CICLOSPORIN AS UPFRONT THERAPY IN SUBCUTANEOUS PANNICULITIS-LIKE T-CELL LYMPHOMA WITH AGGRESSIVE FEATURES - Two Case Reports and Literature Review

Q: Why is ciclosporin considered a front-line option in SPTCL?

The letter notes that ciclosporin has emerged as a potential front-line agent, and it may suppress T-cell activation and proliferation through pathways related to calcineurin inhibition and downstream signaling.

Q: What treatment approach was used in the first case with aggressive disseminated disease and HLH concern?

The first patient was started on ciclosporin with high-dose prednisolone; fevers resolved, inflammatory markers normalized, and PET imaging showed near-complete resolution within four months.

Q: How was relapse managed in the first case and what was the outcome?

At 18 months, relapse was confirmed by biopsy; ciclosporin trough levels were subtherapeutic, so the dose was increased and high-dose prednisolone was reintroduced. Remission was again achieved with continued ciclosporin and tapering of prednisolone, without further relapses and without significant adverse effects.

Sep 30, 2026

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Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is described as a rare cutaneous T-cell lymphoma (<1% of cases) that usually runs an indolent course but may relapse, become refractory, or be complicated by hemophagocytic lymphohistiocytosis (HLH). Letters to the editor present two cases in which upfront ciclosporin combined with corticosteroids achieved rapid reduction of FDG-avid lesions, with therapy adjusted for relapse in one patient. The report summarizes literature suggesting higher complete response rates with ciclosporin-based regimens than chemotherapy, while addressing proposed immunologic mechanisms and treatment limitations due to limited validated studies.

LanguageEnglish

CategoryHealthcare

FormatPDF

Pages4

Reading Time10 min

AccessFree to download

To the Editor

Clinical background of SPTCL and treatment rationale

Case 1: aggressive disseminated disease with HLH features

Case 2: localized disease without HLH

Summary of ciclosporin-based literature and rationale

Letters to the Editor CICLOSPORIN AS UPFRONT THERAPY IN SUBCUTANEOUS PANNICULITIS-LIKE T-CELL LYMPHOMA WITH AGGRESSIVE FEATURES AND IN UNCOMPLICATED DISEASE: TWO CASE REPORTS AND LITERATURE REVIEW Brandon K.J. Tan1a, Kiat Hoe Ong1,2,3,4,5, Yong Howe Ho6, Lip Leong Chong1,2,3, Christian A. Gallardo1,2,3 1 Department of Haematology, Tan Tock Seng Hospital, Singapore, Singapore, 2 Lee Kong Chian School of Medicine, Nanyang Technological University, Singapore, 3 Yong Loo Lin School of Medicine, National University of Singapore, Singapore, 4 Department of Laboratory Medicine, Tan Tock Seng Hospital, Singapore, 5 Molecular Diagnostic Laboratory, Tan Tock Seng Hospital, Singapore, 6 Department of Pathology, Tan Tock Seng Hospital, Singapore, Singapore Keywords: Subcutaneous panniculitis-like T-cell lymphoma (SPTCL), Ciclosporin, T-cell lymphoma [https://doi.org/10.46989/001c.154639](https://doi.org/10.46989/001c.154639) | Clinical Hematology International
Vol. 8, Issue 1, 2026 | | | --- | --- | | TO THE EDITOR,
Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare subtype of cutaneous T-cell lymphoma, accounting for less than 1% of cases. It generally exhibits an indolent course but can be relapsing, refractory, or even complicated by hemophagocytic lymphohistiocytosis (HLH) . It usually manifests as subcutaneous nodules, plaques, or ulcers, predominantly on the limbs and trunk. Histologically, it demonstrates a lobular panniculitis pattern with adipocyte rimming by atypical CD8+ cytotoxic T-lymphocytes.
Corticosteroids are often effective, but durable responses are uncommon with monotherapy.1 Chemotherapy and stem cell transplantation (SCT) are generally considered for refractory or HLH-complicated disease.2 Ciclosporin, though historically used in relapsed or refractory cases, has emerged as a potential front-line agent. Herein, we present two cases of SPTCL, one with aggressive disseminated disease and HLH features, successfully managed with upfront ciclosporin and corticosteroids, underscoring its efficacy and favourable safety profile.
Our first case illustrates a 37-year-old woman with systemic lupus erythematosus and prior class III lupus nephritis. She presented with a 3-month history of painful, indurated subcutaneous nodules on her limbs, thorax, and abdomen. Positron Emission Tomography (PET) scan revealed [18F]Fluorodeoxyglucose (FDG)-avid subcutaneous lesions, generalized lymphadenopathy, and retroperitoneal soft tissue thickening (Figure 1) . Biopsies from subcutaneous tissue and iliopsoas muscle demonstrated cytotoxic CD8+ T-cell infiltrates with adipocyte rimming, high Ki-67
index (70–80%), and monoclonal TCRB/TCRG gene rearrangements, confirming SPTCL with disseminated disease. | HLH was a concern due to persistent fever, markedly elevated ferritin and hemophagocytosis on bone marrow examination. Our patient was not keen to receive chemotherapy or SCT. She was initiated on ciclosporin 100 mg BD and high-dose prednisolone. Fevers subsided and inflammatory markers normalized over days. A PET scan at 4 months (Figure 1) demonstrated near-complete resolution of FDG-avid lesions. Prednisolone was tapered, and ciclosporin continued with regular disease surveillance.
At 18 months, she developed disease recurrence with axillary nodules and retroperitoneal FDG-avid lesions. Biopsy confirmed relapse. Ciclosporin troughs were detected tobe subtherapeutic (60–80 ug/L) and dose was increased to 125mg BD with high-dose prednisolone initiated. She again achieved remission with no further relapses while prednisolone was tapered. There were no significant adverse effects from ciclosporin use.
Our second case was a 54-year-old woman presenting with a three-month history of non-tender right axillary lumps. PET scan showed localized FDG-avid axillary disease, and biopsy confirmed SPTCL. There were no HLH features. She was treated with ciclosporin 50 mg BD and prednisolone 0.5 mg/kg/day, achi

FAQs

Why is ciclosporin considered a front-line option in SPTCL?

The letter notes that ciclosporin has emerged as a potential front-line agent, and it may suppress T-cell activation and proliferation through pathways related to calcineurin inhibition and downstream signaling.

What treatment approach was used in the first case with aggressive disseminated disease and HLH concern?

The first patient was started on ciclosporin with high-dose prednisolone; fevers resolved, inflammatory markers normalized, and PET imaging showed near-complete resolution within four months.

How was relapse managed in the first case and what was the outcome?

At 18 months, relapse was confirmed by biopsy; ciclosporin trough levels were subtherapeutic, so the dose was increased and high-dose prednisolone was reintroduced. Remission was again achieved with continued ciclosporin and tapering of prednisolone, without further relapses and without significant adverse effects.

Sep 30, 2026

Uploaded by Rainbow Cat