Subcutaneous panniculitis-like T-cell lymphoma with hemophagocytic syndrome: A case report

INTRODUCTION@#Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare disease, accounting for less than 1 % of non-Hodgkin's lymphomas that is characterized by infiltration of T-cells in the subcutaneous adipose tissue.@*CASE PRESENTATION@#A 21-year-old Filipino female presented with intermittent fever which eventually was associated with multiple eruption of cutaneous lesions. The serum antinuclear antibody (ANA) titer and pattern revealed negative results. Further clinical investigation prompted a skin lesion punch biopsy revealing atypical lymphoid infiltrates with lobular panniculitis consistent with SPTCL. lmmunohistochemical studies stained strongly posItIve for CD3, CDS, granzyme B and negative for CD20, CD4, and CD56. The case was also compounded with the existence of hemophagocytic syndrome having fulfilled five of the eight criteria . She was given prednisone (1 mg/kg/day) with gradual resolution of cutaneous lesions leaving marked hyperpigmentation and lipoatrophy.@*DISCUSSION@#SPTCL presents with a myriad of systemic symptoms but will always present with skin lesions. The biopsy for histopathological interpretation is commonly performed as a first diagnostic step followed by immunohistochemical staining. Clinical presentation, histo-pathological findings, and 1mmunohistochemical results together clinched the diagnosis of SPTCL.@*CONCLUSION@#Patients with history of intermittent fever associated with cutaneous lesions and systemic clinical findings, SPTCL should be a differential diagnosis. However, lupus erythematosus panniculitis (LEP) must be ruled out first as its closest mimicker. This case report along with other large case series provides further evidence that monotherapy with oral prednisone can be an initial choice of therapy.

Hemophagocytic lymphohistiocytosis: A rare, potentially fatal complication in subcutaneous panniculitis like T cell lymphoma

Subcutaneous panniculitis-like T cell lymphoma is a rare subtype of cutaneous lymphomas with distinct clinical, histological and immunophenotypic characteristics, as well as an indolent clinical course. Rarely, it may be complicated with hemophagocytic lymphohistiocytosis: a hyperinflammatory syndrome which, if not diagnosed early, carries a dismal outcome. In this article, we describe a case of subcutaneous panniculitis-like T cell lymphoma in a middle-aged female patient which was complicated with secondary hemophagocytic lymphohistiocytosis with a favorable outcome following etoposide-based therapy. The various histological mimics of subcutaneous panniculitis-like T cell lymphoma and the management options are also briefly discussed.

Cyclosporin for treatment of subcutaneous panniculitis-like T-cell lymphoma: report of one case and review of literature / 白血病·淋巴瘤

Objective@#To investigate the effect of cyclosporin (CsA) on initial, relapsed or refractory subcutaneous panniculitis-like T-cell lymphoma (SPTCL).@*Methods@#The clinical data of one case with long-survival SPTCL in China-Japan Friendship Hospital was retrospectively analyzed, and the literature was reviewed.@*Results@#After CsA treatment as the initial therapy, the patient obtained rapid response and complete remission (CR). Lymphoma occurred relapse several times, but the use of CsA could got rapid remission. CR sustained from 1 to 6 years after drug withdrawal. However, CsA did not bring remission after the recent relapse. Then CHOP-like regimen was carried out, and partial remission could be reached. The patient achieved CR and 22 years survival time with CsA maintenance therapy until now.@*Conclusion@#CsA has a favorable effect on initial, relapsed or refractory SPTCL.

Clinical analysis of 14 cases of rare non-Hodgkin lymphoma in children / 中华实用儿科临床杂志

