A Case with an Indolent Course of Subcutaneous Panniculitis-Like T-Cell Lymphoma Demonstrating Epstein-Barr Virus Positivity and Simulating Dermatitis Artefacta

Soylu, Seçil; Gül, Ülker; Kılıç, Arzu; Heper, Aylın Okçu; Kuzu, Işınsu; Minareci, Burcu Gönen

doi:10.2165/11311060-000000000-00000

Cited by 9 publications

(3 citation statements)

References 20 publications

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“…The exact etiology and pathogenesis of SPTCL are still unclear. In the past, some scholars thought that the Epstein‒Barr virus infection might be associated with its occurrence [ 14 ], but an increasing number of studies have shown that the disease and the Epstein‒Barr virus do not have an obvious correlation [ 15 ]. The EB virus infection was not detected by EBV-DNA and EBER in situ hybridization in our 18 children.…”

Section: Discussionmentioning

confidence: 99%

A retrospective study of 18 children with subcutaneous panniculitis-like T-cell lymphoma: multidrug combination chemotherapy or immunomodulatory therapy?

Duan

Gao

Zhou

et al. 2022

Orphanet J Rare Dis

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Background Subcutaneous panniculitis T-cell lymphoma (SPTCL) is a rare, cytotoxic T-cell lymphoma with which some patients have accompanying hemophagocytic syndrome (HPS). There is currently no standard treatment regimen. In the past, the most commonly used treatment was multidrug chemotherapy. In contrast, numerous case reports or small series suggest that immunosuppressive drugs could also be effective for some patients. Since this NHL subtype is extremely rare in children and adolescents, to improve the understanding of this disease and standardize its rational treatment, we retrospectively summarized the treatment regimens of 18 pathologically diagnosed children with SPTCL to compare the clinical efficacy of multidrug chemotherapy and immunomodulatory therapy. Results The median age of onset was 11.1 years. Painless subcutaneous nodules or skin patchy lesions were found in all patients, most commonly involving the lower extremities and/or trunk. Before January 1, 2019, the treatment was mainly chemotherapy, and 10 patients were initially treated with chemotherapy, among whom was one patient who progressed during initial treatment, was voluntarily discharged and was subsequently lost to follow-up, one patient who died of disease progression, and the remaining 8 patients who all achieved sustained remission, with a complete remission (CR) rate of 80% (8/10). Corticosteroids combined with cyclosporine A or ruxolitinib were the most common initial immunosuppressive agents at our center after January 1, 2019 and had a CR rate of 71.4% (5/7). In addition, 1 patient achieved partial remission (PR) during follow-up, and one had autologous hematopoietic stem cell transplantation (AHSCT) after 4 months of drug withdrawal. There were 7 patients (38.9%, one case in chemotherapy group and six cases in immunotherapy group) with HPS and 4/5 screened patients (80%) with positive HAVCR2 gene mutations. The median follow-up was 17 months. Conclusion The prognosis of SPTCL is relatively good. Previous multi-drug and long-term chemotherapy treatment has clear efficacy, and recent immunomodulatory therapy as pre-chemotherapy therapy can also benefit patients.

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Section: Discussionmentioning

confidence: 99%

A retrospective study of 18 children with subcutaneous panniculitis-like T-cell lymphoma: multidrug combination chemotherapy or immunomodulatory therapy?

Duan

Gao

Zhou

et al. 2022

Orphanet J Rare Dis

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“…SPTCL tumor cells are more aggressive, which results in rapid disease progression, and the natural course of the disease is dangerous. At present, there is no standard treatment for SPTCL ( 10 ), but it is sensitive to systemic combination chemotherapy such as cyclophosphamide + doxorubicin, vincristine + prednisone regimens (CHOP regimens) ( 13 , 28 - 30 ), and topical radiotherapy is sensitive and effective ( 16 , 31 , 32 ). Despite the diagnosis of SPTCL in this case, the patient could not receive chemotherapy due to severe liver failure and secondary infection, which eventually led to her unfortunate death in February following the onset of the disease.…”

Section: Discussionmentioning

confidence: 99%

A case report and literature review: subcutaneous panniculitis-like T-cell lymphoma with liver failure as the main manifestation

