LYMPHOPROLIFERATIVE DISORDER OF GRANULAR LYMPHOCYTES ASSOCIATED WITH SEVERE NEUTROPENIA - RESPONSE TO GRANULOCYTE-COLONY-STIMULATING FACTOR

Lymphoproliferative disorder of granular lymphocytes (LPGL) is an indo lent process that is often associated with neutropenia. Although splen ectomy, corticosteroids and cytotoxic agents have all been used to tre at patients with life-threatening neutropenia, there are few data supp orting their effectiveness. We describe a patient with LPGL, severe ne utropenia, and a life-threatening infection who had a dramatic respons e after treatment with granulocyte colony-stimulating factor (G-CSF). The neutrophil count increased from less than 10 cells/mul to more tha n 10,000/mul after seven doses of G-CSF. The infection promptly healed . A review of the literature indicates that 8 of 11 patients with LPGL and severe neutropenia responded to treatment with G-CSF or granulocy te-macrophage colony-stimulating factor (GM-CSF). In view of their rel ative lack of toxicity and rapid onset of action, the colony-stimulati ng factors should be considered for initial therapy in patients with L PGL and severe neutropenia. In addition, the high rate of response ach ieved with colony stimulating factors suggests that in many cases, a d efect in myeloid maturation rather than accelerated granulocyte remova l is the cause of neutropenia.