Indirect study of thrombopoiesis (TPO, reticulated platelets, glycocalicin) in patients with hereditary macrothrombocytopenia

F. Fabris, I. Cordiano, A. Steffan, R. Ramon, R. Scandellari, J. L. Nichol, A. Girolami

Research output: Contribution to journalArticlepeer-review

Abstract

Chronic isolated hereditary macrothrombocytopenia (CHMT) is a congenital form of macrothrombocytopenia that seems to be due to defective production secondary to a disturbance in megakaryocyte fragmentation. To better understand the pathogenesis of thrombopoiesis in this hereditary thrombocytopenic disorder, we determined the percentage of reticulated platelets (RP), plasma glycocalicin (GC) and thrombopoietin (TPO) levels in 29 patients with CHMT, 23 patients with immune thrombocytopenic purpura (ITP), and 17 patients with thrombocytopenia secondary to decreased bone marrow megakaryocytes (hypoplasia). The % RP was similar in CHMT (2.27 ± 1.33) and hypoplasia (1.98 ± 1.35) patients and markedly lower than that in ITP patients (8.80 ± 7.97; p <0.001), suggesting that the production of new platelets is reduced in CHMT. Plasma GC was within the normal range (0.84 ± 0.16 μg/mL) both in patients with CHMT (0.63 ± 0.20 μg/mL) and ITP (0.82 ± 0.90 μg/mL), while it was significantly decreased in patients with hypoplasia (0.16 ± 0.04 μg/mL; p

Original languageEnglish
Pages (from-to)151-156
Number of pages6
JournalEuropean Journal of Haematology
Volume64
Issue number3
DOIs
Publication statusPublished - 2000

Keywords

  • Glycocalicin
  • Macrothrombocytopenia
  • Reticulated platelets
  • Thrombopoietin

ASJC Scopus subject areas

  • Hematology

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