Hairy cell leukemia

The hairy cell leukemia is one of the low-grade B- cell non -Hodgkin's lymphoma. The disease takes its name from the characteristic fringed foothills ( hair-like Zytoplasmafortsätzen ), which are microscopic in the malignant cells ( " hair cells") can be seen obtained.

Epidemiology and Causes

The hairy cell leukemia is a rare disease, it accounts for about 2% of all leukemias from. The median age at diagnosis is 52 years, male gender predominates significantly (ratio 4:1). The reasons for the emergence of a hairy cell leukemia are largely unknown.

Symptoms

  • Pancytopenia by diffuse bone marrow infiltration and marrow fibrosis Anemia with fatigue and weakness
  • Leukopenia with increased susceptibility to infection
  • Thrombocytopenia with increased bleeding tendency

Diagnosis

Only about 10-20% of patients, there is an excessive proliferation of white Blutkörpcherchen ( leukocytosis ), in the majority of the white blood cell counts are reduced even in the blood ( in the sense of leukopenia ). Nevertheless ( smear of venous blood ) can be found in the peripheral blood smear in most patients, the typical hair cells. In these cells, or in Knochenmarksausstrichen can be cytochemically an enzyme, tartrate - resistant acid phosphatase detected. The diagnosis is confirmed by histological and immunohistological examination of bone marrow.

Differential Diagnosis

  • Aplastic anemia
  • Chronic Lymphocytic Leukemia
  • Myelodysplastic syndrome

Therapy

The treatment of choice is a treatment with the purine analog cladribine ( 2- CdA Chlorodeoxyadenosin or 2 ) by subcutaneous injection. The response rate is over 90 % with numerous complete cures ( remissions ). As another purine derivative is pentostatin available. Before the introduction of purine analogues the disease with interferon- α was treated. Also, removal of the spleen ( splenectomy ) in many cases leads to remission with normalization of the blood picture, but plays in therapy due to the very good drug treatment options are not essential more.

  • Non -Hodgkin's lymphoma
  • Leukemia
International Classification of Diseases for Oncology B cell Bleeding diathesis Hepatomegaly Blood film Complete blood count
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