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.     

Subcutaneous panniculitis-like T-cell lymphoma in a 26-month-old child with a review of the literature

Subcutaneous panniculitis-like T-cell lymphoma is a rare cytotoxic T-cell lymphoma of the skin that preferentially infiltrates the subcutaneous tissue. We report here this lymphoma occurring in a 26-month-old Korean girl. She presented with multiple erythematous subcutaneous nodules on both extremities and her back along with systemic symptoms. She had a protracted course of multiple erythematous subcutaneous nodules for 1 month and a spiking fever was often noted. The histopathologic findings for the subcutaneous nodules were lobular panniculitis-like material that was composed of atypical lymphocytes and histiocytes. The atypical lymphocytes characteristically rimmed individual fat cells in a lace-like pattern and some of the histiocytes showed phagocytosed white blood cells occasionally. Immunophenotypic studies showed CD3(+), CD45RO(+), CD20(-), CD4(-), CD8(+), and CD56(-). She is currently being treated with combination chemotherapy of cyclophosphamide, doxorubicin, vincristine, and prednisolone.     

From circumscribed scleroderma (morphea) to subcutaneous panniculitis-like T-cell lymphoma: case report

Subcutaneous panniculitis-like T-cell lymphoma is a rare form of non-Hodgkin lymphoma. It presents clinically as panniculitis, with erythematous, firm subcutaneous infiltrates and recurrent papulonodules. A 45-year-old male Croat presented with a 6-month history of tender erythematous subcutaneous skin lesions without systemic symptoms. Analysis of a lesion biopsy specimen showed circumscribed scleroderma (morphea). During hospitalization, skin biopsies from two subcutaneous lesions on the stomach were repeated, and pathohistologic analysis corresponded primarily to panniculitis in the context of morphea. Pathohistologic analysis and immunohistochemistry of a specimen obtained by tumor excision from the back indicated subcutaneous panniculitis-like T-cell lymphoma. Histologic analysis revealed a dense infiltrate of atypical T-lymphoid cells expressing CD8(+) phenotype located in the subcutaneous tissue with histiocyte-phagocytizing apoptotic cells. Specific hematologic analysis and medical treatment were continued. This case report illustrates the importance of continuous follow-up with repeat biopsy and use of immunohistologic techniques for early diagnosis.     

Rimming of adipocytes by neoplastic lymphocytes: a histopathologic feature not restricted to subcutaneous T-cell lymphoma

Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare cytotoxic T-cell lymphoma of the skin presenting with histopathologic features simulating those of a lobular panniculitis. The presence of neoplastic T-lymphocytes forming a rim around the individual fat cells in the subcutaneous lobules, so-called "rimming" of adipocytes, is considered a characteristic morphologic feature of this type of cutaneous lymphoma. In this study we reviewed a series of 45 biopsy specimens of primary and secondary cutaneous B- and T-cell lymphomas and one of myeloid leukemia involving the subcutaneous tissues and showing rimming of adipocytes (subcutaneous panniculitis-like T-cell lymphoma: n = 16; mycosis fungoides, tumor stage: n = 3; aggressive epidermotropic CD8(+) T-cell lymphoma: n = 2; cutaneous gamma/delta T-cell lymphoma: n = 4; extranodal NK/T-cell lymphoma, nasal type: n = 4; cutaneous medium-large pleomorphic T-cell lymphoma, NOS: n = 5; CD4(+)/CD56(+) hematodermic neoplasm (blastic NK-cell lymphoma): n = 7; secondary cutaneous large B-cell lymphoma: n = 3; secondary cutaneous lymphoplasmacytic lymphoma: n = 1; specific cutaneous manifestations of acute myelogenous leukemia: n = 1). We could demonstrate that rimming of adipocytes by neoplastic cells can be recognized not only in subcutaneous panniculitis-like T-cell lymphoma, but also in several different entities of malignant lymphoma with skin involvement. Precise classification of cases with prominent involvement of the subcutaneous tissues can only be achieved upon precise correlation of clinicopathologic and phenotypic features. Rimming of adipocytes should not be considered specific of subcutaneous panniculitis-like T-cell lymphoma.     

