Granulomatous slack skin T-cell lymphoma: an important differential diagnosis with giant cell tumor of soft tissue

Granulomatous slack skin is an indolent T-cell lymphoma, considered to be a variant of mycosis fungoides. Clinically it is characterized by areas of redundant skin, wrinkled, inelastic, with variable erythema and infiltration besides a poikilodermic surface. A differential diagnosis unknown to most dermatologists is the giant cell tumor of soft tissue, which is an extremely rare low-grade sarcoma. The authors report a patient who had undergone extensive surgery because of a primary diagnosis of giant cell tumor of soft tissue, but which proved to be granulomatous slack skin after a second interventional procedure with confirmatory histopathology.     

Fine T-Cell Subsets in Alcoholics as Determined by the Expression of l-Selectin, Leukocyte Common Antigen, and β-Integrin

Alcoholics admitted to the hospital solely for detoxication have been studied by flow cytometry to evaluate changes in the surface markers of peripheral blood leukocytes. As we have shown previously, such patients have an elevated percentage of CD8^hl lymphocytes that are HLA DR⁺; we now demonstrate that they also have striking alterations in the quantitative relationships of the fine T-cell subsets. Both CD4⁺ and CD8^hl lymphocytes have a sharply reduced percentage of the l -selectin⁺ CD45RA⁺ subset, increased percentages of the CD45RA-subsets, and several other fine subset alterations. The fine subset profile suggests, according to current correlations of phenotype and function, that both CD4⁺ suppressor inducer and CD4-dependent CD8⁺ suppressor effector cells are reduced, whereas other subsets, including CD8⁺ CTL or their precursors, are increased in relative percentages. Some of the phenotypic changes are reversible over the several days following withdrawal. In other results, the percentage of CD8^hl lymphocytes expressing CD11b (β-integrin) is shown to be reciprocal with the percentage expressing l -selectin both in normals and alcoholics. However, the regression function of CD11b vs. l -selectin on CD8^hl cells is different for the alcoholics than for the normals, indicating an abnormality in the regulation of the expression of these two adhesion markers. Taken together, this abnormality of adhesion molecules and the fine subset alterations previously described indicate widespread changes in the peripheral lymphocytes of currently drinking alcoholics. These changes suggest functional deficiencies that may include alterations of lymphocyte traffic and other adhesion-dependent functions, and a shift in the balance of regulatory interactions.     

Alcohol Modulation of Human Normal T-Cell Activation, Maturation, and Migration

We are interested in the characterization of the effects of alcohol on human T-cell activation, maturation, and migration, because this cell population is crucial in the initiation, regulation, and propagation of cellular immunity. We and others have described the effects of both acute and chronic exposure of human immune cells to ethanol (EtOH) in vitro. Herein, we briefly, review these reports and expand this body of literature with the inclusion of new data recently obtained in our laboratory. We confirm the blunting effects of EtOH on the production of interleukin-2 and mitogen proliferative response following T-cell mitogen stimulation, and on the expression of membrane markers of activation. We show that EtOH significantly alters the expression of the CD4 cell-associated marker of activation, CD26. We report the effect of EtOH on the expression of the homing receptor CD62L by CD4⁺ cells, and on their ability to adhere by a CD18-mediated process to a defined cellular substratum. Furthermore, we demonstrate the effects of EtOH and EtOH and 0-endor-phin pretreatment on the activation of CD4⁺ lymphocytes endowed with the homing receptor CD62L.     

皮下脂膜炎样T细胞淋巴瘤叠加红斑狼疮病理特征一例

患者男,49岁。因皮下结节9个月余,发热5 d,于2018年11月19日至我院就诊。患者9个月前无明显诱因全身出现多个散在直径2 ~ 5 cm皮下结节,曾于我院行皮损活检,提示“皮下脂膜炎,不除外皮肤T细胞淋巴瘤”,当时未予特殊处理……     

Mycosis Fungoides and Variants of Mycosis Fungoides: A Retrospective Study of 93 Patients in a Chinese Population at a Single Center

BackgroundMycosis fungoides (MF) is the most common types of cutaneous T cell lymphoma. It typically presents with erythematous patches and plaques in the early stage and tumors and extracutaneous involvement with possibly fatal outcomes in the late stage. To facilitate early and accurate diagnosis of MF, it is essential to be knowledgeable of classic and variants of this disease. However, there is limited published data in the Chinese population.ObjectiveTo characterize our patient group and to provide additional insight into these malignancies.MethodsPatients diagnosed with mycosis fungoides and its variants from October 2012 to January 2018 were retrospectively analyzed. Disease-specific survival (DSS) rate and curve according to early and advanced stages were also calculated.ResultsThe mean age at diagnosis of ninety-three MF patients was 38.9±1.73 years (range: 6~77). Forty-five males (48.4%) and 48 females (51.6 %) were included in this study. The DSS rate of early-stage MF was 98.6%, while that of advanced stage MF was 88.9%. There was a significant difference in DSS rate between early stage and advanced stage MF (p=00.042, log-rank test). The median age of 10 patients with hypopigmented MF (hMF) was 10.5 years (range: 6~28). The age of hMF was younger than that of classical MF patients (p<0.05).ConclusionEarly-stage MF has a better prognosis than advanced stage and hMF affects younger people than classic MF among Chinese. This study provides an insight into mycosis fungoides and its variants in a Chinese population.     

