?Chronic myelogenous? leukemia of juvenile type

Abstract

The juvenile type of “chronic myelogenous” leukemia (CMLJT) is a rare disease with only 40 cases reported to date. Clearly distinguishable from adult CML on both clinical and laboratory grounds, is is often confused with “congenital” leukemia, pseudoleukemia, leukemoid reactions or chronic granulomatous disease. According to studies of muramidasuria and colony-forming cells it is neither a chronic nor a granulocytic leukemia. It is a panmyelopathy with monocyte predominance and should thus be classified as a variant of myelomonocytic leukemia. We review reported responses to chemotherapy and splenectomy and report our results with cytosine arabinoside in the treatment of 2 cases with this disease. Chemotherapy may prolong life and splenectomy may be useful in some cases; but the survival rate is 0%, justifying new approaches.