Myelofibrosis, myeloproliferative syndrome and monosomy C in children

Abstract

We have studied six cases presenting as myeloproliferative disease with myelofibrosis in children and compared our findings with those of other investigators. Besides the classic chronic juvenile or adult type of leukemia, two groups of myeloproliferative syndrome are most often seen in pediatric practice: (1) myeloproliferative disease associated with monosomy C, which has clear-cut clinical features and a poor prognosis, with bone marrow grafting the only potentially successful treatment at this time; and, (2) idiopathic myelofibrosis with normal karyotype, which occurs less frequently and seems to have less distinct boundaries. We conclude that determination of karyotype is mandatory in these syndromes.

Key Words:

• Children
• myelofibrosis
• myeloproliferative disease