Paroxysmal Nocturnal Hemoglobinuria in the Context of a Myeloproliferative Neoplasm: A Case Report and Review of the Literature

Paroxysmal nocturnal hemoglobinuria (PNH) is characterized by intravascular hemolytic anemia and thrombosis and is notoriously associated with aplastic anemia and myelodysplastic syndromes. Rarer associations include myeloproliferative neoplasms (MPNs), which are also burdened by increased thromboti...

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Autores principales:	Juri Alessandro Giannotta, Bruno Fattizzo, Wilma Barcellini
Formato:	article
Lenguaje:	EN
Publicado:	Frontiers Media S.A. 2021
Materias:	paroxysmal nocturnal hemoglobinuria myeloproliferative neoplasm complement inhibitors thrombosis bone marrow failure case report Neoplasms. Tumors. Oncology. Including cancer and carcinogens RC254-282
Acceso en línea:	https://doaj.org/article/2bd01cf112534f479256db26ca43b4a0
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spelling	oai:doaj.org-article:2bd01cf112534f479256db26ca43b4a02021-11-11T08:52:34ZParoxysmal Nocturnal Hemoglobinuria in the Context of a Myeloproliferative Neoplasm: A Case Report and Review of the Literature2234-943X10.3389/fonc.2021.756589https://doaj.org/article/2bd01cf112534f479256db26ca43b4a02021-11-01T00:00:00Zhttps://www.frontiersin.org/articles/10.3389/fonc.2021.756589/fullhttps://doaj.org/toc/2234-943XParoxysmal nocturnal hemoglobinuria (PNH) is characterized by intravascular hemolytic anemia and thrombosis and is notoriously associated with aplastic anemia and myelodysplastic syndromes. Rarer associations include myeloproliferative neoplasms (MPNs), which are also burdened by increased thrombotic tendency. The therapeutic management of this rare combination has not been defined so far. Here, we describe a 62-year-old man who developed a highly hemolytic PNH more than 10 years after the diagnosis of MPN. The patient started eculizumab, obtaining good control of intravascular hemolysis but without amelioration of transfusion-dependent anemia. Moreover, we performed a review of the literature regarding the clinical and pathogenetic significance of the association of PNH and MPN. The prevalence of PNH clones in MPN patients is about 10%, mostly in association with JAK2V617F-positive myelofibrosis. Thrombotic events were a common clinical presentation (35% of subjects), sometimes refractory to combined treatment with cytoreductive agents, anticoagulants, and complement inhibitors. The latter showed only partial effectiveness in controlling hemolytic anemia and, due to the paucity of data, should be taken in consideration after a careful risk/benefit evaluation in this peculiar setting.Juri Alessandro GiannottaBruno FattizzoBruno FattizzoWilma BarcelliniFrontiers Media S.A.articleparoxysmal nocturnal hemoglobinuriamyeloproliferative neoplasmcomplement inhibitorsthrombosisbone marrow failurecase reportNeoplasms. Tumors. Oncology. Including cancer and carcinogensRC254-282ENFrontiers in Oncology, Vol 11 (2021)
institution	DOAJ
collection	DOAJ
language	EN
topic	paroxysmal nocturnal hemoglobinuria myeloproliferative neoplasm complement inhibitors thrombosis bone marrow failure case report Neoplasms. Tumors. Oncology. Including cancer and carcinogens RC254-282
spellingShingle	paroxysmal nocturnal hemoglobinuria myeloproliferative neoplasm complement inhibitors thrombosis bone marrow failure case report Neoplasms. Tumors. Oncology. Including cancer and carcinogens RC254-282 Juri Alessandro Giannotta Bruno Fattizzo Bruno Fattizzo Wilma Barcellini Paroxysmal Nocturnal Hemoglobinuria in the Context of a Myeloproliferative Neoplasm: A Case Report and Review of the Literature
description	Paroxysmal nocturnal hemoglobinuria (PNH) is characterized by intravascular hemolytic anemia and thrombosis and is notoriously associated with aplastic anemia and myelodysplastic syndromes. Rarer associations include myeloproliferative neoplasms (MPNs), which are also burdened by increased thrombotic tendency. The therapeutic management of this rare combination has not been defined so far. Here, we describe a 62-year-old man who developed a highly hemolytic PNH more than 10 years after the diagnosis of MPN. The patient started eculizumab, obtaining good control of intravascular hemolysis but without amelioration of transfusion-dependent anemia. Moreover, we performed a review of the literature regarding the clinical and pathogenetic significance of the association of PNH and MPN. The prevalence of PNH clones in MPN patients is about 10%, mostly in association with JAK2V617F-positive myelofibrosis. Thrombotic events were a common clinical presentation (35% of subjects), sometimes refractory to combined treatment with cytoreductive agents, anticoagulants, and complement inhibitors. The latter showed only partial effectiveness in controlling hemolytic anemia and, due to the paucity of data, should be taken in consideration after a careful risk/benefit evaluation in this peculiar setting.
format	article
author	Juri Alessandro Giannotta Bruno Fattizzo Bruno Fattizzo Wilma Barcellini
author_facet	Juri Alessandro Giannotta Bruno Fattizzo Bruno Fattizzo Wilma Barcellini
author_sort	Juri Alessandro Giannotta
title	Paroxysmal Nocturnal Hemoglobinuria in the Context of a Myeloproliferative Neoplasm: A Case Report and Review of the Literature
title_short	Paroxysmal Nocturnal Hemoglobinuria in the Context of a Myeloproliferative Neoplasm: A Case Report and Review of the Literature
title_full	Paroxysmal Nocturnal Hemoglobinuria in the Context of a Myeloproliferative Neoplasm: A Case Report and Review of the Literature
title_fullStr	Paroxysmal Nocturnal Hemoglobinuria in the Context of a Myeloproliferative Neoplasm: A Case Report and Review of the Literature
title_full_unstemmed	Paroxysmal Nocturnal Hemoglobinuria in the Context of a Myeloproliferative Neoplasm: A Case Report and Review of the Literature
title_sort	paroxysmal nocturnal hemoglobinuria in the context of a myeloproliferative neoplasm: a case report and review of the literature
publisher	Frontiers Media S.A.
publishDate	2021
url	https://doaj.org/article/2bd01cf112534f479256db26ca43b4a0
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Paroxysmal Nocturnal Hemoglobinuria in the Context of a Myeloproliferative Neoplasm: A Case Report and Review of the Literature

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