Steroid-responsive Anemia in Patients of Ghosal Hematodiaphyseal Dysplasia
- 1 May 2015
- journal article
- case report
- Published by Ovid Technologies (Wolters Kluwer Health) in Journal of Pediatric Hematology/Oncology
- Vol. 37 (4), 285-289
- https://doi.org/10.1097/mph.0000000000000279
Abstract
Ghosal hematodiaphyseal dysplasia (GHDD) is a recently recognized cause of steroid-responsive anemia. We would like to report 3 cases of GHDD who presented in early childhood with moderate to severe anemia, splenomegaly, and a hypocellular marrow with increased reticulin. They were easily diagnosed with long-bone x-rays showing diaphyseal and metaphyseal widening and loss of diaphyseal constriction. All cases dramatically responded to oral steroid and no longer needed blood transfusion. They required steroid at low doses for long term (up to 5 y). GHDD is easy to diagnose with long-bone radiography and consistently responds to steroid. It should therefore be considered as a differential diagnosis of unusual anemia in early childhood, especially in children from the Middle East or the Indian subcontinent.Keywords
This publication has 12 references indexed in Scilit:
- Ghosal Hematodiaphyseal Dysplasia with MyelofibrosisIndian Journal of Pediatrics, 2012
- Ghosal hematodiaphyseal dysplasia: A rare cause of a myelophthisic anemiaPediatric Blood & Cancer, 2010
- Ghosal type hemato-diaphyseal dysplasia: A rare variety of Engelmann’s diseaseIndian Journal of Pediatrics, 2007
- A gene responsible for Ghosal hemato-diaphyseal dysplasia maps to chromosome 7q33–34Human Genetics, 2007
- Un nouveau cas de dysplasie hématodiaphysaire de GhosalArchives de Pédiatrie, 2005
- About hemato-diaphyseal dysplegia.Pediatric Hematology and Oncology, 2004
- Early Manifestation of Ghosal-Type Hemato-Diaphyseal DysplasiaPediatric Hematology and Oncology, 2003
- Ghosal haemato-diaphyseal dysplasia: A new disorderEuropean Journal of Pediatrics, 1993
- High-dose intravenous methylprednisolone therapy for anemia associated with diaphyseal dysplasiaThe Journal of Pediatrics, 1989
- Diaphyseal dysplasia associated with anemiaThe Journal of Pediatrics, 1988