Beltran Manuel, Khurana Sharad, Gil Yennifer, Lewis Jason T, Kumar Rohit, Foran James M
Division of General Internal Medicine, Mayo Clinic, Jacksonville, FL, USA.
Division of Hematology & Oncology, University of Arizona Cancer Center, Tucson, AZ, USA.
Case Rep Hematol. 2020 Oct 19;2020:8856411. doi: 10.1155/2020/8856411. eCollection 2020.
Crystal-storing histiocytosis (CSH) is an uncommon condition in which histiocytes accumulate a crystalline matter within their cytoplasm. Generally, those crystals are composed of either monoclonal or polyclonal immunoglobulin chains, which have a strong association with an underlying lymphoproliferative or plasma cell disorder (LP-PCD). Rarely, CSH has been reported as local or generalized manifestation of a variety of benign disorders. These cases are associated with crystals composed of nonimmunoglobulin substances. We are reporting an exceptional case of a local colonic CSH with Charcot-Leyden crystals. This patient underwent a screening colonoscopy that detected some polyps. The biopsy reported tubular adenomas, with a markedly dense, transmural inflammatory infiltrates, which were predominantly composed of eosinophils and crystal-storing histiocytes containing Charcot-Leyden crystals. The patient had a negative workup for LP-PCD and autoimmune conditions, including a normal skeletal survey and bone marrow aspirate/biopsy. The only positive laboratory workup was an elevated absolute eosinophil count and a positive IgG anti- antibody. Giving those findings, this parasitic infection is the most likely etiology of the CSH in our patient. Although there was an initial negative evaluation for LP-PCD, close monitoring of patients with either immunoglobulin or nonimmunoglobulin CSH is recommended.
晶体储存组织细胞增多症（CSH）是一种罕见病症，其中组织细胞在其细胞质内积累一种结晶物质。通常，这些晶体由单克隆或多克隆免疫球蛋白链组成，这与潜在的淋巴增殖性或浆细胞疾病（LP - PCD）密切相关。很少有报道称CSH是各种良性疾病的局部或全身性表现。这些病例与由非免疫球蛋白物质组成的晶体有关。我们报告一例伴有夏科 - 莱登晶体的局部结肠CSH特殊病例。该患者接受了筛查结肠镜检查，发现了一些息肉。活检报告为管状腺瘤，伴有明显密集的透壁炎性浸润，主要由嗜酸性粒细胞和含有夏科 - 莱登晶体的晶体储存组织细胞组成。该患者针对LP - PCD和自身免疫性疾病的检查结果均为阴性，包括骨骼检查和骨髓穿刺/活检结果正常。唯一阳性的实验室检查结果是绝对嗜酸性粒细胞计数升高和IgG抗抗体阳性。基于这些发现，寄生虫感染是我们患者CSH最可能的病因。尽管最初对LP - PCD的评估为阴性，但建议对免疫球蛋白或非免疫球蛋白CSH患者进行密切监测。
