The abnormal cell is a clonal B-cell lymphocyte . It infiltrates the patient's reticuloendothelial system and interferes with bone marrow function, resulting in failure or pancytopenia, and infiltrates the liver and spleen, resulting in organomegaly. Most patients are white males with a median age of 50 years. Abdominal discomfort is a common symptom, due to hepatosplenomegaly. It is usually controlled with new, effective drug therapy, although late recurrences may occur.
Recurrences are usually responsive to medications.
This disease is more commonly seen in whites. It has been reported to occur in Ashkenazi Jewish males and it is rare in Japan. Symptoms related to organ infiltration of the reticuloendothelial system may occur. Some patients may present with weight loss, fever, and night sweats similar to other lymphoproliferative disorders. A low-grade fever is part of the disease, but may be due to an infection from the resulting neutropenia.