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형질 세포와 근위 신세관 세포내 Auer rod양 봉입체를 함유한판코니 증후군 동반 IgA kappa형 경쇄침착질환 1예 보고

A Case of IgA kappa Light Chain Deposition Disease and Combined Adult Fanconi Syndromewith Auer rod-like Intracytoplasmic Inclusions in Plasma Cells and Proximal Renal Tubular Cells

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참고문헌

주제분류병리학

초록

저자들은 54세 여자 환자에서 골수의 형질세포 세포질과 신세관 상피세포내에 Auer rod와 흡사한 형태의 방추형 또는 바늘 모양의 봉입체를 갖고있는, Fanconi 증후군을 동반한 IgA kappa형경쇄침착질환 사례를 경험하였다. 세포화학 염색에서 봉입체들은acid phosphatase 염색에 강양성을 나타내었으며, -naphthylacetate esterase 음성이었고, 면역염색 결과 IgA-kappa형임을알 수 있었다. 신장 생검 결과 역시 kappa형 경쇄에 의한 벤스-존스 신증이 확인되었으며, Congo red 음성이었다. 전자현미경하에서 근위 신세관 상피세포내에 위치한 다수의 바늘과 같은 봉입체들을 관찰할 수 있었다.

참고문헌 (20)

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