[Guidelines for diagnosis and treatment of secondary iron overload in patients with congenital anemia]

Klin Padiatr. 2010 Nov;222(6):399-406. doi: 10.1055/s-0030-1265178. Epub 2010 Sep 22.
[Article in German]

Abstract

In Germany and Central Europe, congenital disorders leading to secondary hemochromatosis are rare. The majority of these patients are treated in peripheral medical institutions. As a consequence, the experience of each institution in the treatment of secondary hemochromatosis in patients with congenital anemia is limited. Recent developments concerning new chelating agents, their combination for intensified chelation and new possibilities to diagnose and monitor iron overload have important consequences for the management of patients with secondary hemochromatosis and increase its complexity enormously. Therefore, the development of a guideline for rational and efficient diagnostics and treatment was necessary. The new guideline was developed within a formal consensus process and finally approved by a consensus conference with participants from both the pediatric and adult German hematology societies (GPOH and DGHO). Apart from general information and recommendations, the guideline contains 9 consensus statements on diagnostics (iron status, siderotic complications, chelator side-effects), the start of chelation, indications for intensified chelation, iron elimination in specific disorders, and iron elimination after stem cell transplantation. Here, these consensus statements are presented and discussed in detail. For the complete text of the guideline, please visit the AWMF homepage at http://www.leitlinien.net .

Publication types

  • Consensus Development Conference
  • English Abstract
  • Practice Guideline

MeSH terms

  • Anemia, Aplastic / blood
  • Anemia, Aplastic / therapy*
  • Anemia, Diamond-Blackfan / blood
  • Anemia, Diamond-Blackfan / therapy
  • Anemia, Dyserythropoietic, Congenital / blood
  • Anemia, Dyserythropoietic, Congenital / therapy*
  • Anemia, Sickle Cell / blood
  • Anemia, Sickle Cell / therapy
  • Chelating Agents / adverse effects
  • Chelating Agents / therapeutic use*
  • Child
  • Deferoxamine / adverse effects
  • Deferoxamine / therapeutic use
  • Erythrocyte Transfusion / adverse effects*
  • Ferritins / blood
  • Germany
  • Hematopoietic Stem Cell Transplantation / adverse effects
  • Hemochromatosis / blood
  • Hemochromatosis / diagnosis
  • Hemochromatosis / drug therapy*
  • Hemosiderosis / blood
  • Hemosiderosis / diagnosis
  • Hemosiderosis / drug therapy*
  • Humans
  • beta-Thalassemia / blood
  • beta-Thalassemia / therapy

Substances

  • Chelating Agents
  • Ferritins
  • Deferoxamine