The definition of remission in a malignancy is established in order to distinguish the effectiveness of different treatment options and determine whether treatment has an impact on survival and quality of life. The development of these definitions for HCL has paralleled the improved management of this disease.

For many years, the major treatment for patients with HCL was splenectomy.5 Splenectomy was undertaken either due to pain and discomfort secondary to an enlarged spleen or for the cytopenias seen in this disease. Responses to splenectomy were classified according to those established by Catovsky6 (Table 32.1). A complete response (CR) was defined as an increase in hemoglobin level above 11 g/dL, neutrophils above 1 X 109/L, and platelets above 100 X 109/L. A partial response (PR) was the same degree of improvement in one or two of the blood elements or improvement in all three, but below the values for CR. These criteria took into account that splenectomy did improve cytopenias in 60-100% of the patients, but did not significantly affect the bone marrow infiltration or circulating hairy cells.5 7 In fact, the degree of bone marrow involvement prior to splenectomy appeared to predict the degree of success with this procedure, and thus patients with marked infiltration of the marrow with hairy cells were less likely to respond.7

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