Objective To analyze the clinical characteristics and prognosis of 4 rare types of non -Hodgkin lymphoma(NHL)in children,and to discuss the progress in treatment.Methods Clinical data of 1 4 patients with rare types of NHL at Shanghai Children′s Medical Center,Shanghai Jiaotong University School of Medicine between January 2004 and December 201 4 were retrospectively analyzed,and their clinical features,treatment and prognosis were dis-cussed.Results Fourteen cases were reported including 6 subcutaneous panniculitis -like T -cell lymphoma (SPTCL),3 hydroa vacciniforme -like cutaneous lymphoma(HVLL),2 pediatric follicular lymphoma(PFL)and 3 ex-tranodal NK/T -cell lymphoma,and nasal type(ENKTL).Ten patients (71 .4%)primarily presented with skin lesions and underwent a long course of illness before they were finally diagnosed (the median was 1 0 months),71 .4%(1 0 /1 4 cases)of them associated with fever and 50.0%(7 /1 4 cases)with liver and spleen enlargement,and no evidence of central nervous system (CNS)and bone marrow (BM)involvement was observed,while 28.6% patients (4 /1 4 cases) had more than two lines′abnormalities in peripheral blood examination.Since there were no standard treatment guide-lines,most patients received CHOP (Cyclophosphamide +Adriamycin +Vincristine +Prednisone)and /or mature B -cell NHL -like therapy,and 50.0%(7 /1 4 cases)of them received interferon therapy in addition,while 1 patient re-ceived allogeneic hematopoietic stem cell transplantation after recurrence.The complete remission was achieved in 71 .4%(10 /14 cases)of all the patients.Two of them died,3 lost follow -up,and 1 relapsed.The 3 -year overall survi-val and event free survival rates were 0.84 and 0.57,respectively,after a median follow -up of 26 months (range 12 -64 months).Conclusions Pediatric rare types of NHL show atypical clinical manifestation,low incidence of CNS /BMinfil-tration and long course.It is hard to make pathological diagnosis and differentiation.It is also inappropriate to apply the commonly used staging system to these rare types of NHL.No standard treatment has been found by now.SPTCL,HVLL and PFL have relatively good outcomes when treated with mature B -cell NHL -type therapy plus interferon therapy.

Treatment analysis of liver cirrhosis with hepatitis B complicated by subcutaneous panniculitis-like T-cell lymphoma associated hemophagocytic syndrome / 药学实践杂志

Objective To investigate the clinical features and treatment of liver cirrhosis with hepatitis B complicated by subcutaneous panniculitis-like T-cell lymphoma (SPTCL) associated hemophagocytic syndrome (HPS) .Methods A retrospec-tive analysis of case clinical data with liver cirrhosis complicated by SPTCL associated HPS was done in August 2014 in our hospital .Results Because of different phenotypes of T cell receptor (TCR) ,the aggression ,treatment response and prognosis of the disease were significantly different .The patients with HPS had poor treatment effect and short survival period .Conclu-sion Liver cirrhosis with hepatitis B complicated by SPTCL associated HPS is rare ,Bone marrow morphology ,pathology , immunohistochemistry and gene rearrangement detection as soon as possible are important for early diagnosis .To control HBV early and effectively is particularly important .Early diagnosis and treatment are important to prolong survival .

Atypical Lymphocytic Lobular Panniculitis / 대한피부과학회지

Atypical lymphocytic lobular panniculitis (ALLP) is a recently described entity characterized by self-limiting plaques that manifest a waxing and waning course. Although ALLP is known as a benign condition, it needs adequate follow-up to determine the risk of progression to subcutaneous panniculitis-like T-cell lymphoma (SPTCL) because ALLP has morphologic and biologic similarities with SPTCL. We report a case of ALLP in a 13-year-old girl who presented with an unusual tumor-like erythematous mass on the left thigh but without systemic symptoms or laboratory abnormalities. The histopathologic findings showed a dense and diffuse infiltration of the fat lobules by small-to-medium-sized atypical lymphocytes including focal atypical mitosis. Some cells showed characteristic peripheral rimming of individual adipocytes. However, prominent lymphoid atypia, fat necrosis, vasculitis, vascular thrombosis, erythrophagocytosis, mucin deposition, and the striking dominance of CD8+ lymphocytes seen in SPTCL were not detected. The lesion resolved spontaneously in 6 months and there was no recurrence over a 24-month follow-up period.

