Acute myeloid leukemia associated with hemophagocytic syndrome and t(4;7)(q21;q36)

Hemophagocytic syndrome (HS) is a histiocytic reactive process often associ ated with infections and/or malignancies. Clonal karyotypic abnormalities h ave been the hallmark of several hematological malignancies and have been s hown to be of clinical significance in terms of both diagnosis and prognosi s. While there are limited reports of both clonal and nonclonal abnormaliti es in HS, their clinical significance has not been established. Detection o f such clonal abnormalities, as seen in some cases of HS, may indicate the presence of an occult malignant process, even when there is no microscopic evidence of a hematological malignancy. We report a case of HS in a child w ith clonal t(4;7)(q21;q36) which later progressed to acute myeloid leukemia (AML) with further clonal evolution. Our case strengthens the argument tha t cytogenetic studies in HS may be important in identifying the underlying occult malignant process. (C) 2000 Elsevier Science Inc. All rights reserve d.