research paper
Intrinsically impaired platelet production in some patients with persistent or chronic immune thrombocytopenia
Etienne Riviere,1,2,3 Jean-Francßois Viallard,1,2,3 Alexandre Guy,1,2,3 Badr Kilani,2,3 Juliana Vieira-Dias,2,3 Anne C. Pons,4 Thierry Couffinhal,2,3 Jean-Luc Pellegrin1 and Chloe James2,3,4 1
Internal Medicine Department, Bordeaux Uni-
versity Hospital Centre, 2Adaptation Cardiovasculaire a l’Ischemie, University of Bordeaux, Adaptation Cardiovasculaire a l’Ischemie,
3
INSERM, and 4Laboratory of Haematology, Bordeaux University Hospital Centre, Pessac, France Received 19 November 2014; accepted for publication 9 March 2015 Correspondence: Chloe James, INSERM U1034, Haut-Leveque Hospital, Bordeaux University Hospital Centre, 1, avenue Magellan, Pessac 33600, France. E-mail: [email protected]
Summary 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 9 109/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: thrombocytopenia, immune thrombocytopenia, platelet, megakaryocyte, megakaryocytopoiesis.
Primary immune thrombocytopenia (ITP) is an autoimmune disorder characterized by a platelet count
Intrinsically impaired platelet production in some patients with persistent or chronic immune thrombocytopenia
Etienne Riviere,1,2,3 Jean-Francßois Viallard,1,2,3 Alexandre Guy,1,2,3 Badr Kilani,2,3 Juliana Vieira-Dias,2,3 Anne C. Pons,4 Thierry Couffinhal,2,3 Jean-Luc Pellegrin1 and Chloe James2,3,4 1
Internal Medicine Department, Bordeaux Uni-
versity Hospital Centre, 2Adaptation Cardiovasculaire a l’Ischemie, University of Bordeaux, Adaptation Cardiovasculaire a l’Ischemie,
3
INSERM, and 4Laboratory of Haematology, Bordeaux University Hospital Centre, Pessac, France Received 19 November 2014; accepted for publication 9 March 2015 Correspondence: Chloe James, INSERM U1034, Haut-Leveque Hospital, Bordeaux University Hospital Centre, 1, avenue Magellan, Pessac 33600, France. E-mail: [email protected]
Summary 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 9 109/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: thrombocytopenia, immune thrombocytopenia, platelet, megakaryocyte, megakaryocytopoiesis.
Primary immune thrombocytopenia (ITP) is an autoimmune disorder characterized by a platelet count
