Medicine 5th year, 4th lecture/part one (Dr. Abdulla Sharief)

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The lecture has been given on May 8th, 2011 by Dr. Abdulla Sharief.

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Medicine 5th year, 4th lecture/part one (Dr. Abdulla Sharief)

  1. 1. CLL Dr.Abdulla Sharief.
  2. 2. CHRONIC LYMPHOCYTIC LEUKAEMIA ( CLL) <ul><li>This is the most common variety of leukaemia, accounting for 30% of cases . </li></ul><ul><li>The male to female ratio is 2:1 and the median age at presentation is between 65 and 70 years . </li></ul><ul><li>In this disease B lymphocytes, which would normally respond to antigens by transformation and antibody formation, fail to do so . </li></ul><ul><li>An ever - increasing mass of immuno - incompetent cells accumulate, to the detriment of immune function and normal bone marrow haematopoiesis . </li></ul>
  3. 3. Clinical features <ul><li>The onset is very insidious . </li></ul><ul><li>Indeed, in around 70% of patients the diagnosis is made incidentally on a routine full blood count . </li></ul><ul><li>Presenting problems may be anaemia, infections, painless lymphadenopathy and systemic symptoms such as night sweats or weight loss. these more often occur later in the progress of the disease . </li></ul>
  4. 4. Investigations <ul><li>The diagnosis is based on the peripheral blood findings of a mature lymphocytosis ( > 5 × 10 9/ l ) with characteristic morphology and cell surface markers . </li></ul><ul><li>Immunophenotyping reveals the lymphocytes to be monoclonal B cells expressing the B - cell antigens CD19 and CD23 with either kappa or lambda immunoglobulin light chains and, characteristically, a T - cell antigen, CD5 . </li></ul><ul><li>Other useful investigations in CLL include a reticulocyte count and a direct Coombs test as autoimmune haemolytic anaemia may occur ( </li></ul><ul><li>Serum immunoglobulin levels should be estimated to establish the degree of immunosuppression, which is common and progressive . </li></ul><ul><li>Bone marrow examination by aspirate and trephine is not essential for the diagnosis of CLL, but may be helpful in difficult cases, for prognosis ( patients with diffuse marrow involvement tend to do worse ) and to monitor response to therapy . </li></ul>
  5. 5. Investigations <ul><li>The main prognostic factor is stage of disease </li></ul><ul><li>Newer markers such as CD38 expression, mutations of IgVH genes, and cytogenetic abnormalities of chromosome 11 or 17 may also suggest a poorer prognosis . </li></ul>
  6. 6. Clinical stage B (30% patients ) Clinical stage C (10% patients ) Clinical stage A (60% patients) <ul><li>No anaemia or thrombocytopenia and less than three areas of lymphoid enlargement </li></ul>No anaemia or thrombocytopenia, with three or more involved areas of lymphoid enlargement Staging <ul><li>Anaemia and/or thrombocytopenia, regardless of the number of areas of lymphoid enlargement </li></ul>
  7. 7. Management: <ul><li>No specific treatment is required for most clinical stage A patients unless progression occurs . </li></ul><ul><li>Life expectancy is usually normal in older patients . </li></ul><ul><li>The patient should be offered clear information about CLL, and reassured about the 'benign' nature of the disease, as the diagnosis of leukaemia inevitably causes anxiety . </li></ul><ul><li>Treatment is only required if there is evidence of bone marrow failure, massive or progressive lymphadenopathy or splenomegaly, systemic symptoms such as weight loss or night sweats, a rapidly increasing lymphocyte count or autoimmune cytopenias . </li></ul><ul><li>Initial therapy for those requiring treatment ( stages B and C ) usually consists of oral chemotherapy with the alkylating agent chlorambucil,to reduce the abnormal lymphocyte mass and produce symptomatic improvement in most patients . </li></ul><ul><li>The median survival is 5-6 years . </li></ul>
  8. 8. Management: <ul><li>The purine analogue fludarabine is also useful, although it may lead to an increased risk of infection . </li></ul><ul><li>Bone marrow failure or autoimmune cytopenias may respond to corticosteroid treatment . </li></ul><ul><li>Supportive care is increasingly required in progressive disease, e . g . transfusions for symptomatic anaemia or thrombocytopenia, prompt treatment of infections and for some patients with hypogammaglobulinaemia, immunoglobulin replacement . </li></ul><ul><li>Radiotherapy may be used for lymph nodes causing discomfort or local obstruction, and for symptomatic splenomegaly . </li></ul><ul><li>Splenectomy may be required to improve low blood counts due to autoimmune destruction or to hypersplenism, and can relieve massive splenomegaly . </li></ul>
  9. 9. Prognosis: <ul><li>The overall median survival for patients with CLL is about 6 years. </li></ul><ul><li>The majority of clinical stage A patients have a normal life expectancy but stage C patients have a median survival of between 2 and 3 years. </li></ul><ul><li>Approximately 50% of patients die of infection and 30% of causes unrelated to CLL. </li></ul><ul><li>Unlike CML, CLL rarely transforms to an aggressive high-grade lymphoma, called Richter's transformation. </li></ul>
  10. 10. Prognosis: <ul><li>The overall median survival for patients with CLL is about 6 years. </li></ul><ul><li>The majority of clinical stage A patients have a normal life expectancy but stage C patients have a median survival of between 2 and 3 years. </li></ul><ul><li>Approximately 50% of patients die of infection and 30% of causes unrelated to CLL. </li></ul><ul><li>Unlike CML, CLL rarely transforms to an aggressive high-grade lymphoma, called Richter's transformation. </li></ul>
  11. 11. PROLYMPHOCYTIC LEUKAEMIA <ul><li>This is a variant of chronic lymphatic leukaemia found mainly in males over the age of 60; 25% of cases are of the T-cell variety. </li></ul><ul><li>There is massive splenomegaly with little lymphadenopathy and a very high leucocyte count, often in excess of 400 × 109/l; the characteristic cell is a large lymphocyte with a prominent nucleolus. </li></ul><ul><li>Treatment is generally unsuccessful and the prognosis very poor. Leukapharesis, splenectomy and chemotherapy may be tried. </li></ul>
  12. 12. HAIRY CELL LEUKAEMIA <ul><li>This is a rare chronic lymphoproliferative B-cell disorder. </li></ul><ul><li>The male to female ratio is 6:1 and the median age at diagnosis is 50. </li></ul><ul><li>Presenting symptoms are those of general ill health and recurrent infections. </li></ul><ul><li>Splenomegaly occurs in 90% but lymph node enlargement is unusual. </li></ul><ul><li>Severe neutropenia, monocytopenia and the characteristic hairy cells in the blood and bone marrow are typical. </li></ul><ul><li>These cells usually type as B lymphocytes characteristically express CD25 and CD103. </li></ul><ul><li>A useful test is the demonstration that the acid phosphatase staining reaction in the cells is resistant to the action of tartrate. </li></ul><ul><li>Over recent years a number of treatments have been shown to produce long-lasting remissions. </li></ul><ul><li>Cladribine and deoxycoformycin are effective in producing long periods of disease control. </li></ul>

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