Cyclosporine A as a Primary Treatment for Panniculitis-like T Cell Lymphoma: A Case with a Long-Term Remission / Journal of the Korean Cancer Association, 대한암학회지

Subcutaneous panniculitis-like T cell lymphoma (SPTL) is a distinctive cutaneous lymphoma characterized by an infiltration of subcutaneous tissue by neoplastic T cells, similar to panniculitis. It is well-established that patients who are diagnosed with SPTL usually respond poorly to chemotherapy, showing fatal outcome. As a first line treatment for SPTL, anthracycline-based chemotherapy was most frequently used. For the treatment of SPTL, the efficacy of cyclosporine A has been recently reported in relapsed SPTL after anthracycline-based chemotherapy. However, it is still not clear whether cyclosporine A can be used as a first-line treatment against SPTL. Here, we report a case of SPTL, which achieved complete remission for nine years after first-line cyclosporine A therapy. This study suggests that cyclosporine A can induce a complete long-term remission as a first-line treatment.

Subcutaneous Panniculitis-Like T-Cell Lymphoma of the Breast

Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare subtype of cutaneous lymphoma. There have been a few case reports describing the radiologic imaging findings of SPTCL. We report a case of SPTCL, rarely presented with a breast mass. Here, we review her clinical history and radiologic (mammography and ultrasound) findings.

Cyclosporine A treatment for relapsed subcutaneous panniculitis-like T-cell lymphoma: a case with long-term follow-up / 대한혈액학회지

Subcutaneous panniculitis-like T-cell lymphoma (SPTL) is a distinctive lymphoma characterized by an infiltration of subcutaneous tissue by neoplastic cytotoxic T cells. There was no distinction between TCR alpha/beta phenotype and TCR gamma/delta phenotype, and anthracycline-based chemotherapy was usually used for both. Here, we report a patient with recurrent SPTL who achieved a second long-term complete remission by repeated cyclosporine A (CsA) treatment. From 2000 to 2001, the patient received anthracycline-based combination chemotherapy. However, the treatment did not induce long-term remission. In 2002, he received cyclosporine treatment for about 6 months. This resulted in a 5-year remission that ended in relapse in 2008. He received CsA treatment once again and attained a second long-term remission. This case suggests that re-treatment with CsA can be a good option for relapsed SPTL cases and can result in long-term remission.

Subcutaneous Panniculitis-Like T-cell Lymphoma: A Clinical and Pathologic Study of 14 Korean Patients

BACKGROUND: Subcutaneous panniculitis-like T-cell lymphoma (SPTL) is a distinctive skin lymphoma characterized by neoplastic T-cell infiltration of the subcutaneous tissue, mimicking panniculitis. OBJECTIVE: To describe the clinical and pathologic features of SPTL in Korean patients. METHODS: Fourteen SPTL patients evaluated over 15 years were retrospectively reviewed. RESULTS: The mean patient age was 35 years (range: 7~73 years), with male predominance (2.5:1). Most patients presented with either nodules or plaques, occurring most commonly on the trunk, with two patients (14%) having hemophagocytic syndrome. Histopathologically, all patients showed infiltrates of small-to-medium pleomorphic cells mimicking panniculitis, with some also showing rimming, bean-bag cells, and fat necrosis. Most patients were positive for CD3 (14/14), CD8 (12/13), TIA-1 (9/9) and betaf1 (5/5), but were negative for CD4 (11/12), CD20 (8/8), CD56 (14/14) and Epstein-Barr virus (8/8). Ten patients (71%) received chemotherapy and 2 (14%) died due to the disease, with an average survival time of 4 months. Survival analysis did not reveal any significant prognostic factors. CONCLUSION: This is the first series of patients with SPTL in Korea. Due to its indolent clinical course and relatively high survival rate, SPTL should be differentiated from cutaneous gammadelta T-cell lymphoma.

Acute onset of proptosis secondary to subcutaneous panniculitis-like T cell lymphoma / 国际眼科杂志(Guoji Yanke Zazhi)