He¹,

Wang²,

Feng³

et al. 2022

Ann Transl Med

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Background: Subcutaneous lipofuscinosis-like T-cell lymphoma (SPTCL) is a rare cutaneous lymphoma that often presents as recurrent subcutaneous nodules and may progress to hemophagocytic syndrome with fever and hepatic impairment in some patients. However, no cases of progression to hepatic failure have been reported. Here, we present a case of an adult diagnosed with fever and liver failure as the main manifestation, which was eventually confirmed as SPTCL by skin biopsy, but the patient eventually died due to disease progression.Case Description: This study retrospectively reports a rare case of SPTCL in a 34-year-old female patient who was admitted with scleral jaundice for 1 month and fever for 20 days. Examination revealed multiple small subcutaneous nodules on the skin of the chest and tibial surface and an enlarged liver and spleen. Laboratory tests revealed hepatic impairment, and common causes of liver failure (viral infection, fatty liver, immune liver damage, etc.) were excluded. We continued to refine the positron emission tomography-computed tomography (PET-CT) examination, which revealed multiple flocculent and nodular hyperdense shadows with increased metabolism in the subcutaneous fat interstices. And then, we performed a skin biopsy and the final pathological diagnosis was SPTCL, but the patient died 1 month after diagnosis due to poor treatment outcome because the disease progressed too rapidly. With this case report, we hope to improve clinicians' understanding of liver injury caused by SPTCL. A review of the literature revealed that this is the first case report in the literature of SPTCL leading to severe liver failure.Conclusions: For patients presenting with fever and liver injury, primary liver disease cannot simply be assumed, as this presentation may be a manifestation of some extra-hepatic diseases, including SPTCL. For this condition, early detection and early diagnosis may improve the prognosis of patients.

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“…Angiocentricity and angioinvasion with ischemic necrosis, as well as epidermotropism, are common histopathological findings [ 2 ]. Additionally, various microscopic patterns, including lobular panniculitis, granulomatous dermatitis, and interface dermatitis patterns have been reported in the cutaneous ENK/T lymphoma [ 3 , 4 , 5 , 6 , 7 , 8 , 9 ].…”

Section: Introductionmentioning

confidence: 99%

Cutaneous Involvement of Extranodal NK/T Cell Lymphoma, Nasal Type, a Clinical and Histopathological Mimicker of Various Skin Diseases

et al. 2022

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Background: Extranodal NK/T cell lymphoma, nasal type (ENK/T) with cutaneous involvement has various histopathological findings and diverse clinical manifestations. Methods: A retrospective study of cutaneous involvement of ENK/T lymphoma between 2006 and 2018 was conducted. Results: Twenty-two cases were eligible for this study. Twelve cases could be proven as secondary cutaneous involvement by ENK/T lymphoma, while the remaining could not be confirmed as primary cutaneous ENK/T lymphoma. The histopathological patterns included dermal and subcutaneous nodular infiltration pattern in 11/22 cases (50%), lobular panniculitis pattern in 6/22 cases (27.3%), interface dermatitis pattern in 4/22 cases (18.2%), and granulomatous dermatitis pattern in 1/22 case (4.5%). The median follow-up was 18.3 months. Overall, the one-year and five-year survival rates were 31.3% and 13.3%, respectively. Conclusions: A variety of histopathological patterns of cutaneous involvement by ENK/T lymphoma should be differentiated from other cutaneous lymphomas, dermatitis, and infection. When atypical medium or large-sized lymphoid cells are encountered within skin lesions, pathologists should realize these lesions can be ENK/T lymphoma, especially in cases with coexisting tumor necrosis or angioinvasion. A complete evaluation of the upper aerodigestive tract is mandatory to identify the occult primary site of ENK/T lymphoma before establishing primary cutaneous ENK/T lymphoma.

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A Case with an Indolent Course of Subcutaneous Panniculitis-Like T-Cell Lymphoma Demonstrating Epstein-Barr Virus Positivity and Simulating Dermatitis Artefacta

Cited by 9 publications

References 20 publications

A retrospective study of 18 children with subcutaneous panniculitis-like T-cell lymphoma: multidrug combination chemotherapy or immunomodulatory therapy?

A retrospective study of 18 children with subcutaneous panniculitis-like T-cell lymphoma: multidrug combination chemotherapy or immunomodulatory therapy?

A case report and literature review: subcutaneous panniculitis-like T-cell lymphoma with liver failure as the main manifestation

Cutaneous Involvement of Extranodal NK/T Cell Lymphoma, Nasal Type, a Clinical and Histopathological Mimicker of Various Skin Diseases

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