Intensive immunosuppression therapy for aplastic anemia associated with dyskeratosis congenita: Report of a case

Patrizia Comoli, Sabrina Basso, Guan Cheng Huang

Research output: Contribution to journalArticle

Abstract

Dyskeratosis congenita (DC) is a very rare inherited disorder characterized by skin pigmentation, nail dystrophy, and mucosal leukoplakia. It is also associated with a variety of noncutaneous abnormalities, such as fatal pulmonary complications, malignancy, and bone marrow failure. We report the case of a 32-year-old man with DC associated with severe aplastic anemia (SAA). The traditional treatment of DC-associated SAA is allogeneic hematopoietic stem cell transplantation (HSCT). However, in this case, an HLA-matched donor was not available. Therefore our patient was given intensive immunosuppressive therapy with antilymphocyte globulin (ALG) and cyclosporine A (CsA). The hemogram findings improved after the treatment, but the patient died of pulmonary complications after being in stable condition for 6 months. The results support the possible use of intensive immunosuppression with ALG and CsA for DC-associated SAA as an alternative treatment for patients who are not eligible for HSCT.

Original languageEnglish
Pages (from-to)35-37
Number of pages3
JournalInternational Journal of Hematology
Volume82
Issue number1
DOIs
Publication statusPublished - Jul 2005

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Keywords

  • Antilymphocyte globulin
  • Aplastic anemia
  • Dyskeratosis congenita
  • Immunosuppression therapy

ASJC Scopus subject areas

  • Hematology

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