To report a rare case of acute onset of proptosis secondary to subcutaneous panniculitis-like T cell lymphoma (SPTCL). METHODS: A case report. RESULTS: A 27-year-old Malay man presented with history of acute onset of proptosis in the left eye for 2 weeks. It was associated with history of prolonged high fever. He also developed multiple erythematous swelling on his body and both thighs during this period. On examination, visual acuity in both eyes was 6/6. The left eye was proptosed and chemotic.The extraocular move-ment was limited in all directions.The cornea and the anterior segment were normal. Fundoscopy showed normal optic disc and retina.The examination of the right eye was unremarkable.His body temperature was 40.0℃.There was presence of multiple erythematous subcu-taneous lesions over the body mainly in the left axillary, right hypochondriac, both thighs and the suprapubic area.The inguinal lympnodes were also palpable.MRI of orbit and brain revealed generalized soft tissue swelling in the left extraconal and retro-orbital space suggesting of inflammatory reaction.The cavernous sinus and brain were normal.Skin biopsy from the erythematous lesion revealed as SPTCL.He was referred to the haematologist and was started on chemotherapy-CHOP regime. The patient responded well to the chemotherapy and the proptosis reduced. CONCLUSION: Proptosis secondary to SPTCL is very rare. This is a variant of a peripheral T cell lymphoma characterised by multiple subcutaneous nodules presented with proptosis and fever.

A Case of Atypical Lymphocytic Lobular Panniculitis / 대한피부과학회지

We report here on a 63-year-old woman who presented with recurrent bruise-like infiltrative plaques on the left leg without systemic symptoms or laboratory abnormalities. The histopathologic findings showed an infiltration of panniculus by small to medium-sized atypical lymphocytes. But the prominent lymphoid atypia, fat necrosis, vascular thrombosis, erythrophagocytosis and striking dominance of CD8+ lymphocytes seen in subcutaneous panniculitis-like T-cell lymphoma (SPTCL) were not detected. The diagnosis of atypical lymphocytic lobular panniculitis (ALLP) was made based on these histopathologic and clinical features. The lesions responded well to systemic steroid treatment. But the CD4/CD8 ratio in the later lesion was more decreased than that of the initial lesion, and the patient is under clinical follow up.

Clinical analysis of subcutaneous panniculitis-like T cell lymphoma misdiagnosed as rheumatic diseases:8 cases report / 中华内科杂志

Objective To investigate the characteristics of subcutaneous panniculitis-like T cell lymphoma ( SPTCL) in order to facilitate prompt identification and proper treatment of this rare and heterogeneous disease entity. Methods For 8 patients who had been misdiagnosed as rheumatic diseases but eventually confirmed as SPTCL though pathology and immunohistochemistry, a retrospective chart review was made with regard to their clinical symptoms, laboratory test results, pathological features, responses to therapy as well as outcomes. Results These 8 patients with a male to female ratio of 1: 1 were initially misdiagnosed as a variety of rheumatic diseases such as erythema nodosa, nodular panniculitis, systemic vasculitis,etc. The period from the onset of symptoms to the confirmation of diagnosis as SPTCL was 28. 6 months on average (range, 4-84 months). All the cases presented with multiple subcutaneous nodules, plaques or tumors which involved various anatomic sites including the head and neck, the trunk, and the extremities. Fever was the most frequently accompanying symptom (7/8) , followed by lymphadenopathy (4/8) , hepatomegaly (3/8), splenomegaly (3/8). Hemophagocytic phenomenon was seen in 3 cases.A total of 22 times of biopsy involving multiple anatomic sites were performed on these 8 cases with 2. 75 times on average (range, 1-5 times). All cases demonstrated a dense lymphoid infiltrate with significant cellular heteromorphism located in the subcutaneous tissue. CD_3 was positive in the majority of the cases.Immunostaining for γδTCR was positive in one case. The anti-rheumatic therapy including steroids and immunosuppressants administered before the identification of SPTCL attained minimal therapeutic effect. In contrast, 6 cases gained partial response after chemotherapy except that the other 2 cases died of fatal pulmonary infiltration and subsequent infection. Conclusions SPTCL is a rare and heterogeneous entity which is unseldomly misdiagnosed as rheumatic disease. The anti-rheumatic therapy including steroids and immunosuppressants can attain minimal therapeutic effect. Early identification by means of histology and immunohistochemistry as well as immunostaining for PCR is critical for proper treatment.

F-18 FDG PET/CT Findings of Subcutaneous Panniculitis - Like T- Cell Lymphoma : A Case Report / 핵의학분자영상

F-18 FDG PET is a metabolic imaging modality that is efficacious in staging and assessment of treatment response for variety of lymphomas. We report usefulness of F-18 FDG PET/CT in evaluating severity of the disease and response to therapy in a patient with subcutaneous panniculitis- like T-cell lymphoma (SPTCL). Here we describe a case of SPTCL in 24-year-old man who had wide spread firm and tender nodular lesions with increased F-18 FDG uptake. After chemotherapy follow up F-18 FDG PET/CT image shows disseminated malignancy and then the patient died with hemophagocytic syndrome. This report suggests that F-18 FDG PET/CT may be useful in determining disease activity at the time of initial diagnosis, after treatment, and evaluating a suspected outcome of SPTCL.

