Peripheral T-cell lymphoma

A 32-year-old man presented with a 5-year history of cutaneous nodules on his head and a diffuse, lichenified eruption. Histopathologic examination showed an atypical lymphocytic infiltrate. Immunophenotyping studies determined that the lymphocyte population to be CD4-positive, with partial loss of CD3 and CD7, and immunogenotyping studies showed a clonal rearrangement of the T-cell receptor. A positron-emission tomography scan showed increased uptake in cervical, axillary, and inguinal lymph nodes. A diagnosis of peripheral T-cell lymphoma was made, and the patient is undergoing chemotherapy.     

Peripheral T-cell lymphoma with erysipelaslike spread

An 88-year-old man from the Dominican Republic with a history of gastric adenocarcinoma was admitted with one month of fatigue, anorexia, weight loss, and abdominal pain. The dermatology department was consulted to evaluate an asymptomatic, shiny, firm, red nodule on the lower left chest, with an expanding rim of erythema. Skin biopsies were performed from the nodule and surrounding rim of erythema, which were both diagnostic of peripheral T-cell lymphoma (PTCL). This case is a unique example of PTCL with erysipelaslike spread.     

皮下脂膜炎样T细胞淋巴瘤1例

报道1例皮下脂膜炎样T细胞淋巴瘤，患者男性，25岁，临床表现皮下结节伴高热，组织病理检查示肿瘤细胞（UCHL1阳性）主要累及皮下脂肪组织，呈脂膜多样生长，伴多量组织细胞（CD68阳性）反应性增生，给予CHOP方案，病情持续恶化，确诊后半年死亡。     

皮下脂膜炎样T细胞淋巴瘤1例

报道1例皮下脂膜炎样T细胞淋巴瘤。患者女，34岁。全身反复出现肿块、结节性损害1年。4个月前部分肿块破溃，伴发热、乏力、贫血等症状。皮损组织病理显示皮下脂肪层的淋巴细胞、组织细胞浸润，脂膜炎样T细胞瘤。免疫组化证明下细胞来源，伴有TCRγ基因克隆性重排，诊断为脂膜炎样T细胞瘤。     

皮下脂膜炎样T细胞淋巴瘤

报告1例皮下脂膜炎样T细胞淋巴瘤。患者男,26岁。曾无防护地接触放射性元素钴3年。躯干部出现暗红色肿块1．5个月,伴有全血细胞减少。皮损组织病理学改变主要为真皮中下部及皮下脂肪层弥漫性瘤细胞浸润。免疫组化染色显示LCA（＋）、CD45RO（＋）、CD3（＋）、CD2（＋）、CD8（＋）、TIA-1（＋）、CD20（-）。     

皮下脂膜炎样T细胞淋巴瘤一例

患者女,39岁.全身反复红斑、结节8个月,伴破溃、发热2个月.8个月前不明原因于左小腿胫前出现红斑及皮下结节,皮损逐渐增大,约5个月后全身多处分批出现相同皮损.2个月前全身各处皮损破溃,并发热,发热以夜间明显,体温波动于38℃～39℃之间,自觉乏力.经当地医院诊断为脂膜炎,给予"青霉素,头孢菌素"等抗炎治疗无效后就诊于我院.     

