Thrombopoietin level is inversely related to blast count, not platelet number, in Down syndrome neonates with transient myeloproliferative disorder

Motoki Bonno, Eiichi Azuma, Hajime Kawasaki, Xao Li Zhang, Yoshihiro Komada, Masahiro Hirayama, Masamune Higashigawa, Masakazu Umemoto, Tadashi Koike, Takashi Kato, Tomoyuki Tahara, Hiroshi Miyazaki, Minoru Sakurai

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12 Citations (Scopus)

Abstract

Transient myeloproliferative disorder (TMD) in neonates with Down syndrome is characterized by increased megakaryoblastic cells in the peripheral blood. Despite their spontaneous regression in weeks, prognosis is not always favorable because of fatal hepatic fibrosis. In this study, blood thrombopoietin (TPO) levels were measured by ELISA in six TMD patients and the expression of c-Mpl, a ligand for TPO, was examined on the blast cells from four patients by flow cytometer. At the onset, TPO level was undetectable in one patient and significantly lower in five patients than six neonatal controls (mean 0.52 fmol/ml, range 0.30-0.93 vs. 3.70, 1.38-8.33, P < 0.001), although platelet counts were similar (mean 321 x 109/l, range 42- 1,040 vs. 253 x 109/l, 124-381). Two patients died of hepatic failure. TPO levels were measured in five patients after regression of the blast cells. With regression of blast cells, TPO levels were remarkably increased in four survived patients. In one patient with hepatic failure, TPO level was poorly elevated and relatively lower compared to the others. TPO levels were inversely correlated with blast numbers (r = -0.85, P < 0.001), but not with platelet counts (r = 0.426). Blast cells from four patients were all positive for c-Mpl. Our findings suggest that megakaryocyte mass is a major regulator of TPO levels and hepatic failure may affect the TPO level because liver is a major source of TPO production.

Original languageEnglish
Pages (from-to)267-272
Number of pages6
JournalAmerican Journal of Hematology
Volume58
Issue number4
DOIs
Publication statusPublished - 1998 Aug 1
Externally publishedYes

Keywords

  • C-Mpl
  • Down syndrome
  • Megakaryopoiesis
  • Thrombopoietin
  • Transient myeloproliferative disorder (TMD)

ASJC Scopus subject areas

  • Hematology

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    Bonno, M., Azuma, E., Kawasaki, H., Zhang, X. L., Komada, Y., Hirayama, M., Higashigawa, M., Umemoto, M., Koike, T., Kato, T., Tahara, T., Miyazaki, H., & Sakurai, M. (1998). Thrombopoietin level is inversely related to blast count, not platelet number, in Down syndrome neonates with transient myeloproliferative disorder. American Journal of Hematology, 58(4), 267-272. https://doi.org/10.1002/(SICI)1096-8652(199808)58:4<267::AID-AJH3>3.0.CO;2-O