Subcutaneous Panniculitis-like T cell Lymphoma with Hemophagocytic Syndrome / 대한피부과학회지

Subcutaneous pnniculitis-like T cell lymphoma (SPTCL) is a rare subtype of cutaneous T cell lymphoma which clinically and histopathologically mimics benign panniculitis. It presents as tender erythematous, subcutaneous nodules or plaques on the extremities and trunk, and displays systemic symptoms such as weight loss, fever, chills, fatigue or myaligia. The association of hemophagocytic syndrome (HPS) portends a poor prognosis. We report a case of SPTCL that occurred in a 20-month-old girl. She presented with multiple erythematous subcutaneous nodules on the face, right thigh, buttocks and trunk, plus fever and vomiting. Histopathological examination of the subcutaneous nodules revealed a dense infiltration of atypical T-lymphoid cells in the subcutaneous fat layer, with rimming of fat cells, numerous karyorrhetic bodies and histiocytes, phagocytosed apoptotic cells and Bean bag cells In spite of combination chemotherapy, she died of disseminated intravascular coagulation due to HPS.

A Case of Subcutaneous Panniculitis-like T-Cell Lymphoma / 대한피부과학회지

Subcutaneous panniculitis-like T-cell lymphoma(SPTL) is a rare cytotoxic T-cell lymphoma of the skin, which preferentially infiltrates subcutaneous tissue. In the World Health Organization(WHO) classification of T-cell and natural killer cell lymphoma it is listed as an example of extranodal lymphoma. We report a case of SPTL in a 31-year-old man, who presented with fever, night sweats, fatigue and three, tender, erythematous, indurated, subcutaneous plaques on the trunk. Typical histologic features of panniculitic infiltration with rimming of fat cells and sparing of the dermis and epidermis were seen. The immunophenotypic studies showed a cytotoxic T-cell phenotype by CD3+, CD8+, TIA-1+, CD20- and CD56-. In situ hybridization for Epstein-Barr virus-encoded RNA was negative.

A Case of Subcutaneous Panniculitis-like T Cell Lymphoma in Childhood

Subcutaneous pannicultis-like T cell lymphoma is a rare cutaneous T cell lymphoma. It presents with multiple subcutaneous nodules or plaques involving the extremities or trunk, and with constitutional symptoms that include fever, malaise, fatigue, myalgia, chills and weight loss. Histologically, the lesions of this disease are reminiscent of panniculitis and are composed of a mixture of small and large atypical lymphoid cells infiltrating between adipocytes. The optimal treatment for this disease is undefined and prognosis of this disease is poor, even when treated with multiagent chemotherapy regimens considered optimal for agressive lymphoma of other types. Poor prognosis factors include clinical features such as anemia, leukocytopenia, hepatosplenomegaly, lymphadenopathy and coagulopathy, which are suggestive of hemophagocytosis. Much of the mortality of this disease is due not to disseminated lymphoma with organ failure, but rather to complications of the cytopenias associated with the hemophagocytic syndrome. We report a case of subcutaneous panniculitis-like T cell lymphoma in a 12 year-old boy who presented with initial complaints of fever and multiple subcutaneous nodules, and briefly review the related literature.

Two Cases of Subcutaneous Panniculitis-like T-cell Lymphoma / 대한피부과학회지

Subcutaneous panniculitis-like T-cell lymphoma(SPTCL) is a rare subtype of cutaneous T-cell lymphoma and needs to be differentiated from benign causes of panniculitis and other cutaneous T-cell lymphomas involving subcutis, especially nasal type NK/T-cell lymphoma(NKTL) involving the panniculus. We report herein two cases of SPTCL which was distinguished from nasal type NKTL by the following findings; CD8+, CD56-, Ebstein Barr virus RNA- and clonal T cell receptor gene arrangement.