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Chapter 21in haematology (multiple myeloma and related disorder)summary -The term paraprotienaemia refers to the presnce of a monoclonal immunoglobulin band in serum and reflects the synthesis of immunoglobin from asingle clone of plasma cells. -Multiple myeloma is a tumour of plasma cekks that accumulate in the bone marrow, release a prarprotein and cause tissue damage. The disease has apeak incidence in the seventh decade . -Almost all cases of myeloma develop from a pre-existing mnonclonal gammopathy of undetermined sigificance (MGUS)in which there is low level paraprotien and no 1% of cases progress to myeloma each year. -A useful reminder for the spectrum of tissue damage in myeloma is CRAB- hypercalaemia,renal impairment,anaemia,bone disease. -in patient younger than 75 years myeloma is usually treated bu intensve chemotherapy followed by anautologous stem cell transplant using stem cells harvested from the patient. -In older patiet chemotherapy alone is u

Chapter 20 in haematology (Non-Hodgkin lymphoma)summary -Non-Hodgkin lymphomas are a large group of clonal lymphiod tumours.Appromixately 85% are of B-cell origin and 15% derive from T or NK cells. -Their clinical prestation and natural history are more variable than hodgkin lymphoma and can vary from very indolent disease subtypes though to rapidly progressive subtypes that need urgent treatment . -For many years clinicans have divided lymphomas into lowgrade and high-grade disease. This is useful as low –grade disorder are typically slowly progressive,respond well to chemothrapy but are vary difficult to cure,whereas high-grade lymophomas are aggressive more often curable. -Investigation is with lymph node biopsy,blood tests and radiology. Immuohistochemistry of the lymph node is valuable and cytogenetic analysis is performed in may cases. -Clinial staging is performed as for hodgki lymphoma. -Treatment for NHL   are based on a variety of chemotherapy regim

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

  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

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

Chapter 16 in haematology (Myelodysplasia)summary -Myelodysplasia include a group of clonal disorders of heamopoietic stem cells that lead to bone marrow failure and low blood cell counts.Ahallmark of the disease is simultaneous proliferation and apoptosis is haemopoietic cells leading to the paradox of a hypercellular bone marrow but pancytopenia in peripheral blood . there is a tendency to progress to acute myeloid leukaemia. -In most cases, the disease is primary but it may be secondary to chemotherapy given for treatment of another malignancy. -The main clinical features of anaemia, infectionand bleeding, are caused by reduction in the bone count.most patients are over 70 years of age. -Diagnosis is made by examination of the blood and bone marrow together with genetic studies of the tumour cells . they are calssfiedinto eight major subtypes. -Scoring systems can divide patients in those with low-grade or high-grade disease . -Low-grade disease may not nee