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

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

  • CLL Dr.Abdulla Sharief.
  • CHRONIC LYMPHOCYTIC LEUKAEMIA ( CLL)
    • This is the most common variety of leukaemia, accounting for 30% of cases .
    • The male to female ratio is 2:1 and the median age at presentation is between 65 and 70 years .
    • In this disease B lymphocytes, which would normally respond to antigens by transformation and antibody formation, fail to do so .
    • An ever - increasing mass of immuno - incompetent cells accumulate, to the detriment of immune function and normal bone marrow haematopoiesis .
  • Clinical features
    • The onset is very insidious .
    • Indeed, in around 70% of patients the diagnosis is made incidentally on a routine full blood count .
    • 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 .
  • Investigations
    • The diagnosis is based on the peripheral blood findings of a mature lymphocytosis ( > 5 × 10 9/ l ) with characteristic morphology and cell surface markers .
    • 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 .
    • Other useful investigations in CLL include a reticulocyte count and a direct Coombs test as autoimmune haemolytic anaemia may occur (
    • Serum immunoglobulin levels should be estimated to establish the degree of immunosuppression, which is common and progressive .
    • 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 .
  • Investigations
    • The main prognostic factor is stage of disease
    • Newer markers such as CD38 expression, mutations of IgVH genes, and cytogenetic abnormalities of chromosome 11 or 17 may also suggest a poorer prognosis .
  • Clinical stage B (30% patients ) Clinical stage C (10% patients ) Clinical stage A (60% patients)
    • No anaemia or thrombocytopenia and less than three areas of lymphoid enlargement
    No anaemia or thrombocytopenia, with three or more involved areas of lymphoid enlargement Staging
    • Anaemia and/or thrombocytopenia, regardless of the number of areas of lymphoid enlargement
  • Management:
    • No specific treatment is required for most clinical stage A patients unless progression occurs .
    • Life expectancy is usually normal in older patients .
    • 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 .
    • 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 .
    • 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 .
    • The median survival is 5-6 years .
  • Management:
    • The purine analogue fludarabine is also useful, although it may lead to an increased risk of infection .
    • Bone marrow failure or autoimmune cytopenias may respond to corticosteroid treatment .
    • 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 .
    • Radiotherapy may be used for lymph nodes causing discomfort or local obstruction, and for symptomatic splenomegaly .
    • Splenectomy may be required to improve low blood counts due to autoimmune destruction or to hypersplenism, and can relieve massive splenomegaly .
  • Prognosis:
    • The overall median survival for patients with CLL is about 6 years.
    • 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.
    • Approximately 50% of patients die of infection and 30% of causes unrelated to CLL.
    • Unlike CML, CLL rarely transforms to an aggressive high-grade lymphoma, called Richter's transformation.
  • Prognosis:
    • The overall median survival for patients with CLL is about 6 years.
    • 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.
    • Approximately 50% of patients die of infection and 30% of causes unrelated to CLL.
    • Unlike CML, CLL rarely transforms to an aggressive high-grade lymphoma, called Richter's transformation.
  • PROLYMPHOCYTIC LEUKAEMIA
    • 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.
    • 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.
    • Treatment is generally unsuccessful and the prognosis very poor. Leukapharesis, splenectomy and chemotherapy may be tried.
  • HAIRY CELL LEUKAEMIA
    • This is a rare chronic lymphoproliferative B-cell disorder.
    • The male to female ratio is 6:1 and the median age at diagnosis is 50.
    • Presenting symptoms are those of general ill health and recurrent infections.
    • Splenomegaly occurs in 90% but lymph node enlargement is unusual.
    • Severe neutropenia, monocytopenia and the characteristic hairy cells in the blood and bone marrow are typical.
    • These cells usually type as B lymphocytes characteristically express CD25 and CD103.
    • A useful test is the demonstration that the acid phosphatase staining reaction in the cells is resistant to the action of tartrate.
    • Over recent years a number of treatments have been shown to produce long-lasting remissions.
    • Cladribine and deoxycoformycin are effective in producing long periods of disease control.