Subcutaneous panniculitis-like T-cell lymphoma with vacuolar interface dermatitis resembling lupus erythematosus panniculitis

Lupus erythematosus (LE) panniculitis (LEP) is a form of chronic cutaneous LE most often characterized by erythematous subcutaneous nodules. The histopathology of LEP may be distinctive, allowing a diagnosis even in the absence of any other LE features. Lymphocytic infiltration of the panniculus is termed lymphocytic lobular panniculitis. This entity has been reported in both LEP and subcutaneous panniculitis-like T-cell lymphoma. We describe a 67-year-old woman who presented with multiple dermal and subcutaneous nodules on her legs and hips. Biopsy specimens of roughly 15 lesions were interpreted as being diagnostic of LEP, however, with each subsequent biopsy specimen more atypical cells were observed. The patient responded only to moderate doses of oral prednisone, and failed intralesional triamcinolone acetonide, oral immunosuppressive agents, oral antimalarial agents, and oral thalidomide. Three years after the onset of her disease, ulcerated plaques and nodules developed. At this time a T-cell receptor gene rearrangement was present and subcutaneous panniculitis-like T-cell lymphoma was diagnosed. Despite multiple doses of chemotherapy she died approximately 1 year after diagnosis. Patients with apparent LEP that have atypical lymphocytes in their biopsy specimens should be followed up closely for the development of T-cell malignancy.     

Cytotoxic γ/δ subcutaneous panniculitis-like T-cell lymphoma: report of a case with pulmonary involvement unresponsive to therapy

Peripheral subcutaneous panniculitis-like T-cell lymphoma (PSPTCL) is a rare form of cutaneous lymphoma recently proposed as a distinct clinicopathological entity. It usually presents with multiple indurated subcutaneous plaques or tumours, most commonly located on the extremities and trunk and clinically mimicking lobular panniculitis. Associated constitutional symptoms due to haemophagocytic syndrome may advance or, more often, complicate the clinical course in about 40-70% of cases. Finding of TIA-1+ and perforin + cytolytic granules in atypical pleomorphic lymphocytes suggests PSPTCL origin from granular cells of T-cell or natural killer cell phenotype. Cells have a CD3+ CD4+ CD8- or CD3+ CD4- CD8+ T-cell phenotype. Moreover, these lymphomas can express natural killer cell associated antigens, such as CD56, especially in gamma/delta variants. PSPTCL following an indolent clinical course with recurrent self-healing lesions have been described. The prognosis of most PSPTCL is poor even when treated with aggressive chemotherapy. This paper reports a case of PCTCL in a young woman with T-cytotoxic differentiation, with rapid progression unresponsive to several treatments.     

Indolent subcutaneous panniculitis-like T cell lymphoma in a 1-year-old child

Subcutaneous panniculitis-like T-cell lymphoma is an uncommon form of CD8-positive cytotoxic T-cell lymphoma of the skin that predominantly affects the subcutaneous tissue and is extremely rare in early childhood (<3 yrs). Here, we present an early pediatric case with an indolent form of subcutaneous panniculitis-like T-cell lymphoma occurring at 12 months old. The subcutaneous nodules gradually disappeared spontaneously, and the girl showed excellent prognosis with no aggressive treatment.     

儿童皮下脂膜炎样T细胞淋巴瘤合并嗜血细胞综合征一例附文献复习

患儿,女,8岁。皮肤结节、斑块8个月,右大腿肿胀1个月,伴发热1周。肝脏肋下约1~2 cm可触及、脾脏肋下约2~3 cm可触及,质软,无触痛。实验室检查:血细胞降低,肝酶、血脂、乳酸脱氢酶、C反应蛋白升高,血沉加快,凝血功能异常,铁蛋白升高。免疫组化:CD3、CD8、颗粒酶B、βF1阳性,Ki-67:约50%(+)。骨髓活检:骨髓增生活跃。皮损病理检查:皮下脂肪见异型细胞。诊断为脂膜炎样T细胞淋巴瘤合并嗜血细胞综合征。我们对相关文献进行了复习。     

Subkutanes pannikulitisartiges T-Zell-Lymphom

Histopathology, immunohistochemical, and molecular genetic findings revealed the diagnosis of subcutaneous panniculitis-like T-cell-lymphoma in two patients, aged 44 and 70 years. The clinical morphology of the lymphoma manifestations showed varied significantly. One patient presented with a singular erythematous nodule in the chin region. The other patient suffered from extended plate-like resistances and atrophy of the face, upper arms and left breast. Hemophagocytic syndrome was not present in either patient. Prognosis of subcutaneous panniculitis-like T-cell lymphoma without associated hemophagocytic syndrome is reported to be favorable. Radiotherapy of the singular lesion on the chin and systemic corticosteroids of the extended plaques induced complete remission in both patients.     

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(-).     

