J Korean Med Sci.  2008 Apr;23(2):320-323. 10.3346/jkms.2008.23.2.320.

Complete Hematopoietic Recovery after Continuous Iron Chelation Therapy in a Patient with Severe Aplastic Anemia with Secondary Hemochromatosis

• Park SJ
• Han CW

• KMID: 1713468
• DOI: http://doi.org/10.3346/jkms.2008.23.2.320

Abstract

A 16-yr-old male patient with hemochromatosis due to multiple packed red blood cell transfusions was referred to our emergency center for the treatment of severe aplastic anemia and dyspnea. He was diagnosed with aplastic anemia at 11-yr of age. He had received continuous transfusions because an HLA-matched marrow donor was unavailable. Following a continuous, approximately 5-yr transfusion, he was noted to develop hemochromatosis. He had a dilated cardiomyopathy and required diuretics and digitalis, multiple endocrine and liver dysfunction, generalized bleeding, and skin pigmentation. A total volume of red blood cell transfusion before deferoxamine therapy was about 96,000 mL. He received a regular iron chelation therapy (continuous intravenous infusion of deferoxamine, 50 mg/kg/day for 5 days q 3-4 weeks) for approximately seven years after the onset of multiple organ failures. His cytopenia and organ dysfunctions began to be gradually recovered since about 2002, following a 4-yr deferoxamine treatment. He showed completely normal ranges of peripheral blood cell counts, heart size, and liver function two years ago. He has not received any transfusions for the last four years. This finding suggests that a continuous deferoxamine infusion may play a role in the immune regulation in addition to iron chelation effect.

Keyword

Aplastic Anemia; Hemochromatosis; Deferoxamine

MeSH Terms

Adolescent
Anemia, Aplastic/pathology/*therapy
Chelation Therapy/*methods
Deferoxamine/therapeutic use
Erythrocyte Transfusion
Hemochromatosis/*complications/therapy
Humans
Immune System
Iron/*therapeutic use
Iron Chelating Agents/therapeutic use
Male
Radiography, Thoracic/methods
Time Factors
Treatment Outcome

Figure

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  Fig. 1 Posteroanterior chest radiographs reveal a marked cardiomegaly with right pleural effusion before the treatment (A) and a normalized heart after the deferoxamine therapy (B).

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  Fig. 2 Hemoglobin changes before and after deferoxamine therapy.

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  Fig. 3 The full clinical course of the patient: Pre- and post-deferoxamine therapy. ALG, anti-lymphocyte globulin; EPO, erythropoietin; PRC, packed red blood cell; PC, platelet concentrate; CR, complete response.

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