Unusual case of subcutaneous panniculitis-like T-cell lymphoma

An unusual case of subcutaneous panniculitis-like T-cell lymphoma is presented involving multiple organ systems, which eventually culminated in rapid demise from the haemophagocytic syndrome, after an initial protracted course. A 44-year-old man presented in April 2001 with bronchiolitis obliterans organising pneumonia that initially responded well to corticosteroids. However, the condition relapsed on attempted prednisone withdrawal in January 2002 and the patient was noted to have developed truncal subcutaneous nodules. Initial skin biopsy revealed lobular panniculitis, with negative microbiological culture. In July 2002, mononeuritis multiplex was diagnosed after the patient presented with paresthesiae and was treated with pulse cyclophosphamide therapy. By November 2002 there was ulceration of the subcutaneous nodules. Repeat skin biopsy revealed subcutaneous panniculitis-like T-cell lymphoma. The clinical manifestations were supportive of an unifying diagnosis of malignancy involving pulmonary, cutaneous and nervous systems. Combination chemotherapy with fludarabine, mitoxantrone and dexamethasone was commenced. However, the patient deteriorated, with fevers, weight loss, pancytopenia and laboratory features consistent with the haemophagocytic syndrome. Despite maximal supportive therapy the patient succumbed to his disease.     

皮下脂膜炎样T细胞淋巴瘤

报告1例皮下脂膜炎样T细胞淋巴瘤.患者女,49岁.四肢及躯干出现皮下结节、肿块1年,左小腿肿块破溃2个月余,近2个月有低热、乏力、纳差、贫血.皮损组织病理检查示皮下脂肪层内弥漫性异形淋巴细胞浸润,并见局灶性脂肪坏死及噬血细胞现象.免疫组化染色结果示:CD3、CIM3、CD56、CD68均( )、CD30、CD20均(-).     

脂膜炎样T细胞淋巴瘤1例

报告1例皮下脂膜炎样T细胞淋巴瘤,患者女,44岁,左侧臀部出现红肿、结节,伴疼痛和发热半个月,皮损组织病理检查：皮下脂肪层有淋巴细胞、组织细胞浸润,免疫组化证明浸润淋巴细胞为T细胞来源,组织病理改变符合脂膜炎样T细胞淋巴瘤。     

Subcutaneous panniculitic-like T-cell lymphoma and other primary cutaneous lymphomas with prominent subcutaneous tissue involvement

The concept of subcutaneous T-cell lymphoma defines a reduced group of primary cutaneous lymphomas characterized morphologically by a prominent or exclusive subcutaneous tissue involvement. Subcutaneous panniculitic-like T-cell lymphoma is a rare subtype of primary cutaneous T-cell lymphoma clinically mimicking panniculitis. The clinical course is usually protracted with recurrent cutaneous lesions but rarely with early extracutaneous dissemination. The clinical, histopathologic, immunophenotypic, and evolutive features of this heterogeneous and rare group of primary cutaneous lymphomas are reviewed.     

Phagocytized apoptotic cells in subcutaneous panniculitis-like T-cell lymphoma

We reported on a case of subcutaneous panniculitis-like T-cell lymphoma (SPTCL) with multiple erythematous nodular lesions on the extremities, trunk and face. Histological examination of an excised lesion revealed a dense infiltrate of markedly atypical T-lymphoid cells expressing the CD8+ phenotype located in the subcutaneous tissue with histiocyte-phagocytizing apoptotic cells. The 'bean-bag' histiocytic cells, the characteristic finding of SPTCL, are considered to be products of haemophagocytosis. In our case the 'bean-bag' cells were produced by phagocytosis of apoptotic bodies, as confirmed by electron microscopy. It is suspected that 'bean-bag' cells are related not to haemophagocytosis but to phagocytosis of apoptotic cells in the CD8+ T-cell type of SPTCL.     

皮下脂膜炎样T细胞淋巴瘤与皮肤结外鼻型NK/T细胞淋巴瘤的鉴别

皮下脂膜炎样T细胞淋巴瘤和皮肤结外鼻型NK/T细胞淋巴瘤是两类特殊而少见的皮肤淋巴瘤.当后者累及皮下脂肪组织时,二者在临床表现、组织病理、免疫表型上有重叠,鉴别非常困难.该文对这两种皮肤淋巴瘤与EB(Epstein-Barr)病毒感染的关系、临床表现、组织病理学特点、免疫表型、分子遗传学特征及预后等方面作一对比性综述.     

