Subcutaneous panniculitis-like T-cell lymphoma: Clinical features, therapeutic approach, and outcome in a case series of 16 patients.

BACKGROUND: Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare primary cutaneous lymphoma of mature cytotoxic T cells. Initially, patients with SPTCL were treated with doxorubicin-based polychemotherapy. OBJECTIVE: To analyze clinical, biologic, immunophenotypical, molecular, imaging, treatment, and outcome data reflecting the current state of knowledge. METHODS: A retrospective multicenter study of 16 patients with SPTCL that was diagnosed between 1996 and 2016. RESULTS: The female-to-male ratio was 1.7. The median age at diagnosis was 46.5 years. Patients presented with multiple nodular or plaque-like lesions preferentially affecting the legs and/or trunk. Histopathology typically showed a lobular panniculitis with individual adipocytes surrounded by atypical lymphocytes, usually with a CD3+, CD4-, CD8+, CD56-, TIA1 cytotoxic granule associated RNA binding protein 1-positive phenotype and high proliferation rate. SPTCL was associated with autoimmune diseases in 25% of patients, and with the development of hemophagocytic syndrome in 18% of patients. Oral steroids alone or in combination with low-dose methotrexate or cyclosporine A were the most common initial treatment, achieving a complete response in 85% of the treated patients. The median follow-up time was 14 months. The 5-year disease-specific survival rate was 85.7%. LIMITATIONS: This was a retrospective study. CONCLUSIONS: SPTCL has an excellent prognosis. Immunosuppressive agents can be considered for first-line treatment.

Subcutaneous Panniculitis-like T-cell Lymphoma: Immunosuppressive Drugs Induce Better Response than Polychemotherapy.

Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare condition usually considered to have a favourable prognosis. However, it is not known whether polychemotherapy or immunosuppressive-based therapy is the best approach for treating SPTCL. Using data collected between 2000 and 2012 in France, we analysed clinical, biological and pathological data of 27 patients with SPTCL. Medical history revealed that 40% of patients had been previously diagnosed with an autoimmune disorder and 22% with inflammatory panniculitis. Haemophagocytic syndrome was present in 37% of cases. Autoantibodies were positive in 65% of cases. Complete remission (CR) was reached in 74% of cases. Immunosuppressive drug treatment was given in 69.5% of patients (group 1) and polychemotherapy in 30.5% (group 2). CR was 81.2% and 28.5% (p?=?0.025), respectively. Progression rate was 6.2% and 42.8% (p?=?0.067), respectively. This study suggests that immunosuppressive drugs should be considered as the first-line treatment for SPTCL.

Radiological and clinical features of subcutaneous panniculitis-like T-cell lymphoma.

OBJECTIVE: To retrospectively analyze computed tomography (CT) and clinical findings of patients with subcutaneous panniculitis-like T-cell lymphoma (SPTCL). MATERIALS AND METHODS: The CT findings were retrospectively assessed in 8 patients (6 men, 2 women; age range, 23-60 years; mean age, 39.8 years) with pathologically proven SPTCL. The tumor location, number, distribution, size, and morphological characteristics on CT were evaluated, as were maximum standardized uptake values on positron emission tomography. We also evaluated patient symptoms, laboratory findings, immunophenotype, involvement of bone marrow, treatment, and outcomes. RESULTS: All 8 patients had multiple (range, 4 to numerous) soft tissue lesions involving subcutaneous fat tissue of the chest, abdominal wall, back, and buttock. Lesions varied in size (range, 0.5-10 cm) and showed reticular (n = 1), nodular (n = 4), and diffuse infiltrative (n = 3) patterns. Three patients had lesions involving the entire thickness of fat tissue, 3 had partial lesions, and 2 had variable lesions. Skin thickening was mild in 4 patients, moderate in 2, and severe in 2; fascia thickening was mild in 3 patients, moderate in one, severe in one, and variable in 2. In 7 patients, lesions were accompanied by engorged supplying vessels. At onset, maximum standardized uptake values varied from 1.2 to 4.7, decreasing to 0.0 to 4.2. Five patients had αß type and one had γδ type. Clinical outcome varied: two remain alive with disease, two had no disease, and one died. CONCLUSIONS: The SPTCL is characterized by multiple, nodular, or diffuse soft tissue lesions involving subcutaneous fat tissue, accompanied by engorged vessels and skin and fascial thickening. Patients vary in presentation and in treatment outcomes.

Hematopoietic stem cell transplantation for subcutaneous panniculitis-like T-cell lymphoma: single center experience in an Asian population.

Subcutaneous panniculitis-like T-cell lymphoma (SPTL) is a rare form of cytotoxic T-cell lymphoma. It is believed that SPTL in patients without hemophagocytic syndrome (HPS) follows an indolent course; in contrast, SPTL in patients with HPS has been associated with unfavorable survival. To provide more clinical data on SPTL in Asian populations and to identify optimal therapeutic strategies for SPTL, we assessed the clinicopathological features and long-term follow-up data of 10 Taiwanese SPTL patients diagnosed at a single center. Our study demonstrates a group of patients with high incidence of HPS (50%), rather aggressive courses, and early progression. A total of eight patients underwent hematopoietic stem cell transplant (HSCT), including one autologous HSCT and seven allogeneic HSCT. Seven of eight patients receiving HSCT achieved durable remission and maintained in remission for over 30 months (range 30-132 months). There was no difference in 3-year survival of patients with HPS (80%) compared with patients without HPS (80%). Of long-term survivors in the HPS group, three of four received HSCT (autologous HSCT, n = 1; allogeneic HSCT, n = 2). Our study indicated that HSCT is a curative option for eligible SPTL patients with HPS.

