Delsol G, Al Saati T, Gatter K C, Gerdes J, Schwarting R, Caveriviere P, Rigal-Huguet F, Robert A, Stein H, Mason D Y
Laboratoire d' Anatomie Pathologique, CHU Purpan, Toulouse, France.
Am J Pathol. 1988 Jan;130(1):59-70.
A group of 63 cases of anaplastic large cell lymphomas (variants of diffuse large cell lymphomas often diagnosed as "malignant histiocytosis") was characterized on both morphologic criteria and expression of epithelial membrane antigen (EMA) and Ki-1 antigen (CD30). On the basis of the reactivity of these tumors with anti-EMA and anti-Ki-1 antibodies, four subtypes could be distinguished. In the majority of cases (n = 49), neoplastic cells coexpressed EMA and Ki-1 antigens. Nineteen of these cases were tested for IL-2R, and all were positive (Type I, EMA+, Ki-1+, IL-2R+). In the second group (n = 5), the neoplastic cells expressed EMA but not the Ki-1 antigen. These cases were not tested for the presence of IL-2R (Type II, EMA+, Ki-1-, IL-2R?). There were tumors with similar morphology expressing only Ki-1 antigen (Type III, EMA-, Ki-1+, IL-2R-) or negative for both EMA and Ki-1 antigens (Type IV, EMA-, Ki-1-, IL-2R-). EMA appeared to occur predominantly on activated cells, as has been previously shown for Ki-1 antigen. Analysis using monoclonal antibodies to T-cell, B-cell, or macrophage-associated differentiation antigens showed that these tumors were heterogeneous in terms of cell lineage. Tumors coexpressing EMA, Ki-1, and IL-2R (Type I), were most commonly of T-cell origin (n = 12); the remainder in this type expressed B-cell markers (n = 4), a mixed B/T phenotype (n = 2), or no clear phenotype (n = 9). By contrast, tumors of Types II, III, and IV were mainly from B-cell origin (n = 6) or showed a mixed B/T phenotype (n = 1). Despite the fact that a significant proportion of these cases were initially classified morphologically as "malignant histiocytosis," only 3 of the 63 cases were possibly of histiocytic origin. These results confirm that true malignant histiocytosis is rare and that most tumors with histologic features currently regarded as being consistent with this diagnosis are lymphocytic in origin and express activation antigens such as EMA, Ki-1 antigen, and IL-2R.
一组63例间变性大细胞淋巴瘤（弥漫性大细胞淋巴瘤的变体，常被诊断为“恶性组织细胞增多症”）根据形态学标准以及上皮膜抗原（EMA）和Ki-1抗原（CD30）的表达进行了特征分析。基于这些肿瘤与抗EMA和抗Ki-1抗体的反应性，可以区分出四种亚型。在大多数病例（n = 49）中，肿瘤细胞共表达EMA和Ki-1抗原。其中19例检测了IL-2R，全部为阳性（I型，EMA +，Ki-1 +，IL-2R +）。在第二组（n = 5）中，肿瘤细胞表达EMA但不表达Ki-1抗原。这些病例未检测IL-2R的存在（II型，EMA +，Ki-1 -，IL-2R？）。有形态相似的肿瘤仅表达Ki-1抗原（III型，EMA -，Ki-1 +，IL-2R -）或EMA和Ki-1抗原均为阴性（IV型，EMA -，Ki-1 -，IL-2R -）。如先前对Ki-1抗原的研究所示，EMA似乎主要出现在活化细胞上。使用针对T细胞、B细胞或巨噬细胞相关分化抗原的单克隆抗体进行分析表明，这些肿瘤在细胞谱系方面具有异质性。共表达EMA、Ki-1和IL-2R的肿瘤（I型）最常见的起源是T细胞（n = 12）；该类型中的其余病例表达B细胞标志物（n = 4）、混合B/T表型（n = 2）或无明确表型（n = 9）。相比之下，II型、III型和IV型肿瘤主要起源于B细胞（n = 6）或表现为混合B/T表型（n = 1）。尽管这些病例中有很大一部分最初在形态学上被归类为“恶性组织细胞增多症”，但63例病例中只有3例可能起源于组织细胞。这些结果证实，真正的恶性组织细胞增多症很罕见，并且大多数具有目前被认为与该诊断相符的组织学特征的肿瘤起源于淋巴细胞，并表达活化抗原，如EMA、Ki-1抗原和IL-2R。