Extranodal NK/T-cell lymphoma with extensive subcutaneous involvement,mimicking subcutaneous panniculitis-like T cell lymphoma

A 22‐year‐old man had a subcutaneous nodule on the left thigh for 3 months. Physical examination showed an ulcerative nodule on the left thigh ( Fig. 1 ). He suffered from a fever for 1 week. Laboratory tests revealed a white blood cell count of 7180/mm³, with 22.4% neutrophils, 68.5% lymphocytes, 8.4% monocytes, 0.3% eosinophils, and 0.4% basophils. Hemoglobin was 9.0 g/dL, hematocrit was 28.1%, and the platelet count was 202 × 10⁹/L. Analysis of serum showed the following abnormal results: aspartate aminotransferase, 102 IU/L (normal, 13–37); alanine aminotransferase, 77 IU/L (normal, 7–43); alkaline phosphatase, 1296 (normal, 70–270); LDH, 1105 (normal, 120–520). Results of other laboratory tests including serum electrolytes, blood and tissue culture, and viral markers for hepatitis were negative or normal. Ultrasonography and computed tomography of abdomen and pelvis showed hepatosplenomegaly with ascites. Whole body bone scan showed an increased long bone activity of the left leg. Bone marrow aspiration was negative for neoplastic cells. Histologic examination from the thigh nodule revealed subcutaneous lymphoid infiltrates with involvement of the deep dermis. Examination at high power magnification showed small, medium, and large‐sized, pleomorphic lymphoid cells with hyperchromatic nuclei, nuclear debris, and phagocytic macrophages. Fat necrosis, partial rimming of fat spaces and clustering around blood vessels by pleomorphic lymphoid cells were also seen ( Fig. 2 ). The immunohistochemical studies on paraffin sections revealed that most of the infiltrated lymphoid cells positively stained for CD56 ( Fig. 3 ), granzyme B, TIA‐1, cytoplasmic CD3, and CD45RO, whereas CD4, CD8, CD20, and CD30 showed no immunoreactivity. Clonality of the infiltrate was analyzed by polymerase chain reaction (PCR). Nested PCR was performed using FR3A and VLJH as primers for IgH gene and Vγ – Jγ for T‐cell receptor γ chain gene. Polymerase chain reaction analysis showed a distinct band for TCR gene, confirming T‐cell monoclonality of infiltrating lymphocytes. EBV‐encoded small nuclear RNA was evident in lesional tissue by in situ hybridization. He was treated with two cycles of chemotherapy with CHOP‐BV (Cytoxan 735 mg, adriamycin 45 mg, vincristine 1.45 mg, prednisolone 75 mg, bleomycin 15 mg, VP‐16 150 mg). He died 5 days after chemotherapy.

Figure 1 Open in figure viewer PowerPoint An ulcerative nodule on the left thigh     

儿童皮下脂膜炎样T细胞淋巴瘤的研究

目的 探讨儿童皮下脂膜炎样T细胞淋巴瘤(SPTL)的临床病理、免疫表型、基因重排及EB病毒(EBV)感染情况。方法 将收集的5例儿童SPTL作临床病理和免疫组化分析,采用PCR检测TCRγ、IgH基因重排,运用EBER1/2原位杂交检测EBV感染。结果 男4例、女1例,年龄9～13岁,主要表现为无症状的结节、斑块或肿块。组织学上肿瘤在皮下脂肪内呈脂膜炎样浸润,细胞大小不等、异形性明显,瘤内可见豆袋细胞、上皮样肉芽肿和小片状坏死。瘤细胞表达T细胞标记βF1、CD2、CD3、CD8、CD45RO和细胞毒颗粒相关蛋白标记TIA-1、粒酶B,不表达CD4、CD20。4例检出单克隆性TCRγ基因重排,未检出IgH基因重排。EBER1/2原位杂交阳性2例。获随访的3例中1例死于本病,该例EBER1/2阳性。结论 儿童SPTL面部易受累,全身症状多见,噬血细胞综合征发生率与死亡率较高。伴有EBV感染者可能预后较差。     