The effects of intradermal vaccination with DNA encoding for the T-cell receptor on the induction of experimental autoimmune encephalomyelitis in B10.PL mice

Intradermal gene administration was found to induce a more profound immune response than direct intramuscular gene injection. We performed intradermal vaccination of B10.PL mice with DNA encoding for the V 8.2 region of the T-cell receptors (TCR). Three weeks later, these mice were immunized with rat myelin basic protein (MBP). Daily mean clinical scores and mortality rate were lower in this group compared with controls. The proliferative responses of lymph node cells to rat MBP were slightly less in the vaccination groups than in the control groups (p < 0.05). However, we detected no differences between the two groups with regard to the production of MBP-specific IgG, IgG1, & IgG2a antibodies. The levels of cytokine mRNA expression in the vaccination groups were observed higher than in the control groups without antigen-specific stimulation, but all of cytokine expressions between the vaccination and control groups after antigen-specific stimulation were identical. These results demonstrate that intradermal DNA vaccines encoding for TCR might prove to be useful in the control of autoimmune disease.     

Modulatory effects of fibronectin on in vitro lymphocyte responses

Summary In rat studies, fibronectin (Fn), a ubiquitous glycoprotein, is a T-cell mitogen and stimulates the production of interleukin 1 by peritoneal exudate macrophages. In contrast, Fn has no mitogenic activity for human lymphocytes and does not stimulate interleukin 1 release by human peripheral blood mononuclear cells. Fn increases in vitro peripheral blood mononuclear cell phytohemagglutinin responses in normals and patients with active, but not inactive, rheumatoid arthritis. Fn may alter local immunoregulation and play an active role in the rheumatoid synovial lesion.     

Adult T-cell leukemia/lymphoma with Epstein-Barr virus-positive Hodgkin-like cells

Hodgkin-like cells have been described in a variety of non-Hodgkin lymphomas including chronic lymphocytic leukemia and peripheral T-cell lymphoma. There have been rare reports in the Japanese population of human T-cell lymphotrophic virus-1-associated adult T-cell leukemia/lymphoma harboring Hodgkin-like cells; however, no similar cases have been described in Western patients. We report a 53-year-old African American man who presented with progressive weakness and lethargy, and was found to have generalized lymphadenopathy and hypercalcemia. A lymph node biopsy showed involvement by adult T-cell leukemia/lymphoma with scattered Epstein-Barr virus-positive cells, some of which resembled Hodgkin cells that had a B-cell phenotype, consistent with an Epstein-Barr virus-lymphoproliferative disorder. The patient had stage 4 disease with bone marrow involvement. In light of the associated B-cell lymphoproliferative process, the patient was treated with 6 cycles of intensive chemotherapy that targeted both the adult T-cell leukemia/lymphoma and the Epstein-Barr virus-lymphoproliferative disorder that resulted in a complete response. An awareness of the association of Epstein-Barr virus-lymphoproliferative disorder with Hodgkin-like cells in the context of adult T-cell leukemia/lymphoma is necessary to avoid potential misdiagnosis and to aid in therapeutic decisions.     

粗针穿刺活检在纵隔T淋巴母细胞淋巴瘤与B1型胸腺瘤鉴别诊断中的价值

目的 分析纵隔T淋巴母细胞淋巴瘤(T-LBL)和B1型胸腺瘤病例的临床和病理学特点,提高粗针穿刺活检在这两种疾病鉴别诊断中的准确性.方法 收集连续诊断的34例T-LBL和10例B1型胸腺瘤患者作为研究对象,均接受纵隔粗针穿刺活检获取标本,并经手术标本病理诊断确认.收集两组患者的临床资料,对比其粗针穿刺活检标本的组织病理学和免疫表型的差异.结果 T-LBL患者以小于30岁为主(73％),男性发病明显多于女性,男女比3.3∶1.B1型胸腺瘤平均年龄43岁(40岁以上占50％),男女比2∶3.T-LBL患者均有胸闷、气短、咳嗽等临床症状;B1型胸腺瘤患者中3例出现胸闷气短症状,2例出现重症肌无力症状.影像学特征:两组患者均为前纵隔单发肿物,肿块＞10cm者在T-LBL患者中占88％,在B1型胸腺瘤患者中仅占50％.T-LBL患者全部可见肿瘤侵犯引起的胸腔或心包腔积液,而B1型胸腺瘤无此特征.病理学特征:T-LBL的瘤细胞在纤维组织中呈“列兵样”浸润性生长(65％),有骨骼肌组织侵犯(41％)和肿瘤性坏死(21％),而B1型胸腺瘤无此特征.所有B1型胸腺瘤均保留胸腺小叶结构,可见粗大纤维间隔,但仅3％的T-LBL保留胸腺小叶结构.免疫组化染色显示所有B1型胸腺瘤均可见完整的细胞角蛋白网,但T-LBL的细胞角蛋白网破坏.T-LBL和B1型胸腺瘤出现周围脂肪组织浸润的比例有差异,分别为59％和20％.结论 T-LBL与B1型胸腺瘤在患者性别、发病年龄、临床症状及影像学改变上有明显差异,以病理学特征的改变最具鉴别意义.密切联系临床表现与组织病理学改变有助于提高纵隔粗针穿刺活检在两种疾病鉴别诊断中的价值.