Severe aplastic anemia associated with eosinophilic fasciitis: report of 4 cases and review of the literature.

Abstract

Diffuse eosinophilic fasciitis (Shulman disease) is a rare sclerodermiform syndrome that, in most cases, resolves spontaneously or after corticosteroid therapy. It has been associated with hematologic disorders, such as aplastic anemia. The clinical features and long-term outcomes of patients with eosinophilic fasciitis and associated aplastic anemia have been poorly described. We report the cases of 4 patients with eosinophilic fasciitis and associated severe aplastic anemia. For 3 of these patients, aplastic anemia was refractory to conventional immunosuppressive therapy with antithymocyte globulin and cyclosporine. One of the patients received rituximab as a second-line therapy with significant efficacy for both the skin and hematologic symptoms. To our knowledge, this report is the first to describe rituximab used to treat eosinophilic fasciitis with associated aplastic anemia. In a literature review, we identified 19 additional cases of eosinophilic fasciitis and aplastic anemia. Compared to patients with isolated eosinophilic fasciitis, patients with eosinophilic fasciitis and associated aplastic anemia were more likely to be men (70%) and older (mean age, 56 yr; range, 18-71 yr). Corticosteroid-containing regimens improved skin symptoms in 5 (42%) of 12 cases but were ineffective in the treatment of associated aplastic anemia in all but 1 case. Aplastic anemia was profound in 13 cases (57%) and was the cause of death in 8 cases (35%). Only 5 patients (22%) achieved long-term remission (allogeneic hematopoietic stem cell transplantation: n = 2; cyclosporine-containing regimen: n = 2; high-dose corticosteroid-based regimen: n = 1).

• Authors: Adèle de Masson, Jean-David Bouaziz, Régis Peffault de Latour, Ygal Benhamou, Cécile Moluçon-Chabrot, Jacques-Olivier Bay, Annie Laquerrière, Jean-Michel Picquenot, David Michonneau, Vanessa Leguy-Seguin, Michel Rybojad, Bernard Bonnotte, Fabrice Jardin, Hervé Lévesque, Martine Bagot, Gérard Socié
• Journal: Medicine (Medicine (Baltimore)) Vol. 92 Issue 2 Pg. 69-81 (Mar 2013) ISSN: 1536-5964 [Electronic] United States
• PMID: 23429351 (Publication Type: Case Reports, Journal Article, Review)

Topics

• Adult
• Aged
• Anemia, Aplastic (diagnosis, drug therapy, etiology, mortality)
• Eosinophilia (complications, diagnosis, drug therapy, mortality)
• Fasciitis (complications, diagnosis, drug therapy, mortality)
• Female
• Humans
• Male
• Middle Aged
• Retrospective Studies
• Treatment Failure