皮下脂膜炎样T细胞淋巴瘤研究进展

皮下脂膜炎样T细胞淋巴瘤是指主要累及皮下脂肪细胞且与脂膜炎相似的一种原发于皮肤的外周T细胞淋巴瘤。本文对其临床特点、组织病理学特点、实验室检查、组织学来源,与EB病毒感染的关系、治疗及预后、诊断及鉴别诊断综述如下。     

Peripheral T-cell lymphoma presenting as lipoatrophy and nodules

BACKGROUND: Cutaneous lymphomas have many morphologic forms and clinical features. Lymphoma presents rarely with a constellation of nodules, panniculitis, and localized lipoatrophy. The histopathologic similarities of lymphoma and connective tissue disease panniculitis may create a diagnostic challenge. METHODS: We retrospectively reviewed the case of a 47-year-old man who presented 15 years earlier with recurrent fevers, fatigue, tender subcutaneous nodules, and facial, trunk, and extremity lipoatrophy. RESULTS: Initial biopsy of a cutaneous nodule showed lymphohistiocytic panniculitis without atypical inflammatory cells. Serologic tests showed negative connective tissue serologies, yet the initial clinical impression was most consistent with lupus panniculitis. Initially, the patient was treated with oral prednisone; later, steroid-sparing agents were used with modest improvement evidenced by resolution of his systemic symptoms and stabilization of the cutaneous findings. A decade later, more pronounced facial lipoatrophy, new facial nodules, and posterior thigh pain developed. Laboratory testing showed leukopenia, elevated liver and muscle enzymes, hypertriglyceridemia, and a low level of high-density lipoprotein. Biopsy of a new chin nodule indicated peripheral T-cell lymphoma, whereas an evaluation for systemic malignant involvement was negative. The patient was started on chemotherapy, which resulted in stabilization of the lipoatrophy and decreasing size and frequency of the cutaneous nodules, but the posterior thigh pain persisted. CONCLUSIONS: We report a rare case of lymphoma presenting as nodules and profound lipoatrophy, which exemplifies the complexity of lymphomas. Profound lipoatrophy and panniculitis may be an unusual and diagnostically challenging presentation of cutaneous lymphoma.     

Clinicopathological characterization and genomic aberrations in subcutaneous panniculitis-like T-cell lymphoma

Subcutaneous panniculitis-like T-cell lymphomas (SPTLs) represent a rare, difficult-to-diagnose, and poorly characterized subtype of cutaneous T-cell lymphomas (CTCLs) affecting younger people more than the other CTCL forms. We performed a thorough clinical, immunohistological, and molecular analysis of nine Finnish SPTL patients. Specifically, we performed single-cell comparative genomic hybridization (CGH) from laser microdissected, morphologically malignant SPTL cells, as well as loss of heterozygosity (LOH) and fluorescence in situ hybridization (FISH) analysis for the NAV3 (neuron navigator 3) gene. CGH revealed large numbers of DNA copy number changes, the most common of which were losses of chromosomes 1pter, 2pter, 10qter, 11qter, 12qter, 16, 19, 20, and 22 and gains of chromosomes 2q and 4q. Some of the DNA copy number aberrations in SPTL, such as loss of 10q, 17p, and chromosome 19, overlap with those characteristic of common forms of CTCL (mycosis fungoides (MF) and Sezary syndrome (SS)), whereas 5q and 13q gains characterize SPTL. Allelic NAV3 aberrations (LOH or deletion by FISH), previously found in MF and SS, were identified in 44% of the SPTL samples. This study demonstrates that SPTL is also moleculocytogenetically a uniform entity of CTCL and supports the current World Health Organization-European Organization for Research and Treatment of Cancer (WHO-EORTC) classification defining SPTL as a subgroup of its own.