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Ghosal hematodiaphyseal dysplasia: A rare cause of a myelophthisic anemia
Author(s) -
Mazaheri Parisa,
Nadkarni Girish,
Lowe Elizabeth,
Hines Patrick,
Vuica Melena,
Griffin Matthew,
Resar Linda M.S.
Publication year - 2010
Publication title -
pediatric blood and cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.116
H-Index - 105
eISSN - 1545-5017
pISSN - 1545-5009
DOI - 10.1002/pbc.22662
Subject(s) - medicine , anemia , dysplasia , pediatrics , dermatology
Ghosal hematodiaphyseal dysplasia syndrome (GHDD) is a rare clinical syndrome characterized by increased bone density and a severe, myelophthisic anemia. Few cases have been reported worldwide and there are no detailed descriptions of the associated hematologic abnormalities and long‐term clinical outcomes after treatment. Here, we report two siblings with GHDD who were successfully treated with chronic, low dose, corticosteroid therapy. Although GHDD is uncommon, these cases illustrate the need to consider GHDD in patients with anemia and bone dysplasia and the use of chronic, low‐dose steroid therapy. Pediatr Blood Cancer. 2010;55:1187–1190. © 2010 Wiley‐Liss, Inc.