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An intriguing hematological disorder is seen in neonates with Down's syndrome (DS). Although this disorder clinically and hematologically resembles acute leukemia, it is reported that most of patients spontaneously regressed over weeks or months, except for some cases which lead to leukemic progression. This type of disorder is termed transient leukemoid reaction, transient abnormal myelopoiesis, or transient myelo proliferative disorder, etc. Rosner et al. (1972) speculated that "ineffective regulation of granulopoiesis, erythropoiesis, and perhaps thrombopoiesis in patients with DS could produce a hematological picture which was similar to acute leukemia." Cosson et al. (1974) reported a case of acute megakaryocytic leukemia (FAB, M7) in a neonate with DS, and a number of similar cases have been reported subsequently. Cytogenetic abnormalities of chromosome 21 have been associated with some cases of M7 patients. However it is not confirmed that the abnormalities of chromosome 21 are specific for M7. In this paper, we discuss four newborn patients with DS, who developed transient myeloproliferative disorder (TMD) that showed megakaryocytic proliferation.

Megakaryocyte proliferative disorder in neonates with Down's syndrome.