Lymphoproliferative disorder of granular lymphocytes associated with severe neutropenia. Response to granulocyte colony-stimulating factor.

Lymphoproliferative disorder of granular lymphocytes (LPGL) is an indolent process that is often associated with neutropenia. Although splenectomy, corticosteroids and cytotoxic agents have all been used to treat patients with life-threatening neutropenia, there are few data supporting their effectiveness. We describe a patient with LPGL, severe neutropenia, and a life-threatening infection who had a dramatic response after treatment with granulocyte colony-stimulating factor (G-CSF). The neutrophil count increased from less than 10 cells/microliters to more than 10,000/microliters 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 granulocyte-macrophage colony-stimulating factor (GM-CSF). In view of their relative lack of toxicity and rapid onset of action, the colony-stimulating factors should be considered for initial therapy in patients with LPGL and severe neutropenia. In addition, the high rate of response achieved with colony stimulating factors suggests that in many cases, a defect in myeloid maturation rather than accelerated granulocyte removal is the cause of neutropenia.