiotics. -Herpes viruses are a common cause of infection in ...
Post a Comment
Read more