Megakaryoblastic Leukemia and Transient Abnormal Myelopoiesis with Down's Syndrome
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- SUDA Junko
- The Second Department of Pediatrics, Dokkyo University, School of Medicine
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- EGUCHI Mitsuoki
- The Second Department of Pediatrics, Dokkyo University, School of Medicine
Bibliographic Information
- Other Title
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- 小児巨核球性白血病とDown症候群の関係
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Abstract
Transient abnormal myelopoiesis (TAM) accompanied by Down's syndrome has been characterized as exhibiting self-limiting hematological abnormalities.<br>We studied 8 patients suffering from this disorder in order to clarify the biological nature of their blast cells. The blast cells showed positive reactions for platelet peroxidase (PPO) and with monoclonal antibodies against platelet-megakaryocyte antigen (TP80 and TP82). Furthermore, ultrastractural studies of blast cells showed the presence of α-granules, θ-granules, ferritin granules and/or basophilic granules. These were not observed in megakaryoblastic cells of patients without Down's syndrome. In methylcellulose and liquid culture systems, high plating efficencies were observed, and various mature cells, mainly basophils developed in the presence of phytohemagglutinin stimulated leucocyte conditioned medium.<br>Chromosomal analysis of blast cells of the patient with constitutionally trisomy 21 mosaic (46, X, Y=89/47, X, Y, +21=11) showed exclusively trisomy 21. Simultaneous examination for morphology and chromosomal analysis on single colonies revealed that granulocyte-macrophage colonies and an erythroid colony contained only cells with trisomy 21 karyotype. In two other patients with TAM, we observed one or two sublines in addition to the stem line with trisomy 21. There might exist clonal instability and be a sign of leukemic transition.<br>These findings suggest that TAM represents a selective expansion of chromosome 21 trisomic cells, which often show PPO-positive reaction and a high capacity for differentiation into, particularly, basophils.
Journal
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- Rinsho Ketsueki
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Rinsho Ketsueki 29 (8), 1200-1206, 1988
The Japanese Society of Hematology
