Eosinophilic fasciitis with paroxysmal nocturnal hemoglobinuria.

Abstract

Eosinophilic fasciitis is a rare connective tissue disorder, which can be associated with hematological complications in 10% of cases, such as aplastic anemia or acquired amegakaryocytic thrombocytopenia. Paroxysmal nocturnal hemoglobinuria had never been described in a patient suffering from eosinophilic fasciitis. We report an original case of a 59-year-old patient who developed a moderate aplastic pancytopenia while he was treated for a biopsy-proven eosinophilic fasciitis. A complete set of investigations was carried out and was found to be negative, including a first research of paroxysmal nocturnal hemoglobinuria. Two years after disease onset, while pancytopenia remained stable, occurrence of morning dark urine led to found a paroxysmal nocturnal hemoglobinuria clone. We discuss a potential link between the two conditions and hypothesize that paroxysmal nocturnal hemoglobinuria blood cells may pre-exist for a long time and take a survival advantage in the setting of marrow injury, as observed in eosinophilic fasciitis with hematological complications. We finally suggest that paroxysmal nocturnal hemoglobinuria should be included as a hematological complication of eosinophilic fasciitis.

• Authors: Hubert de Boysson, Stéphane Chèze, Françoise Chapon, Brigitte Le Mauff, Christophe Auzary, Loïk Geffray
• Journal: Joint bone spine (Joint Bone Spine) Vol. 80 Issue 2 Pg. 208-10 (Mar 2013) ISSN: 1778-7254 [Electronic] France
• PMID: 22999899 (Publication Type: Case Reports, Journal Article)
• Copyright: Copyright © 2012 Société française de rhumatologie. Published by Elsevier SAS. All rights reserved.

Topics

• Eosinophilia (complications, pathology)
• Fascia (pathology)
• Fasciitis (complications, pathology)
• Fatal Outcome
• Hemoglobinuria, Paroxysmal (etiology)
• Humans
• Leukemia, Myeloid, Acute (complications)
• Male
• Middle Aged
• Pancytopenia (complications)