Egeler R M, Schmitz L, Sonneveld P, Mannival C, Nesbit M E
Sophia Children's Hospital, Erasmus University, Rotterdam, The Netherlands.
Med Pediatr Oncol. 1995 Jul;25(1):1-7. doi: 10.1002/mpo.2950250102.
Malignant histiocytosis (MH) and true histiocytic lymphoma (THL) are hematopoietic malignancies of the mononuclear phagocytic system distinguished from each other by clinical presentation and presumed cell of origin. THL present as a localized mass derived from the fixed tissue histiocyte which may or may not disseminate. MH originates from the circulating monocyte or tissue macrophage and is characterized by a syndrome of systemic symptoms, pancytopenia, adenopathy, hepatosplenomegaly, and wasting. The distinction between MH and THL is at times arbitrary and overlap exists between these syndromes. The clinicopathologic studies that defined these entities were performed prior to the development of immunophenotyping and other molecular techniques currently used to ensure proper classification of hematopoietic malignancies. Nine patients from the University of Minnesota originally diagnosed with MH were retrospectively analyzed using a panel of antibodies reactive against T cell, B cell, and myelomonocytic antigens. Only one patient was reclassified as a possible histiocytic malignancy after reevaluation. Similar immunophenotyping studies have also shown cases previously diagnosed as MH or THL express lymphoid antigens, and would now be classified as Ki-1 positive anaplastic large cell lymphoma (ALCL) or some other hematopoietic neoplasm. These results indicate true histiocytic neoplasms are extremely rare, and previous concepts concerning clinical presentation and therapeutic outcome of the entities are inaccurate. In this paper we summarize the results of multiple retrospective analyses of cases previously diagnosed as MH or THL, including our experience at University of Minnesota, to illustrate the overall rarity of these entities. The current literature on malignant histiocytic disorders is reviewed, and the clinical presentation of patients determined to have histiocytic malignancies using contemporary analytical techniques is discussed.
恶性组织细胞增多症（MH）和真性组织细胞淋巴瘤（THL）是单核吞噬细胞系统的造血恶性肿瘤，可根据临床表现和推测的起源细胞相互区分。THL表现为源自固定组织组织细胞的局限性肿块，可能会或不会发生播散。MH起源于循环单核细胞或组织巨噬细胞，其特征为全身症状、全血细胞减少、淋巴结病、肝脾肿大和消瘦综合征。MH和THL之间的区分有时是任意的，这些综合征之间存在重叠。定义这些实体的临床病理研究是在免疫表型分析和目前用于确保造血恶性肿瘤正确分类的其他分子技术发展之前进行的。对明尼苏达大学最初诊断为MH的9名患者使用一组针对T细胞、B细胞和骨髓单核细胞抗原的抗体进行回顾性分析。重新评估后，只有一名患者被重新分类为可能的组织细胞恶性肿瘤。类似的免疫表型研究也表明，先前诊断为MH或THL的病例表达淋巴样抗原，现在将被分类为Ki-1阳性间变性大细胞淋巴瘤（ALCL）或其他一些造血肿瘤。这些结果表明真性组织细胞肿瘤极为罕见，并且先前关于这些实体的临床表现和治疗结果的概念是不准确的。在本文中，我们总结了对先前诊断为MH或THL的病例进行的多次回顾性分析结果，包括我们在明尼苏达大学的经验，以说明这些实体的总体罕见性。本文回顾了关于恶性组织细胞疾病的当前文献，并讨论了使用当代分析技术确定患有组织细胞恶性肿瘤的患者的临床表现。
