Successful treatment of a rare subcutaneous panniculitis‐like T‐cell lymphoma: An unusual case report and literature review

Abstract

Dear Editor, SPTCL is a CD8 cytotoxic T-cell lymphoma (SPTCL) that is primarily localized in the subcutaneous tissue. This rare lymphoma accounts for fewer than 1% of all peripheral T-cell lymphomas (PTCL) and natural killer/T-cell lymphomas (NKTCL) (Vose, Armitage, & Weisenburger, 2008) and fewer than 1% of all cutaneous T-cell lymphomas (Tomasini & Berti, 2013). SPTCL was first described by Gonzalez et al. in 1991 (Gonzalez, Medeiros, Braziel, & Jaffe, 1991) and was previously divided into two subtypes: the α/β T-cell phenotype (SPTL-AB) and the γ/δ T-cell phenotype (SPTL-GD). Each subtype is distinct in T-cell phenotype, aggressiveness, and prognosis. SPTCL now refers to only the α/β T-cell receptor phenotype (Willemze et al., 2008), which has an excellent prognosis with a 5-year overall survival (OS) of 82%. Themedian age at SPTCL diagnosis was 36 years, and SPTCL affects twice as many women than men (Willemze et al., 2008). SPTCL patients present with subcutaneous nodular lesions mainly on the extremities and trunk and occasionally on the face with or without B symptoms such as fever, chills, night sweats, and weight loss (Willemze et al., 2008). No standard treatments are currently available, although various treatments, such as cyclophosphamide, epirubicin, vincristine and prednisolone (CHOP) or CHOP-like chemotherapy, radiotherapy, autologous stem cell transplantation (auto-SCT) or allogeneic hematopoietic stem cell transplantation (allo-SCT), and therapies using immunosuppressive drugs, retinoid (bexarotene), and histone deacetylase (HDAC) inhibitors, have been reported to achieve complete remission (Parveen & Thompson, 2009; Thakur, Chaudhari, Thakur, & Chaudhari, 2015; Willemze et al., 2008). In this report, we present a case of SPTCL in a 65-year-old Chinese woman who was diagnosed 4 years after symptoms began and achieved complete remission aftermulti-agent chemotherapy.Written informed consentwas obtained from the patient. A 64-year-old Chinese woman developed subcutaneous nodules in the right upper limb and back in 2013. The nodules spontaneously resolved to areas of lipoatrophy, and no other symptoms were noted. Four years later, the nodular lesions increased in number and became pruritus and painful in early 2017. Moreover, the subcutaneous lesions spread to the left upper limb and upper abdomen. Moreover, there was a mass in the right axilla. Therefore, the patientwas admitted to the Second Affiliated Hospital of Dalian Medical University. Local skin examination revealed multiple tough restricted subcutaneous nodules that varied in size (3.0–5.0 cm) and areas of lipoatrophy, indicated by circumscribed depression in the bilateral limbs, chest, back, and upper abdomen. The mass with a size of 2 cm × 2 cm could be touched in her axilla. The patient had no B symptoms, such as fever, chills, night sweats, andweight loss. Laboratory investigations showed that complete blood count, coagulation function, kidney function, liver function, lactate dehydrogenase and uric acid levels, and erythrocyte sedimentation rate were all normal. HIV and hepatitis test panels were negative. Computed tomography (CT) showed diffuse, infiltrative subcutaneous lesions. In addition, CT and ultrasound examinations showed that there were multiple enlarged lymph nodes with a maximal size of 2 × 2 cm in the axilla. Through the flow cytometric method, the CD2, CD3, CD4, CD8, CD45, CD57, CD16, CD56, CD34, CD5, CD7, CD3, TCR-αβ, TCR-γδ, and TdT markers were examined, which showed no abnormal T-cells were detected in the bone marrow. Skin biopsies of subcutaneous lesions on the right limb demonstrated typical adipotropism, and the fat lobules were infiltrated with atypical lymphoid cells of various sizes in which karyorrhexis and adiponecrosis were apparent. Immunohistochemical (IHC) analysis revealed that the neoplastic cells were positive for CD3, CD2, CD5, CD7, CD8, granzyme and T-cell intracellular antigen-1 (TIA-1) and negative for CD4, CD20, CD68, and Epstein–Barr Virus (Figure 1). Genetic testing was performed using polymerase chain reaction (PCR) at the ChinaMedical University, which indicated a clonal rearrangement of the T-cell receptor (TCR) beta gene (Table 1). Additionally, a lymph node biopsy from the right axilla was conducted and showed reactive lymphoid hyperplasia. Based on the clinical manifestation, laboratory and image results, particularly the high proliferation rate of lymphocytes rimming adipocytes, an α/β immunophenotype and a clonal rearrangement of the T-cell receptor beta gene, a diagnosis of SPTCLwasmade. The patient was started on the CHOP regimen (cyclophosphamide, epirubicin, vincristine, and prednisolone). As the patient could not endure the gastrointestinal adverse reactions, such as nausea, vomiting and poor appetite, after one course of CHOP, she received cyclophosphamide, vincristine and prednisolone for five courses. After six courses, her skin lesions resolved, and the lymph nodes of the bilateral axilla shrunk though they did not have lymphoma infiltration. Additionally, CT showed that the lesions were significantly diminished (Figure 2), and she did not have any adverse effects. A followup F-18 FDG PET/CT scan was performed and showed no abnormal metabolism. One year after the therapy, she remained in complete remission. The diagnosis of SPTCL is challenging and should be based on a combination of clinical, pathological, IHC, and molecular characteristics. Clinically, SPTCL always presents with subcutaneous nodular lesions or deeply seated plagues, which, in most cases, are widely distributed Received: 3 May 2018 Revised: 2 March 2019 Accepted: 14 March 2019