Prepubertal hypertransfusion in talassemia intermedia

A case report of sustained positive efects on growth, splenic function and endocrine parameters

Surekha Tony, Shahina Daar, Mathew Zachariah, Yasser Wali

Research output: Contribution to journalArticle

Abstract

We report a known case of thalassemia intermedia (Hb Dhofar) who in spite of mild thalassemic facies, attained his normal genetic height, pubertal maturity and improved self-image with minimal splenomegaly through a hypertransfusion/chelation regimen that was started just before puberty and maintained for 5 years. As there are no clear guidelines in the management of patients with thalassemia intermedia, the option of hypertransfusion/ chelation during the pubertal growth spurt may alleviate some of the complications associated with thalassemia intermedia.

Original languageEnglish
JournalOman Medical Journal
Volume27
Issue number6
DOIs
Publication statusPublished - 2012

Fingerprint

beta-Thalassemia
Growth
Splenomegaly
Puberty
Guidelines

Keywords

  • Hypertransfusion
  • Prepuberty
  • Talassemia intermedia

ASJC Scopus subject areas

  • Medicine(all)

Cite this

Prepubertal hypertransfusion in talassemia intermedia : A case report of sustained positive efects on growth, splenic function and endocrine parameters. / Tony, Surekha; Daar, Shahina; Zachariah, Mathew; Wali, Yasser.

In: Oman Medical Journal, Vol. 27, No. 6, 2012.

Research output: Contribution to journalArticle

@article{b3faf4450c6d4ca8aea7341e05118c4d,
title = "Prepubertal hypertransfusion in talassemia intermedia: A case report of sustained positive efects on growth, splenic function and endocrine parameters",
abstract = "We report a known case of thalassemia intermedia (Hb Dhofar) who in spite of mild thalassemic facies, attained his normal genetic height, pubertal maturity and improved self-image with minimal splenomegaly through a hypertransfusion/chelation regimen that was started just before puberty and maintained for 5 years. As there are no clear guidelines in the management of patients with thalassemia intermedia, the option of hypertransfusion/ chelation during the pubertal growth spurt may alleviate some of the complications associated with thalassemia intermedia.",
keywords = "Hypertransfusion, Prepuberty, Talassemia intermedia",
author = "Surekha Tony and Shahina Daar and Mathew Zachariah and Yasser Wali",
year = "2012",
doi = "10.5001/omj.2012.125",
language = "English",
volume = "27",
journal = "Oman Medical Journal",
issn = "1999-768X",
publisher = "Oman Medical Specialty Board",
number = "6",

}

TY - JOUR

T1 - Prepubertal hypertransfusion in talassemia intermedia

T2 - A case report of sustained positive efects on growth, splenic function and endocrine parameters

AU - Tony, Surekha

AU - Daar, Shahina

AU - Zachariah, Mathew

AU - Wali, Yasser

PY - 2012

Y1 - 2012

N2 - We report a known case of thalassemia intermedia (Hb Dhofar) who in spite of mild thalassemic facies, attained his normal genetic height, pubertal maturity and improved self-image with minimal splenomegaly through a hypertransfusion/chelation regimen that was started just before puberty and maintained for 5 years. As there are no clear guidelines in the management of patients with thalassemia intermedia, the option of hypertransfusion/ chelation during the pubertal growth spurt may alleviate some of the complications associated with thalassemia intermedia.

AB - We report a known case of thalassemia intermedia (Hb Dhofar) who in spite of mild thalassemic facies, attained his normal genetic height, pubertal maturity and improved self-image with minimal splenomegaly through a hypertransfusion/chelation regimen that was started just before puberty and maintained for 5 years. As there are no clear guidelines in the management of patients with thalassemia intermedia, the option of hypertransfusion/ chelation during the pubertal growth spurt may alleviate some of the complications associated with thalassemia intermedia.

KW - Hypertransfusion

KW - Prepuberty

KW - Talassemia intermedia

UR - http://www.scopus.com/inward/record.url?scp=84873293675&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=84873293675&partnerID=8YFLogxK

U2 - 10.5001/omj.2012.125

DO - 10.5001/omj.2012.125

M3 - Article

VL - 27

JO - Oman Medical Journal

JF - Oman Medical Journal

SN - 1999-768X

IS - 6

ER -