Chronic myeloid leukemia. A possible new variant in a child.
A case of Philadelphia (Ph') chromosome negative chronic myeloid leukemia (CML) in a child is reported. The age of onset (7 years) and duration of survival (40 months) are not typical of juvenile CML and the relatively low leukocyte and hemoglobin F concentrations are more akin to the findings in Ph' negative CML in adults. There was refractoriness to chemotherapy with the exception of a dramatic response to a combination of prednisone and vincristine, despite the absence of terminal transferase activity in circulating blood cells. Splenic irradiation, splenectomy, and leukapheresis were ineffective. Transition to a terminal aggressive phase caused a marked change in the growth characteristics of peripheral blood cells in vitro. This patient may represent a new variant in the spectrum of childhood CML.
D002648:Child; D002904:Chromosomes, Human, 21-22 and Y; D004359:Drug Therapy, Combination; D006801:Humans; D007951:Leukemia, Myeloid / Q000097:blood / Q000235:genetics* / Q000628:therapy; D008297:Male; D011241:Prednisone / Q000008:administration & dosage; D013156:Splenectomy; D014750:Vincristine / Q000008:administration & dosage
D014750:Vincristine; D011241:Prednisone