Splenomegaly is present in the majority of patients with hairy cell leukemia, with leukemic infiltration primarily within the red pulp.3132 Therefore, removal of the spleen eradicates one of the major sites of disease accumulation.

Splenectomy was the treatment of choice for hairy cell leukemia before the introduction of effective cyto-toxic therapies. In the majority of cases, the peripheral blood counts responded favorably and patients survived for years after the operation, often without additional therapy. Splenectomy removes splenic sequestration of blood elements, thereby alleviating pancytopenia. However, in situations in which pancytopenia is secondary to bone marrow infiltration by hairy cells, splenectomy is less beneficial. CR, defined as hemoglobin greater than 11.0 g/dL, granulocyte count greater than 1000/mm3, and a platelet count greater than 100,000/mm3 (Catovsky criteria), has been reported in 40-67% of patients (see Table 31.3). Improvement in at least one cytopenia is seen in up to 90% of patients. The most common hematologic parameter to improve postsplenectomy is the platelet count, often seen within days of the surgery. Patients who achieve a CR have improved survival compared to partial responders.33,35,37,41 However, an overall survival benefit from splenectomy has not been consistently demonstrated.34'36 39 42 Before the introduction of effective systemic therapies, patients who did not respond to splenectomy had a poor prognosis. Both the probability of response as well as the duration of response inversely correlates with the degree of bone marrow involvement. The duration of response to splenectomy has been evaluated extensively (see Table 31.3).

Table 31.3 Splenectomy in the treatment of hairy cell leukemia


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