タラセミアに伴うIgA腎症の1例

IgA nephropathy associated with thalassemia: a case report.

• Jun Ni
• Caifeng Zhu
• Xiaoqiu Ni
• Jiazhen Yin

抄録

背景:

タラセミアは、共通の劣性単発性血液疾患を特徴とする遺伝性疾患群であり、発展途上国において公衆衛生上の重要な関心事である。最近の研究では、タラセミア症候群の腎への影響が明らかになってきた。慢性低酸素血症、長期貧血、鉄過多、鉄キレート剤による腎尿細管機能障害や糸球体濾過異常が主な原因とされているが、糸球体腎炎は尿検査異常の主な原因とは考えられていない。

BACKGROUND: Thalassemia is a group of hereditary diseases characterized by a common recessive monogenic hematological disorder, presenting a significant public health concern in the developing countries. Recent studies have identified the renal effects of thalassemia syndrome. Chronic hypoxia, long-term anemia, iron overload, and iron chelators are the major causes of renal tubular dysfunction and glomerular filtration abnormalities, while glomerulonephritis is not considered a major cause of abnormal urinalysis.

事例紹介:

貧血を伴う免疫グロブリンA（IgA）腎症を呈した38歳女性患者の1例を報告したが，当初は誤診であったが，遺伝子検査の結果，α-タラセミアと診断された。プレドニゾロン，レフルノミド，アンジオテンシン受容体拮抗薬の内服と葉酸，メコバラミンの併用投与を行った．経過観察中に蛋白尿が有意に減少し，貧血も改善した。

CASE PRESENTATION: We report a case of a 38-year-old female patient with immunoglobulin A (IgA) nephropathy accompanied by anemia who was misdiagnosed initially, but was diagnosed with alpha-thalassemia after gene tests. We administered a combination of oral prednisolone, leflunomide, and angiotensin receptor blockers as well as folic acid and mecobalamin. During the follow-up, her proteinuria was significantly reduced, and her anemia was improved.

結論:

難治性貧血を合併したIgA腎症、特に発症率の高い地域では、タラセミアの発生を考慮すべきである。

CONCLUSIONS: The possibility of occurrence of thalassemia should be considered in IgA nephropathy complicated with refractory anemia, especially in high-incidence areas of the disease.