Nasal type natural killer/T-cell lymphoma with subcutaneous panniculitis-like involvement: Association with a poor prognosis

We report the case of a 23-year-old Japanese man with nasal type natural killer (NK)/T-cell lymphoma and subcutaneous panniculitis-like involvement in the skin. The neoplastic cells possessed the following characteristics of NK cells: expression of cytoplasmic CD3ϵ, CD56, T-cell intracellular antigen-1, azurophilic granules in the cytoplasm, Epstein-Barr virus-encoded small nuclear RNA, and germline configurations of T-cell receptor-β and immunoglobulin heavy chain J genes in the involved tissue. Despite multiple courses of several chemotherapeutic regimens, the cutaneous lesions as well as his general condition deteriorated, and he died of subarachnoidal bleeding. We gathered 14 available reported cases of NK/T-cell lymphoma with subcutaneous panniculitis-like involvement and reviewed the 15 cases, including our patient. They were all of Asian descent. Of the 15 patients (5 men, 10 women), the mean age was 47 years (range, 22-77 years). They had frequent common systemic symptoms: hepatosplenomegaly, weight loss, general weakness, and arthralgia. Twelve of the 15 patients died of disease. The mean duration from diagnosis to death was 9.5 months. NK/T-cell lymphoma with subcutaneous panniculitis-like involvement is associated with a poor prognosis.     

Aggressive NK-cell leukaemia associated with reactive haemophagocytic syndrome

We report a case of aggressive NK-cell leukaemia associated with reactive haemophagocytic syndrome in a 29-year-old Korean woman who had several small purpuric patches on both thighs. She also had high fever. Laboratory tests revealed pancytopenia and deranged liver function, and atypical lymphocytes containing toxic granules were detected from peripheral blood and bone marrow. The bone marrow examination showed diffuse histiocytic proliferation with several haemophagocytic macrophages, suggesting an associated reactive haemophagocytic syndrome. Skin biopsy from her thigh lesion demonstrated atypical CD56+ lymphoid cellular infiltrates with angiocentric pattern, and in situ hybridization test for Epstein-Barr virus was positive. Although we treated her with chemotherapy, she died 1 month later.     

Panniculitis-like T-cell lymphoma clinically manifested as alopecia

A 45-year-old women presented with multifocal scalp lesions with the clinical impression of alopecia areata. Histological findings first suggested cytophagic histiocytic panniculitis, although a 'burned-out' panniculitis-like T-cell lymphoma could not be excluded. After a 20-month follow-up period, assessment of the T-cell receptor gamma-chain gene rearrangement verified the diagnosis of subcutaneous panniculitis-like T-cell lymphoma. This case is interesting because of its isolated scalp manifestation as well as its indolent course.     

Transformation of cutaneous gamma/delta T-cell lymphoma following 15 years of indolent behavior

Subcutaneous gamma/delta (gamma/delta) T-cell lymphoma is a rare lymphoma, characterized by its unique immunophenotype and clinical course. It has been shown to behave more aggressively than its counterpart bearing the alpha/beta receptor and has recently been removed from the subcutaneous panniculitis-like T-cell lymphoma category for this very reason. We present a case of a patient with a 15-year running diagnosis of panniculitis. Following these years of indolent behavior, the disease began an aggressive clinical course and she was diagnosed with gamma/delta T-cell lymphoma. Molecular analysis identified a T-cell clone, which through retrospective analysis, was also shown to be present in the patient's original biopsy material. We present this case as a rare example of initial indolent behavior in a lymphoma typically considered very aggressive.     

皮下血管内大B细胞淋巴瘤

报告1例皮下血管内大B细胞淋巴瘤.患者男,73岁.因双侧大腿皮下结节,于2007年2月初就诊.皮损组织病理检查见血管腔内有大量肿瘤细胞聚集,免疫组化染色结果示肿瘤细胞CD20阳性,诊断为皮下血管内大B细胞淋巴瘤(IVLBCL).予环磷酰胺、长春新碱、泼尼松(COP方案)化疗后皮损缩小.IVLBCL恶性程度较高,临床表现无特异性,预后较差,皮损组织病理检查有助于诊断.     

Cutaneous malakoplakia simulating relapsing malignant lymphoma

This report describes the case of a 42-year-old man with malignant lymphoma, diffuse large non-cleaved cell type, who developed cutaneous malakoplakia in the left groin. The patient had widespread lymph node involvement, including a left inguinal mass which was clinically thought to represent recurrent lymphoma. The inguinal mass failed to regress after chemotherapy and irradiation, although lymphoma in other sites responded to chemotherapy. A skin biopsy of the area showed an ulcer and an abscess involving the dermis and subcutaneous tissue. Microscopically, a diffuse infiltrate of foamy histiocytes was seen with numerous intracellular and extracellular, round and laminated bodies. Some of these bodies had a "targetoid" appearance, stained strongly with von-Kossa's calcium stain and showed the typical appearance of Michaelis-Gutmann bodies by electron microscopy. Cultured monocytes from the peripheral blood of the patient showed ultrastructural features similar to their tissue counterparts, suggesting a systemic involvement of the monocyte macrophage lineage. This case represents an unusual presentation of malakoplakia of the skin associated with relapsing malignant lymphoma in a patient on immunosuppressive drugs.     

