Childhood hemophagocytic syndrome associated with Kikuchi's disease.

Haematologica vol. 85(9):September 2000 was negative.The patient was treated with hydroxyurea, which decreased the size of his spleen and the number of peripheral eosinophils.A few months later he underwent an unrelated bone marrow transplantation and was in remission a year later. The diagnosis of CEL does not fit exactly the criteria proposed by Brito-Babapulle, since our patient has marked myelodysplasia. However the presence of fibrosis, hypercellularity in bone marrow, hepatosplenomegaly and the good response to hydroxyurea make diagnoses other than myeloproliferative disorder difficult. The clonality of eosinophils, one of the key criteria could not be established directly since the detected cytogenetic alteration turned out to be constitutional. He fulfills other criteria proposed by BritoBabapulle: major (medullary fibrosis, trilineage dysplasia, presence of ALIP) and minor (splenomegaly, normal levels of Ig, marked morphologic abnormalities of eosinophils). In resemblance of AML M4 Eo, in which the clonality of the eosinophils is well established, the cytochemical abnormalities of these cells are the same as in our case.4 Finally, the presence of a constitutional cytogenetic alteration not previously described associated with any hematological disease raises two questions: does an uncommon traslocation have a possible pathogenic role in an also uncommon disease; and any undescribed cytogenetic alteration must be discarded as constitutional.7