Survival of Subcutaneous Panniculitis-Like T-Cell Lymphoma and Peripheral T-Cell Lymphoma Not Otherwise Specified: A Propensity-Matched Analysis of the Surveillance, Epidemiology, and End Results Database.

BACKGROUND: Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare entity with no previous population-based study. MATERIALS AND METHODS: We used the Surveillance, Epidemiology, and End Results 18 database to identify adult patients with SPTCL and peripheral T-cell lymphoma not otherwise specified (PTCL NOS) diagnosed between 1973 and 2011. The actuarial survival of SPTCL was compared with a propensity-matched cohort of PTCL NOS. Multivariate analysis was conducted using weighted Cox proportional hazard regression model. RESULTS: Patients with SPTCL (n = 118), compared with PTCL NOS (n = 3296), were more likely to be younger (median age of 47 vs. 62 years; P < .01), women (67% vs. 40%, P < .01), and diagnosed with stage I/II disease (46% vs. 36%; P = .01). The 5-year actuarial, relative, and cause-specific survival for SPTCL was 40%, 57%, and 64%, respectively. After propensity-matching, the 5-year overall survival (OS) of SPTCL was better than that of PTCL NOS (57% vs. 40%; P < .01). In a multivariate analysis, mortality was significantly lower among SPTCL versus PTCL NOS (hazard ratio, 0.54; 95% confidence interval, 0.39-0.75; P < .01). Among patients with SPTCL, advanced age (P < .01) and diagnosis before the year 2008 (P = .02) were predictors of worse OS. CONCLUSION: Our study provides characteristics and OS of a large cohort of SPTCL. Compared with PTCL NOS, SPTCL patients were more likely to be younger, female, and diagnosed at an early stage. The OS of SPTCL was better than PTCL NOS.

Subcutaneous panniculitis-like T-cell lymphoma: definition, classification, and prognostic factors: an EORTC Cutaneous Lymphoma Group Study of 83 cases.

In the WHO classification, subcutaneous panniculitis-like T-cell lymphoma (SPTL) is defined as a distinct type of T-cell lymphoma with an aggressive clinical behavior. Recent studies suggest that distinction should be made between SPTL with an alpha/beta T-cell phenotype (SPTL-AB) and SPTL with a gammadelta T-cell phenotype (SPTL-GD), but studies are limited. To better define their clinicopathologic features, immunophenotype, treatment, and survival, 63 SPTL-ABs and 20 SPTL-GDs were studied at a workshop of the EORTC Cutaneous Lymphoma Group. SPTL-ABs were generally confined to the subcutis, had a CD4-, CD8+, CD56-, betaF1+ phenotype, were uncommonly associated with a hemophagocytic syndrome (HPS; 17%), and had a favorable prognosis (5-year overall survival [OS]: 82%). SPTL-AB patients without HPS had a significantly better survival than patients with HPS (5-year OS: 91% vs 46%; P<.001). SPTL-GDs often showed (epi)dermal involvement and/or ulceration, a CD4-, CD8-, CD56+/-, betaF1- T-cell phenotype, and poor prognosis (5-year OS: 11%), irrespective of the presence of HPS or type of treatment. These results indicate that SPTL-AB and SPTL-GD are distinct entities, and justify that the term SPTL should further be used only for SPTL-AB. SPTL-ABs without associated HPS have an excellent prognosis, and multiagent chemotherapy as first choice of treatment should be questioned.

Fatal pancreatic panniculitis associated with acute pancreatitis: a case report.

Pancreatic panniculitis is a rare disease in which necrosis of fat in the panniculus and other distant foci occurs in the setting of pancreatic diseases; these diseases include acute and chronic pancreatitis, pancreatic carcinoma, pseudocyst, and other pancreatic diseases. This malady is manifested as tender erythematous nodules on the legs, buttock, or trunk. Histopathologically, it shows the pathognomonic findings of focal subcutaneous fat necrosis and ghost-like anucleated cells with a thick shadowy wall. We herein report a case of fatal pancreatic panniculitis that was associated with acute pancreatitis in a 50-yr-old man. He presented with a 3-week history of multiple tender skin nodules, abdominal pain and distension. Laboratory and radiologic findings revealed acute pancreatitis, and skin biopsy showed pancreatic panniculitis. Despite intensive medical care, he died of multi-organ failure 3 weeks after presentation.

Cytophagic histiocytic panniculitis is not always fatal.

Two patients with benign cytophagic histiocytic panniculitis are presented and compared with the one benign and ten fatal cases previously reported. Benign-appearing histiocytes which engulf blood cells, nuclear debris, and platelets are the most characteristic feature of the disease. This cytophagia tends to be massive in the subcutaneous tissue and involves extracutaneous organs only in the fatal cases. The course is chronic in the benign form. Patients with the fatal form tend to have fever, hepatosplenomegaly, serosal effusions, ecchymoses, peripheral adenopathy, and mucosal ulcers. Anemia, leukopenia, elevated liver enzyme levels, and coagulopathy are present in almost all and hypocalcemia in many patients with a fatal course. Cytophagic histiocytic panniculitis may represent a form of regional histiocytosis primarily involving subcutaneous tissue. It is midway in the spectrum of the cytophagic histiocytoses.