HistoryThis section has been translated automatically.
DefinitionThis section has been translated automatically.
Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare form of "T-cell non-Hodgkin's lymphoma" in which CD8+ cytotoxic T cells infiltrate adipose tissue and form inflammatory subcutaneous plaques or nodules.
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EtiopathogenesisThis section has been translated automatically.
In the familial forms of subcutaneous panniculitis-like T-cell lymphoma reported by Gayden et al (2018), autosomal recessive mutations in the HAVCR2 gene were causative.
ManifestationThis section has been translated automatically.
Both children and adults can be affected, with an average age at diagnosis of 36 years and a predominance of the female gender.
LocalizationThis section has been translated automatically.
especially the lower extremity.
Clinical featuresThis section has been translated automatically.
Onset with a clinical picture "reminiscent of an infection" with a pronounced general feeling of illness, fever, weight loss.
The skin findings are atypical for cutaneous T-cell lymphoma. There are usually asymptomatic or slightly painful, subcutaneous, red (inflammatory), plate-like or nodular indurations measuring 5.0-20.0 cm in diameter, so that the first clinical impression is more reminiscent of erythema nodosum or atypically localized erythema induratum. Long-term relapsing courses with spontaneous remissions are the rule. Ulcerations are the exception (in about 6% of patients).
If a hemophagocytosis syndrome is detected (in about 17% of patients), the prognostically unfavorable subcutaneous form of systemic gamma/delta T-cell lymphoma must be considered. In this case, sepsis-like clinical pictures may develop.
HistologyThis section has been translated automatically.
Dense, nodular infiltrate in the subcutis, with very pleomorphic lymphoid cells of different sizes, which often accumulate in a ring around lipocytes (so-called rimming). General view as in lobular panniculitis. Angiocentric growth pattern with fat tissue necroses. Granulomatous secondary reaction, erythrophagocytosis in 30% of cases.
Clonal rearragenment of TZR genes can be detected in most cases.
Immunocytogenetic. Expression of the alpha/beta T cell receptor (TZR). Expression of the beta/gamma chain of the T cell receptor indicates subcutaneous infiltration of the systemic form of gamma/delta T cell lymphoma (GD-TZL) with poor prognosis.
Differential diagnosisThis section has been translated automatically.
- Panniculitis: Mostly subacute, mostly painful nodular subcutaneous infiltrates.
- Profound circumscribed scleroderma: Slow, non painful, firm indurations. Histology is diagnostic.
- Subcutaneous form of gamma/delta T-cell lymphoma: expression of the gamma/delta chain of TZR. Important DD(!) because systemic gamma/delta T-cell lymphoma has a significantly worse prognosis.
- Lupus panniculitis: infiltration by small lymphocytes, no cell atypia.
- Extranodal NK/T-cell lymphoma: expression of CD56; association with EBV.
TherapyThis section has been translated automatically.
In 16 patients with SPTCL, Gayden et al (2018) found that immunosuppressive and chemotherapeutic treatment was effective, but some patients achieved complete remission only after hematopoietic stem cell transplantation. One patient treated with anakinra, an IL1 inhibitor, experienced disappearance of symptoms and sustained improvement in general condition.
In individual foci, radiation therapy with fast elecrodes should be considered.
Progression/forecastThis section has been translated automatically.
Mostly slow progressive course. 5-year survival rate of 80%.
Case report(s)This section has been translated automatically.
The 48-year-old patient reported a changeable clinical picture on both legs for 4-5 years with little painful, plate-like indurations, which regressed leaving deeply retracted, also extensive "scarring" of skin and subcutis. For 1 year analogous changes also occurred in the lower abdomen.
The patient's general condition was unremarkable.
Laboratory: blood and differential blood counts were unremarkable. Liver enzymes, lipase, amylase, alpha-1-antitrypsin, kidney values o.B. No pathological immune parameters. Borrelia serology unremarkable.
Imaging procedures: o.B.
Histopathological and molecular biological findings: Dense nodular infiltrate in the subcutis, with very pleomorphic lymphoid cells of different sizes, often accumulating in a ring around lipocytes (so-called rimming). Overview picture as in lobular panniculitis. Immunohistology: Tumor cells express T cell markers: CD3, CD8, CD43, CD45RO, not expressed is CD4! Clonal rearragenment of TZR genes was detectable.
Ther: Initiation of steroidal monotherapy (prednisolone 1mg/kg/KG i.v.). Complete regression of indurations after 8 weeks. Maintenance dose 5mg/p.o. After 6 months local recurrence. Now radiotherapy (GD 36 Gy, fractionation 5 x /week 2,0Gy). Under this complete regression and 2 years free of recurrence.
