Crystal‐Storing Histiocytosis Associated with Lymphoplasmacytic Neoplasms: Report of Three Cases Mimicking Adult Rhabdomyoma
Crystal‐Storing Histiocytosis Associated with Lymphoplasmacytic Neoplasms: Report of Three Cases Mimicking Adult Rhabdomyoma
复制标题
与淋巴浆细胞肿瘤相关的晶体储存组织细胞增多症：模仿成人横纹肌瘤的三例报告
DOI：
10.1097/00000478-199305000-00004
复制
发表时间：
1993
期刊：
影响因子：
--
通讯作者：
G. Frizzera
中科院分区：
文献类型：
--
作者：
S. Kapadia;F. Enzinger;D. K. Heffner;V. Hyams;G. Frizzera
大量晶体沉积在淋巴浆细胞瘤或浆细胞瘤中是罕见的。我们报告三个病例，在组织细胞晶体的积累密切再现成人横纹肌瘤的组织学特征。患者均为女性，年龄分别为18岁、77岁和78岁，均以颈部淋巴结肿物（2例）或耳鼻喉粘膜肿物（2例）为临床表现。此外，两名患者有单克隆血清或尿液免疫球蛋白（fgM-k-1，未知-1），一名患者在活检中有肾脏和骨髓受累。最后一位患者在5个月时死于急性肾衰竭，另一位患者在8年无病存活，其余一位患者失去随访。淋巴结、粘膜和肾脏显示肿瘤性LPc浸润，被大的良性组织细胞片所掩盖，组织细胞片含有成束的晶体。石蜡免疫组织化学显示，所有病例的LPc细胞（fgM-k-2, IgA-k-1）和1例的晶体（IgM-K）均为单克隆染色。在所有患者中，含晶体细胞的KP-1 （CD68）呈阳性，但desmin、肌肉特异性作用或肌红蛋白呈阴性。这些结果表明，在任何组织学表现不典型的成人横纹瘤病例中，应排除晶体储存组织细胞增多症：识别非典型LPc成分和晶体储存细胞的组织细胞免疫表型将有助于防止严重的误诊。
Massive crystal deposition is rare in lymphoplasmacytic (LPc) or plasma cell neoplasms. We report three cases in which the accumulation of crystals in histocytes closely reproduced the histologic features of adult rhabdomyoma. The patients, all female, aged 18, 77, and 78 years, presented with tumor of cervical lymph nodes (two cases) or the otolaryngic mucosa (two cases). In addition, two patients had monoclonal serum or urine immunoglobulin (fgM-k-1, unknown-1), and one had renal and bone marrow involvement on biopsy. This last patient died of acute renal failure at 5 months, another was alive without disease at 8 years, and the remaining one was lost to followup. Lymph nodes, mucosae, and kidney showed a neoplastic LPc infiltrate masked by sheets of large benign histiocytes containing sheaves of crystals. Paraffinsection immunohistochemistry demonstrated monoclonal staining of the LPc cells in all cases (fgM-k-2, IgA-k-1) and of the crystals (IgM-K) in one case. In all patients, the crystal-containing cells were positive for KP-1 (CD68), but not for desmin, muscle-specific action, or myoglobin. These findings suggest that, in any case of adult rhabdomyoma in which the histologic findings are not typical, a crystal-storing histiocytosis should be ruled out: recognition of the atypical LPc component and the histiocytic immunophenotype of the crystal-storing cells will help prevent a serious misdiagnosis.