Masuzawa Naoko, Nishimura Ayako, Kitani Takashi, Tamagaki Keiichi, Sugitani Mio, Nagoshi Hisao, Kuroda Junya, Konishi Eiichi
Division of Surgical Pathology, Department of Pathology, Kyoto Prefectural University of Medicine, Kyoto, Japan.
Department of Diagnostic Pathology, Otsu City Hospital, Shiga, Japan.
Pathol Int. 2017 Dec;67(12):620-625. doi: 10.1111/pin.12589. Epub 2017 Sep 20.
Nephrotic syndrome (NS) rarely occurs in post-hematopoietic stem cell transplantation (HSCT) recipients but represents the renal manifestation of graft-versus-host disease (GVHD). Membranous nephropathy (MN) accounts for almost two thirds of post-HSCT NS and is caused by immune complex deposition. Renal thrombotic microangiopathy (TMA) without fulfillment of clinical criteria for TMA has been underreported because of reduced opportunity for histological examination. However, renal TMA has recently been reported in association with GVHD and humoral immunological reactions. Although both MN and TMA after HSCT are associated with GVHD and immunological abnormalities, these diseases are exceptionally coexistent in renal biopsy specimens. We herein describe a case of post-HSCT NS, histologically showing overlapped lesions of TMA and MN. Renal biopsy specimen after presentation of NS revealed early stage MN and TMA with evidence of chronicity. TMA was thought to have preceded MN, and renal biopsy at the phase of pre-nephrotic proteinuria might reveal earlier histological changes of isolated renal TMA. Detection of subclinical renal TMA earlier by spontaneous renal biopsy can help prevent progression of renal injury or overlapping of other renal pathologies. We also demonstrated Th2 predominant intraglomerular infiltration of lymphocytes by immunohistochemistry.
肾病综合征(NS)在造血干细胞移植(HSCT)受者中很少见,但却是移植物抗宿主病(GVHD)的肾脏表现。膜性肾病(MN)几乎占HSCT后NS的三分之二,由免疫复合物沉积引起。由于组织学检查机会减少,未满足血栓性微血管病(TMA)临床标准的肾血栓性微血管病报道较少。然而,最近有报道称肾TMA与GVHD和体液免疫反应有关。虽然HSCT后的MN和TMA都与GVHD和免疫异常有关,但这些疾病在肾活检标本中异常共存。我们在此描述一例HSCT后NS病例,组织学显示TMA和MN的重叠病变。NS出现后的肾活检标本显示早期MN和TMA并有慢性病变证据。认为TMA先于MN出现,在肾病综合征前期蛋白尿阶段进行肾活检可能会发现孤立性肾TMA的早期组织学变化。通过自发性肾活检更早地检测亚临床肾TMA有助于预防肾损伤进展或其他肾脏病理改变的重叠。我们还通过免疫组化证明肾小球内淋巴细胞以Th2为主浸润。