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皮肤恶性和良性淋巴样浸润的鉴别诊断:一项对57例皮肤诊断或疑似恶性淋巴瘤病例的研究。

Differential diagnosis of malignant and benign cutaneous lymphoid infiltrates: a study of 57 cases in which malignant lymphoma had been diagnosed or suspected in the skin.

作者信息

Evans H L, Winkelmann R K, Banks P M

出版信息

Cancer. 1979 Aug;44(2):699-717. doi: 10.1002/1097-0142(197908)44:2<699::aid-cncr2820440243>3.0.co;2-j.

Abstract

Clinical and histopathologic findings were reviewed in 57 cases in which there had been a diagnosis or suspicion of malignant lymphoma in the skin other than mycosis fungoides. Patients were included only if 1) extracutaneous tissue satisfactory for lymphoma classification was available, or 2) there was no evidence of extracutaneous involvement after a minimum of five years. Thirty-five patients had systemic lymphoma; in eight of these that disease first became manifest in the skin. There were two patients with typical lymphomatoid papulosis and one with a somewhat similar but solitary lesion which did not recur after excision; we have termed the latter a "solitary lymphomatoid papule." The remaining 19 cases without extracutaneous involvement were regarded as benign lymphoid hyperplasia. Clinically, the cutaneous lesions of malignant lymphoma were difficult to distinguish from those of lymphoid hyperplasia; only lymphomatoid papulosis was distinctive. Histologically, it was found that a definite diagnosis of malignant lymphoma (other than mycosis fungoides) could be made in the skin when three criteria were fulfilled: 1) exclusive presence or marked preponderance of medium-sized or larger lymphocytes, 2) absence of preferential involvement of the upper dermis, and 3) absence of extensive epidermal infiltration. These criteria were met in 21 cases of lymphoma. In both cases of lymphomatoid papulosis, the infiltrate was predominantly composed of large lymphocytes but was primarily limited to the upper dermis. The "solitary lymphomatoid papule" contained a similar cell population and involved the full thickness of the dermis; however, it displayed a prominent intraepidermal component. Fourteen cases of malignant lymphoma and all cases of lymphoid hyperplasia were characterized by a mixture of lymphocytes of varying size or a predominance of small lymphocytes. Nine cases of lymphoid hyperplasia exhibited at least one of three findings not observed in lymphoma; these findings were 1) germinal centers, 2) marked paucity of medium-sized lymphocytes in comparison to both small and large lymphocytes, and 3) sharp margination between adjacent groups of small and large lymphocytes. Aside from one case of small lymphocytic lymphoma which showed deep subcutaneous extension, the remaining cases (13 malignant and 10 benign) could not be reliably differentiated from each other by skin biopsy findings alone and were thus considered histologically indeterminate. The 13 lymphoma cases in this group were all of the small lymphocytic or follicular types; the failure of follicular lymphoma to exhibit a follicular pattern in the skin was a major impediment to its recognition in that location. The cutaneous infiltrates in all cases of large lymphocytic, Burkitt's, and lymphoblastic lymphoma were diagnostic of malignancy.

摘要

对57例诊断或疑似除蕈样肉芽肿外的皮肤恶性淋巴瘤的病例进行了临床和组织病理学检查。仅在以下情况下纳入患者:1)有可用于淋巴瘤分类的皮肤外组织;或2)至少五年后无皮肤外受累证据。35例患者患有系统性淋巴瘤;其中8例疾病首先在皮肤表现出来。有2例典型的淋巴瘤样丘疹病患者,1例有类似但孤立的病变,切除后未复发;我们将后者称为“孤立性淋巴瘤样丘疹”。其余19例无皮肤外受累的病例被视为良性淋巴组织增生。临床上,恶性淋巴瘤的皮肤病变很难与淋巴组织增生的病变区分开来;只有淋巴瘤样丘疹病具有特征性。组织学上发现,当满足以下三个标准时,可在皮肤中做出除蕈样肉芽肿外的恶性淋巴瘤的明确诊断:1)中等大小或更大淋巴细胞的排他性存在或显著优势;2)不存在优先累及真皮上层;3)不存在广泛的表皮浸润。21例淋巴瘤病例符合这些标准。在2例淋巴瘤样丘疹病病例中,浸润主要由大淋巴细胞组成,但主要局限于真皮上层。“孤立性淋巴瘤样丘疹”包含类似的细胞群并累及真皮全层;然而,它显示出明显的表皮内成分。14例恶性淋巴瘤病例和所有淋巴组织增生病例的特征是大小不同的淋巴细胞混合或小淋巴细胞占优势。9例淋巴组织增生病例表现出淋巴瘤未观察到的三个发现中的至少一个;这些发现是:1)生发中心;2)与小淋巴细胞和大淋巴细胞相比,中等大小淋巴细胞明显稀少;3)相邻的小淋巴细胞和大淋巴细胞组之间有明显的边界。除1例小淋巴细胞淋巴瘤显示深部皮下扩展外,其余病例(13例恶性和10例良性)仅通过皮肤活检结果无法可靠区分,因此在组织学上被认为是不确定的。该组中的13例淋巴瘤病例均为小淋巴细胞或滤泡型;滤泡性淋巴瘤在皮肤中未表现出滤泡模式是在该部位识别它的主要障碍。所有大淋巴细胞性、伯基特氏和淋巴母细胞性淋巴瘤病例的皮肤浸润均诊断为恶性。

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