Lupus erythematosus panniculitis in children: report of three cases and review of previously reported cases

Lupus erythematosus panniculitis (LEP) is a rare finding in children, with only 12 fully reported prior cases in the English literature. We describe three cases of LEP in children younger than 18 and compare them to previous cases reported in the literature. We examine laboratory tests performed, biopsy results, age at onset and diagnosis, presence or absence of systemic symptoms, and outcomes after treatment. It is unknown what the risk is of these patients developing future systemic lupus erythematosus. We also discuss the relevance of subcutaneous panniculitis-like T-cell lymphoma, because the clinical and pathologic pictures are similar in presentation.     

儿童皮肤非何杰金淋巴瘤与EB病毒的相关性研究

目的研究EB病毒与儿童皮肤非何杰金淋巴瘤(nonhodgkin’lymphoma,NHL)发病的关系。方法采用原位杂交法对36例儿童皮肤NHL的标本进行EB病毒编码的小分子寡核苷酸(Epstein-Barr viralencoded RNA,EBER)检测。结果36例中EBER阳性7例(19.4%),其中皮下脂膜炎样T细胞淋巴瘤(subcutaneous panniculitis-like T-celllym-phoma,SPTCL)5例;外周T细胞淋巴瘤、Burkitt’s瘤各1例;CD30+间变型大细胞淋巴瘤、T淋巴母细胞性淋巴瘤均未见阳性表达。结论EB病毒感染与皮下脂膜炎样T细胞淋巴瘤可能有关,与T淋巴母细胞性淋巴瘤、CD30+间变型大细胞淋巴瘤关系不明显,其与累及皮肤的Burkitt’淋巴瘤的关系有待进一步证实。     

Manual microdissection technique in a case of subcutaneous panniculitis-like T-cell lymphoma: a case report and review

Demonstration of T-cell receptor gene monoclonality often plays an important role in the diagnosis of T-cell lymphoma. When a test to detect monoclonality is performed on whole tissue sections, the presence of a reactive lymphocyte population may reduce sensitivity. This may be especially true for early or borderline cases of lymphoma. Microdissection techniques may be utilized to more readily identify a clonal population of lymphocytes. Subcutaneous panniculitis-like T-cell lymphoma represents a cutaneous lymphoid neoplasm whose clinical course may vary from an indolent, waxing and waning course to an aggressive course resulting in death. We report the first case of a microdissection technique used to facilitate diagnosing a case of subcutaneous panniculitis-like T-cell lympoma.     

Cutaneous lymphomas other than mycosis fungoides in Singapore: a clinicopathological analysis using recent classification systems

BACKGROUND: Cutaneous lymphomas other than mycosis fungoides (MF) are a heterogeneous group with wide variations in clinical presentation, biological behaviour and prognosis. New classification systems have been designed or proposed in recent years, with well-defined disease entities and emphasis on the importance of site. OBJECTIVES: This study aims to analyse a series of non-MF lymphomas in an institution-based dermatological setting in Singapore, based on the European Organization for Research and Treatment of Cancer (EORTC) classification and the World Health Organization (WHO) classification. A secondary objective is to highlight the clinical utility of both classification systems. PATIENTS AND METHODS: Forty cases diagnosed over a 12-year period were examined by immunohistochemistry with antibodies targeting CD3, CD4, CD5, CD8, CD20, CD30, CD43, CD45RO, CD56 and CD68 in paraffin-embedded specimens. The immunohistological diagnosis was correlated with the clinical presentation and staging investigations for the final diagnosis and the course of disease recorded. RESULTS: Non-MF T-cell lymphomas presenting in the skin comprised 31 cases (78%) and were 3(1/2) times more common than B-cell lymphomas, which comprised nine cases (22%). The common subtypes were lymphomatoid papulosis, CD30+ large cell cutaneous T-cell lymphoma and subcutaneous panniculitis-like T-cell lymphoma. The commonly ascribed B-cell pattern with infiltrates in the mid and deep dermis and perivascular spaces was seen in 60% of T-cell lymphomas. Overall, there were equal numbers of primary cutaneous T-cell lymphomas and those due to concurrent or secondary cutaneous lymphoma. Five of six cases of subcutaneous panniculitis-like T-cell lymphoma had concurrent cutaneous and systemic involvement and their median survival was 7 months. CONCLUSIONS: The predominance of cutaneous T-cell lymphomas in this case series closely matched that reported from east Asia; cutaneous B-cell lymphomas are much less common than in Europe. The EORTC classification, which is designed only for primary cutaneous lymphomas, should be used in conjunction with the WHO classification because of the high prevalence of cutaneous lymphomas as the secondary site of disease from systemic lymphoma. In addition, subcutaneous panniculitis-like T-cell lymphoma is a primary cutaneous lymphoma where systemic involvement is common at initial presentation. We propose full immunophenotyping and complete clinical evaluation with staging investigations for all patients presenting with cutaneous lymphomas other than MF.