Intrinsically impaired platelet production in some patients with persistent or chronic immune thrombocytopenia

Br J Haematol. 2015 Aug;170(3):408-15. doi: 10.1111/bjh.13444. Epub 2015 Apr 14.

Abstract

Persistent or chronic immune thrombocytopenias (P/C-ITP) are acquired blood disorders lasting more than 3 months or 1 year, respectively. The pathogenesis of these disorders is thought to be immunological. We hypothesized that some patients with P/C-ITP might have an intrinsic megakaryopoiesis defect. We identified a group of P/C-ITP patients with acquired isolated mild thrombocytopenia (30-100 × 10(9) /l), undetectable anti-platelet antibodies, negative autoimmune investigations and no need for treatment. We examined in vitro megakaryocyte differentiation and compared these patients' results with those of acute-ITP patients and healthy controls. No difference in proliferation, ploidy or expression of surface markers was found. In contrast, P/C-ITP patients had significantly fewer proplatelet-forming megakaryocytes. This novel observation demonstrated that some patients diagnosed with P/C-ITP have an intrinsic megakaryopoiesis defect independent of the bone-marrow environment. Further investigations are needed to dissect mechanisms underlying this impaired proplatelet formation in these patients.

Keywords: immune thrombocytopenia; megakaryocyte; megakaryocytopoiesis; platelet; thrombocytopenia.

Publication types

  • Clinical Trial

MeSH terms

  • Adult
  • Aged
  • Autoantibodies / immunology*
  • Blood Platelets / immunology*
  • Blood Platelets / pathology
  • Cell Differentiation / immunology*
  • Chronic Disease
  • Female
  • Humans
  • Male
  • Megakaryocytes / immunology*
  • Megakaryocytes / pathology
  • Middle Aged
  • Myelopoiesis / immunology*
  • Purpura, Thrombocytopenic, Idiopathic / diagnosis
  • Purpura, Thrombocytopenic, Idiopathic / immunology*
  • Purpura, Thrombocytopenic, Idiopathic / pathology

Substances

  • Autoantibodies