Acta Haematologica

Case Report

Congenital Dyserythropoietic Anemia Type II Associated with G6PD Seattle in a Sicilian Child

Gangarossa S.a · Romano V.a · del Giudice E.M.b · Perrotta S.b · Iolascon A.b · Schiliro G.a

Author affiliations

aDivisione di Ematologia-Oncologia Pediatrica, Università di Catania, e bDipartimento di Pediatria, II Università di Napoli, Italia

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Acta Haematol 1995;93:36–39

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Article / Publication Details

First-Page Preview
Abstract of Case Report

Received: May 08, 1994
Accepted: October 10, 1994
Published online: February 18, 2009
Issue release date: 1995

Number of Print Pages: 4
Number of Figures: 0
Number of Tables: 0

ISSN: 0001-5792 (Print)
eISSN: 1421-9662 (Online)

For additional information: https://www.karger.com/AHA

Abstract

A 2-year-old Sicilian boy was investigated because of chronic nonspherocytic hemolytic anemia (CNSHA) associated with hepatosplenomegaly. Appropriate studies revealed deficiency of glucose-6-phosphate dehydrogenase type Seattle (G6PD Seattle). In addition, bone marrow morphology, serological studies and analysis of red cell membrane proteins revealed congenital dyserythropoietic anemia (CDA) type II (or HEMPAS). Because G6PD Seattle on its own does not cause CNSHA, we believe that the clinical manifestations in this patient are essentially due to the CDA type II abnormality. However, the coexistence of these two different red cell abnormalities may affect the clinical picture specifically by making CDA type II more hemolytic than it would have been otherwise.

© 1995 S. Karger AG, Basel




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Article / Publication Details

First-Page Preview
Abstract of Case Report

Received: May 08, 1994
Accepted: October 10, 1994
Published online: February 18, 2009
Issue release date: 1995

Number of Print Pages: 4
Number of Figures: 0
Number of Tables: 0

ISSN: 0001-5792 (Print)
eISSN: 1421-9662 (Online)

For additional information: https://www.karger.com/AHA


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