Pagetoid reticulosis (Woringer‐Kolopp disease)

Pagetoid reticulosis (Woringer‐Kolopp disease) is a rare subtype of cutaneous CD8‐positive T‐cell lymphoma. A 41‐year‐old man presented with a 7‐year history with a slowly progressive erythematous plaque on his right buttocks. With the working diagnosis of psoriasis, he was treated with topical corticosteroids which produced no improvement. Histological examination showed an epidermotropic T‐cell lymphoma with predominance of CD8‐ vs.CD4‐positive lymphocytes. Based on the clinical picture and the histological findings, we diagnosed pagetoid reticulosis. Excision of the plaque and cream PUVA photo‐chemotherapy produced long‐term remission.     

The usefulness of clonality for the detection of cases clinically and/or histopathologically not recognized as cutaneous T-cell lymphoma

BACKGROUND: The determination of clonality has proven to be a useful adjunct to the diagnosis of cutaneous lymphocytic infiltrates. It is considered particularly helpful for the distinction of mycosis fungoides (MF) and inflammatory dermatoses. OBJECTIVES: To verify the sensitivity of the polymerase chain reaction (PCR)-heteroduplex analysis of T-cell receptor gamma-chain gene (TCRgamma) rearrangements in patients with MF and to establish whether a clinicopathological re-evaluation of lesions previously unclassified or considered to be non-neoplastic entities but found to be monoclonal allowed the recognition of additional cases of MF. METHODS: Included in the study were 116 patients, seen at our Institute from April 2002 to September 2003 and tested for TCRgamma rearrangements. Thirty-six patients were affected by clinically and histopathologically proven MF, while the remaining 80 cases had not been classified or had been classified as non-neoplastic entities. The sensitivity of the molecular analysis was determined on the basis of the results obtained in the 36 patients with MF. The 29 cases of the second series of patients found to be monoclonal were clinically and histopathologically re-evaluated. RESULTS: Clonal rearrangements were found in 87.5% of patients with plaque stage MF and in 20% of those with patch stage MF. The clinicopathological re-evaluation allowed us to reclassify 15 of 29 monoclonal cases of the second series of patients as MF. CONCLUSIONS: The study showed that the PCR-heteroduplex technique can determine a high percentage of monoclonality only in plaque stage MF. However, in spite of the low sensitivity of the method, several cases previously unrecognized could be reclassified as MF when their clinical and histopathological features were re-evaluated taking into account the clonality of the lymphocytic infiltrate.     

眼附属器黏膜相关淋巴组织淋巴瘤的研究进展

黏膜相关淋巴组织（MALT）淋巴瘤是非霍奇金淋巴瘤中结外边缘带淋巴瘤（MZL）的一种特殊类型。眼附属器MALT淋巴瘤是眼眶恶性肿瘤中较常见的一种，可发生在结膜、泪腺、眼睑和眼眶。近年来，国外许多学者在MALT淋巴瘤的临床特点、病理形态学、免疫表型、分子遗传学及分子生物学方面对它的发生机制在基因水平有了新的认识。就国外对眼附属器MALT淋巴瘤的分子遗传学异常在肿瘤细胞内部信号转导通路中作用的研究做一综述。     

Characterization of the lymphoproliferative diseases in the skin by DNA analysis.

Various samples from lymphoproliferative diseases in the skin were analyzed by Southern blotting technique with probes from the T cell receptor gene, immunoglobulin genes, and human T cell leukemia virus-I genome. Samples were taken from 10 mycosis fungoides (MF) patients, 1 parapsoriasis en plaque patient, 10 Adult T cell leukemia/lymphoma (ATL) patients, 1 cutaneous T cell lymphoma (CTCL) patient, 4 lymphomatoid papulosis (LP) patients, 4 B cell lymphoma patients, and 2 actinic reticuloid (AR) patients. In MF, the monoclonality of the T cells became detectable first in the skin when plaques develop to tumors then in lymph nodes, and finally in the blood lymphocytes, indicating this disease develops from local (skin) malignancy to systemic malignancy. In parapsoriasis en plaque, no monoclonality was detected in any sample. We could distinguish cutaneous ATL from the carrier state by detecting the T cell monoclonality and HTLV-I integration with these probes. One patient with CTCL showed detectable T cell monoclonality; 1 out of 4 patients with LP did the same. Four samples from patients with B cell lymphoma revealed detectable monoclonal rearrangement of immunoglobulin heavy and light chain genes. In AR, no monoclonality was detected in any sample. From these data, we conclude that DNA analysis is useful in determining the monoclonality, cell origin, and distribution of monoclonal cells from skin samples.     

维布妥昔单抗治疗Sézary综合征一例

患者,男,41岁。全身弥漫性红斑伴严重瘙痒1年,多发淋巴结肿大3个月。PET/CT符合淋巴瘤;淋巴结病理：非霍奇金T细胞淋巴瘤,伴不规则、扭曲、脑回样核;皮肤病理：非霍奇金T细胞淋巴瘤,呈蕈样肉芽肿改变。结合患者症状、体征、影像学、病理及免疫组化结果,以及外周血T细胞基因重排均为阳性,诊断为Sézary综合征。给予化疗等一线药物治疗效果不佳。更换为含有维布妥昔单抗的治疗方案后患者红斑消退,瘙痒消失,淋巴结减小,达到完全缓解。     

Absence of the t(14;18) chromosomal translocation in primary cutaneous B-cell lymphoma

