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CHRONIC LYMPHOCYTIC LEUKEMIA

o Increased white cells of B-cell origin
o Lymphocytosis greater than 5000 cells
o Failure of white cells to "die"
o The most common type of leukemia
o Peripheral smear shows increased white cells and 'smudge' cells
o Associated clinical conditions include autoimmune hemolytic anemia, recurrent infections, lung and skin cancers
o Subsequent change to prolymphocytic leukemia
o Etiology of this condition is unknown

SIGNS AND SYMPTOMS

o Usually discovered on routine physical
o Most patients are asymptomatic
o It usually runs a protracted course
o Other complaints include swellings in the neck, groin, axial and abdomen
o Generalized weakness secondary to anemia
o Bleeding due to low platelets
o Recurrent respiratory infections
o Liver and spleen enlargement may also be present
o Chromosome abnormalities include trisomy 12 and t (11; 14)

RAI'S STAGING

Stage 0 Lymphocytosis
State I Lymphocytosis and Lymphadenopathy
Stage II Enlargement of liver and spleen
State III Anemia
Stage IV Low platelet count

DIAGNOSIS

o Bone marrow aspirate not essential to make a diagnosis
o Lymph node biopsy also not essential for diagnosis
o Positive CD19, CD20, CD5 and CD21
o Serum immunoglobulin levels are low

TREATMENT

o Most patients do not need treatment because of the protracted course of the disease
o Indications for treatment include but are not limited to
o Disfiguring lymph node enlargement
o Abdominal swelling and pain
o Evidence of bone marrow failure (low platelets and anemia)
o Steroids might be necessary in patients with autoimmune hemolytic anemia
o Intravenous gammaglobulin may be required in patients with recurrent bacterial infections
o Fludarabine produces remission in about 70% of cases
o Other agent that had been in use before the emergence of fludarabine is chlorambucil
o Splenectomy may also be necessary in patients with autoimmune conditions
o Other forms of treatment in patients who have fludarabin-resistant disease include:

  1. monoclonal antibodies (rituximab, campath)
  2. 'mini' allogeneic transplantation
  3. gene therapy
  4. protein kinase C (PKC) and cyclin dependent kinase (CDK) inhibitors e.g. flavopiridol