Pálos Kata, Szakonyi József, Csomor Judit, Gremsperger Åsa, Zajta Erik, Marschalkó Márta, Szepesi Ágota
Department of Pathology and Experimental Cancer Research, Semmelweis University, Budapest, Hungary.
Department of Dermatology, Venereology and Dermatooncology, Semmelweis University, Budapest, Hungary.
J Cutan Pathol. 2024 Nov;51(11):868-875. doi: 10.1111/cup.14697. Epub 2024 Jul 30.
Primary cutaneous marginal zone lymphoma (PCMZL) and primary cutaneous CD4+ small/medium T-cell lymphoproliferative disease (CD4+ TLPD) are two distinct entities with excellent prognosis; however, they show profound clinical and histopathological similarities, leading to differential diagnostic uncertainty.
Our aim was to review and reanalyze cases of primary cutaneous lymphoproliferations diagnosed at Semmelweis University, featuring characteristics of PCMZL and CD4+ TLPD.
Cutaneous lymphoma biopsy specimens between 2018 and 2022 were collected and re-evaluated. Medical history, clinical picture, imaging, and laboratory findings were collected. Immunohistochemical staining for CD20, CD3, BCL6, CD10, PD1, CD3, CD4, CD8, and PCR tests for IGH, IGK, TCRB, and TCRG were repeated in selected cases.
Among 55 cases diagnosed as PCMZL (16) or CD4+ TLPD (39), 3 patients had been diagnosed with both LPDs at different time points of their disease course. Four additional patients were identified with single lesions featuring overlapping histopathological characteristics of both LPDs and both monoclonal IGH and TCR rearrangements. All patients are currently in complete remission with local treatment.
We propose that besides the overlapping histopathological, molecular, and clinical features, the subsequent appearance of PCMZL and CD4+ TLPD in a short timeframe in the same patients may suggest a common pathogenic background.
原发性皮肤边缘区淋巴瘤（PCMZL）和原发性皮肤CD4+小/中T细胞淋巴增殖性疾病（CD4+ TLPD）是两种预后良好的不同疾病实体；然而，它们在临床和组织病理学上表现出显著的相似性，导致鉴别诊断存在不确定性。
我们的目的是回顾并重新分析在塞梅尔维斯大学诊断的原发性皮肤淋巴增殖病例，这些病例具有PCMZL和CD4+ TLPD的特征。
收集并重新评估2018年至2022年期间的皮肤淋巴瘤活检标本。收集病史、临床表现、影像学和实验室检查结果。对选定病例重复进行CD20、CD3、BCL6、CD10、PD1、CD3、CD4、CD8的免疫组化染色以及IGH、IGK、TCRB和TCRG的PCR检测。
在55例诊断为PCMZL（16例）或CD4+ TLPD（39例）的病例中，3例患者在疾病病程的不同时间点被诊断为这两种淋巴增殖性疾病。另外4例患者被发现有单个病变，具有两种淋巴增殖性疾病重叠的组织病理学特征以及单克隆IGH和TCR重排。所有患者经局部治疗后目前均处于完全缓解状态。
我们提出，除了重叠的组织病理学、分子和临床特征外，同一患者在短时间内先后出现PCMZL和CD4+ TLPD可能提示存在共同的致病背景。