BACKGROUND: The t(14;18)(q32;q21) chromosomal translocation is found in the majority of nodal follicular lymphomas and in a lower percentage of systemic high-grade diffuse large B-cell lymphomas. The translocation results in the juxtaposition of the bcl-2 gene on chromosome 18 with the immunoglobulin heavy chain joining region on chromosome 14. Bcl-2 protein prevents apoptosis and the translocation leads to overexpression of a functionally normal Bcl-2 protein that prevents apoptosis of neoplastic cells. OBJECTIVES: The purpose of our study was to analyse cases of primary cutaneous B-cell lymphoma (PCBCL) for the presence of the t(14;18) translocation and to correlate the results with Bcl-2 expression and histological subtype. METHODS: Forty-four cutaneous B-cell lymphoid proliferations (36 PCBCL, four follicular B-cell lymphomas with cutaneous presentation and four reactive B-cell infiltrates) were analysed by polymerase chain reaction amplification and polyacrylamide gel electrophoresis using consensus primers for the joining region on the immunoglobulin heavy chain gene in combination with either a primer for the major breakpoint region (MBR) or the minor cluster region (mcr) on chromosome 18. RESULTS: None of 36 PCBCL analysed demonstrated a t(14;18) translocation; however, three of four systemic follicular B-cell lymphomas presenting in the skin were found to have a translocation in the MBR, which was confirmed by sequence analysis. Correlation with Bcl-2 immunostaining showed that of seven patients with high-grade cutaneous diffuse large B-cell lymphoma, four were Bcl-2 positive but had no evidence of a t(14;18) translocation. In the five cases classified as primary cutaneous follicle centre cell lymphoma, the neoplastic cells within the germinal centres failed to express Bcl-2. However, Bcl-2-positive neoplastic cells were present in all four cases of systemic follicular lymphoma, including the case that did not show a t(14;18) translocation. In all cases of marginal zone lymphoma the marginal zone lymphocytes were Bcl-2 positive. CONCLUSIONS: These findings indicate that the t(14;18) translocation does not occur in PCBCL, which suggests the involvement of different pathogenetic mechanisms compared with their nodal counterparts. Furthermore, the detection of a t(14;18) translocation in cutaneous B-cell lymphoma should suggest the presence of systemic disease, which underlies the need for exhaustive staging procedures.     

Successful treatment of a patient with lymphomatoid papulosis by methotrexate

We report a case of lymphomatoid papulosis (LyP) that occurred in a 44-year-old Japanese male patient. Reddish papules with a small number of pustules and nodules were observed on the extremities, chest and upper back. Most lesions were also associated with central necrosis, ulceration and crusting, and regressed spontaneously within 4 to 6 weeks. Histopathological examination revealed wedge-shaped dense cellular infiltrate in the dermis, which was mixed with large atypical lymphoid cells, small lymphocytes, eosinophils and neutrophils. These large atypical cells expressed CD30 on their cell membrane and cytoplasm. Rearrangement of the T-cell receptor (TcR) beta-chain gene was detected in the skin lesion. Lymphadenopathy with histopathologic change similar to the skin lesions, but without TcR gene rearrangement, was found at the left inguinal area. Systemic administration of methotrexate (7.5-15.0 mg/week) was found to be dramatically effective in resolution of skin lesions and prevention of their recurrence.     

T-zone lymphoma with cutaneous involvement: a case report and review of the literature

T-zone lymphoma (TZL) is a rare subtype of nodal peripheral T-cell lymphoma characterized by a clonal expansion of T-zone lymphocytes accompanied by a proliferation of other T-zone constituents. Non-specific cutaneous alterations are seen in about one-third of all cases, but specific cutaneous involvement is extremely rare. We present a case of TZL with secondary skin infiltration, review the literature on cutaneous manifestations of TZL and discuss the differential diagnosis of TZL.     

Topical 5-aminolaevulinic acid photodynamic therapy for the treatment of skin conditions other than non-melanoma skin cancer

Topical 5-aminolaevulinic acid (ALA) photodynamic therapy (PDT) is used increasingly for superficial non-melanoma skin cancer (NMSC) and dysplasia. However, the relative accumulation of the photosensitizer protoporphyrin IX (PpIX) in diseased tissue is not specific for neoplastic disease, and has been shown after the application of ALA to benign proliferative skin conditions such as viral warts and psoriasis. This review appraises the quality of evidence available for the use of topical ALA-PDT in the treatment of skin conditions other than NMSC. The diseases that have been studied in most detail are recalcitrant viral warts, acne, psoriasis and cutaneous T-cell lymphoma. Publications relating to the treatment of other diseases by topical PDT are restricted to small case series or case reports. The relevant literature will be discussed and the potential for topical PDT in the treatment of several skin diseases is highlighted, although more detailed studies are required to clarify the role of PDT beyond the treatment of NMSC.     

眼附属器淋巴瘤的临床病理分析

目的：探讨眼附属器淋巴瘤(OAL)的发病情况、临床表现及病理分型,以提高临床诊治水平。

方法：选取2005-08/2013-07在华西医院眼科确诊的OAL患者147例,收集患者的一般情况、临床表现、临床检查(影像学、病理学)结果、治疗及预后情况等临床资料进行回顾性研究。

结果：本研究纳入OAL患者147例,其中男91例(61.9%),女56例(38.1%); 年龄3.5～87岁; 病程20d～10a; 发病部位以眼眶最多见(106/147); 最常见的临床表现为眼部包块、眼睑肿胀、眼球突出; 病理分型以黏膜相关淋巴组织边缘区B细胞淋巴瘤最多见(112/147),且其预后最好,而弥漫大B细胞淋巴瘤、结外NK/T细胞淋巴瘤,鼻型预后最差。

结论：OAL病理类型复杂,发病部位和临床表现多样,免疫组织化学染色是OAL鉴别诊断的重要依据,其预后与病理类型密切相关。