title: 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.affiliation: Department of Internal Medicine, Hôpital Robert-Bisson, 4, rue Roger-Aini, 14100 Lisieux, France. hubertdeboysson@yahoo.fr
authors.forename: Hubert
authors.initials: H
authors.lastname: de Boysson
authors.forename: Stéphane
authors.initials: S
authors.lastname: Chèze
authors.forename: Françoise
authors.initials: F
authors.lastname: Chapon
authors.forename: Brigitte
authors.initials: B
authors.lastname: Le Mauff
authors.forename: Christophe
authors.initials: C
authors.lastname: Auzary
authors.forename: Loïk
authors.initials: L
authors.lastname: Geffray
mesh_terms: D004802:Eosinophilia / Q000150:complications* / Q000473:pathology*; D005205:Fascia / Q000473:pathology; D005208:Fasciitis / Q000150:complications* / Q000473:pathology*; D017809:Fatal Outcome; D006457:Hemoglobinuria, Paroxysmal / Q000209:etiology*; D006801:Humans; D015470:Leukemia, Myeloid, Acute / Q000150:complications*; D008297:Male; D008875:Middle Aged; D010198:Pancytopenia / Q000150:complications*