Fatal cardiac hemochromatosis in a patient with hereditary spherocytosis

Takeo Fujino, Shujiro Inoue, Shunsuke Katsuki, Taiki Higo, Tomomi Ide, Yoshinao Oda, Hiroyuki Tsutsui

Research output: Contribution to journalArticle

Abstract

A 31-year-old man was admitted to our hospital with atrial tachycardia and cardiogenic shock. He had been diagnosed with hereditary spherocytosis (HS) during childhood, but he never received any red blood cell transfusions. Right ventricular endomyocardial biopsy revealed multiple myocardial hemosiderin deposits, and he was diagnosed with cardiac hemochromatosis. In addition to the iron deposition in the heart, the loss of myocyte and severe interstitial fibrosis were present. His cardiac function did not improve even after the cardioversion for atrial tachycardia, and he suffered from recurrent heart failure. Despite intensive medical treatment for heart failure and arrhythmias in combination with iron chelation therapy, he eventually died of progressive and refractory heart failure. Hemochromatosis is a systemic disorder characterized by the excessive deposition of iron in multiple organs. The occurrence of hemochromatosis in HS is extremely rare, and previous reports have shown that the coexistence of heterozygosity for the HFE gene mutation in HS patients causes excess iron storage. The prognosis is poor due to progressive congestive heart failure and refractory arrhythmias. Here we report a rare case of fatal cardiac hemochromatosis associated with HS. The possibility of cardiac hemochromatosis needs to be considered in cases of heart failure or arrhythmia in patients with HS.

Original languageEnglish
Pages (from-to)427-430
Number of pages4
JournalInternational heart journal
Volume59
Issue number2
DOIs
Publication statusPublished - Jan 1 2018

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Hereditary Spherocytosis
Hemochromatosis
Heart Failure
Iron
Cardiac Arrhythmias
Tachycardia
Chelation Therapy
Hemosiderin
Erythrocyte Transfusion
Electric Countershock
Cardiogenic Shock
Treatment Failure
Muscle Cells
Fibrosis
Biopsy
Mutation
Genes

All Science Journal Classification (ASJC) codes

  • Cardiology and Cardiovascular Medicine

Cite this

Fatal cardiac hemochromatosis in a patient with hereditary spherocytosis. / Fujino, Takeo; Inoue, Shujiro; Katsuki, Shunsuke; Higo, Taiki; Ide, Tomomi; Oda, Yoshinao; Tsutsui, Hiroyuki.

In: International heart journal, Vol. 59, No. 2, 01.01.2018, p. 427-430.

Research output: Contribution to journalArticle

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