Myasthenia gravis and paroxysmal nocturnal hemoglobinuria after thymectomy: A rare association

Autor: Jean Galtier, Fanny Duval, Irène Machelart, Carine Greib, Estibaliz Lazaro, Jean‐Luc Pellegrin, Jean‐François Viallard, Regis Peffault de laTour, Etienne Rivière
Jazyk: angličtina
Rok vydání: 2021
Předmět:
Zdroj: eJHaem, Vol 2, Iss 4, Pp 834-837 (2021)
Druh dokumentu: article
ISSN: 2688-6146
DOI: 10.1002/jha2.244
Popis: Abstract Paroxysmal nocturnal hemoglobinuria (PNH) is a very rare clonal autoimmune disease manifesting with hemolysis, thrombosis, or bone marrow failure. We present an atypical association of myasthenia gravis, aplastic anemia, and PNH occurring years after thymectomy. While this association might be extremely rare, it may not be coincidental as there is a common pathophysiology between PNH and aplastic anemia, with the latter reported in several thymoma/thymectomy cases. Eculizumab was introduced with good efficacy and without safety concern in our patient, leading to long‐term control of PNH without worsening of myasthenia gravis.
Databáze: Directory of Open Access Journals