Sukpanichnant S, Hargrove N S, Kachintorn U, Manatsathit S, Chanchairujira T, Siritanaratkul N, Akaraviputh T, Thakerngpol K
Department of Pathology, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok, Thailand.
Am J Surg Pathol. 2000 Jan;24(1):129-35. doi: 10.1097/00000478-200001000-00016.
Clofazimine-induced crystal-storing histiocytosis is a rare but well-recognized condition in the literature. Besides the common reddish discoloration of the skin, clofazimine produces gastrointestinal disturbances-sometimes severe abdominal pain, prompting exploratory laparotomy, because pathologic and radiologic findings can produce diagnostic difficulties if the pathologic changes caused by clofazimine are not recognized. The authors report such a case in a leprosy patient to emphasize the importance of history taking, the radiologic abnormalities of the small intestine, and the pathologic findings in small intestine and lymph node biopsies. Clofazimine crystals are red in the frozen section and exhibit bright-red birefringence. However, they are clear in routinely processed histologic sections because they dissolve in alcohol and organic solvents. They also appear as clear crystal spaces during electron microscopic study, but some osmiophilic bodies can be observed. Histiocytosis caused by clofazimine crystals produces infiltrative lesions in radiologic studies mimicking malignant lymphoma or other infiltrative disorders. Associated plasmacytosis in the histologic sections can simulate lymphoplasmacytic lymphoma or multiple myeloma with crystal-storing histiocytosis. With the knowledge of this rare condition caused by clofazimine, appropriate management to avoid an unnecessary laparotomy is possible.
氯法齐明诱导的晶体贮积性组织细胞增多症是一种在文献中罕见但已被充分认识的病症。除了常见的皮肤发红变色外，氯法齐明还会引起胃肠道紊乱，有时会导致严重腹痛，促使进行剖腹探查，因为如果未认识到氯法齐明引起的病理变化，病理和放射学检查结果可能会造成诊断困难。作者报告了一名麻风病患者的此类病例，以强调病史采集、小肠的放射学异常以及小肠和淋巴结活检的病理结果的重要性。氯法齐明晶体在冰冻切片中呈红色，并呈现鲜红色双折射。然而，在常规处理的组织学切片中它们是透明的，因为它们可溶于酒精和有机溶剂。在电子显微镜研究中它们也表现为透明的晶体间隙，但可观察到一些嗜锇小体。在放射学研究中，由氯法齐明晶体引起的组织细胞增多症会产生类似恶性淋巴瘤或其他浸润性疾病的浸润性病变。组织学切片中的相关浆细胞增多症可模拟伴有晶体贮积性组织细胞增多症的淋巴浆细胞性淋巴瘤或多发性骨髓瘤。了解这种由氯法齐明引起的罕见病症后，有可能采取适当的处理措施以避免不必要的剖腹探查。