Gayden et al (2018) reported 16 patients, including 2 sibling pairs, with SPTCL. Twelve patients were of East Asian origin, including Polynesian, three were of Caucasian and European origin, and one was of North African descent. Most patients presented in the first or second decade of life (usually late childhood) with various symptoms of inflammatory disease, including fever, facial swelling, fatigue, painful flanks, pancytopenia or anemia, and subcutaneous nodules suggestive of panniculitis. However, several patients presented as adults. Skin biopsies confirmed panniculitis with cytotoxic CD8+ T-cell infiltrations with adipocyte rims and necrosis, consistent with a diagnosis of SPTCL. Molecular studies of the skin lesions, when performed, showed T-cell receptor-beta chain rearrangements that were monoclonal in most cases.
Bone marrow examination revealed hemophagocytic lymphohistiocytosis (HLH) in 14 of 16 patients and often showed adipocyte infiltration by CD8+ T cells.
Laboratory findings in 1 patient (P4) showed signs of inflammation with elevated serum levels of inflammatory cytokines during active disease. Patient-derived T cells and macrophages produced elevated levels of inflammatory cytokines in vitro, including TNFA, IL2, and IL1B. Other variable laboratory findings included abnormal liver enzymes, autoantibodies, and features associated with hemophagocytic lymphohistiocytosis, such as elevated triglycerides, hyperferritinemia, and hypofibrinogenemia.
Polprasert et al (2019) reported 11 unrelated patients of East Asian descent with SPTCL. The median age at diagnosis was 32 years (range, 5 to 59). Multiple SPTCL lesions were commonly observed on the trunk, eyelids, extremities, and buccal mucosa. Pathologic analysis revealed atypical CD8+ cytotoxic T cells infiltrating the subcutaneous adipose tissue and lining the adipocytes. 2 of the 11 patients developed hemophagocytic lymphohistiocytosis. Most patients had a monoclonal T-cell receptor (TCR) gene rearrangement. All patients responded to immunosuppressive therapy.
LiteratureThis section has been translated automatically.
- Gayden T et al (2019) Germline HAVCR2 mutations altering TIM-3 characterize subcutaneous panniculitis-like T cell lymphomas with hemophagocytic lymphohistiocytic syndrome. Nature Genet 50: 1650-1657.
- Go RS et al (2003) Subcutaneous panniculitis-like T-cell lymphoma: complete remission with fludarabine. Ann Hematol 82: 247-250.
- Gonzalez CL et al (1991) T-cell lymphoma involving subcutaneous tissue. A clinicopathologic entity
- commonly associated with hemophagocytic syndrome.
- Am J Surg Pathol 15:17-27.
- Guizzardi M et al (2003) Cytotoxic gamma/delta subcutaneous panniculitis-like T-cell lymphoma: report of a case with pulmonary involvement unresponsive to therapy. J Eur Acad Dermatol Venereol 17: 219-222.
- Hoque SR et al (2003) Subcutaneous panniculitis-like T-cell lymphoma: a clinicopathological, immunophenotypic and molecular analysis of six patients. Br J Dermatol 148: 516-525.
- Jaffe ES et al (2003) Classification of cytotoxic T-cell and natural killer cell lymphomas. Semin Hematol 40: 175-184
- Kempf W, Cerroni L (2016) Cutaneous lymphomas. In: Cerroni L et al. Histopathology of the skin. Springer-Verlag Berlin Heidelberg New-York pp 915-917.
- Kim YC et al (2002) Extranodal NK/T-cell lymphoma with extensive subcutaneous involvement, mimicking subcutaneous panniculitis-like T cell lymphoma. Int J Dermatol 41: 919-921
- Kumar S et al (1989) Subcutaneous panniculitis T-cell lymphoma is a tumor of cytotoxic T-lymphocytes. Hum Pathol 29: 397-403
- Marzano AV (2000) Cytophagic histiocytic panniculitis and subcutaneous panniculitis-like T-cell lymphoma: report of 7 cases. Arch Dermatol 136: 889-896
- Mukai HY et al (2003) Successful treatment of a patient with subcutaneous panniculitis-like T-cell lymphoma with high-dose chemotherapy and total body irradiation. Eur J Haematol 70: 413-416.
- Polprasert C et al (2019) Frequent germline mutations of HAVCR2 in sporadic subcutaneous panniculitis-like T-cell lymphoma. Blood Adv 3: 588-595.
- Reimer P et al (2003) Subcutaneous panniculitis-like T-cell lymphoma during pregnancy with successful autologous stem cell transplantation. Ann Hematol 82: 305-309
- Wagner G et al (2016) Subcutaneous panniculitis-like T-cell lymphoma. Dermatol 67: 399-402
- Yamashita Y et al (1999) A case of natural killer/T cell lymphoma of the subcutis resembling subcutaneous panniculitis-like T cell lymphoma. Pathol Int 49: 241-246
Incoming links (11)Granzyme; HAVCR2 Gene; Panniculitis histiocytic, cytophagic; Panniculitis-like cutaneous t-cell lymphoma; Panniculitis-like t-cell lymphoma of the skin; PID autoinflammatory diseases ; Primary immunodeficiencies and skin; PTCL; Subcutaneous panniculitis-like t-cell lymphoma; Subcutaneous panniculitis-like t-cell lymphoma; ... Show all
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