RESUMO
Primary cutaneous gamma-delta T-cell lymphoma (CGD-TCL) is a rare cutaneous lymphoma. Panniculitis-like T-cell lymphoma (SPTCL) has a better prognosis than CGD-TCL. SPTCL is sometimes associated with autoimmune disease. A 64-year-old Japanese female with a history of dermatomyositis presented with subcutaneous nodules on the upper extremities and exacerbated dermatomyositis. A skin biopsy showed lobular panniculitis, a vacuolar interface change, and a dermal mucin deposit. Fat cells rimmed by neoplastic cells, fat necrosis, and karyorrhexis were observed. The atypical lymphoid cells showed CD3+, CD4-, CD8+, granzyme B+, CD20-, and CD56-. Polymerase chain reaction analysis demonstrated a T-cell receptor rearrangement. The patient was initially diagnosed with SPTCL, so the dose of prednisone was raised from 7.5 to 50 mg daily (1 mg/kg). After one month, erythematous nodules regressed, and muscle symptoms improved. Subsequently, prednisone was tapered, and cyclosporin A was added. After one year, the patient remained symptom-free and continued taking 7.5 mg prednisone and 100 mg cyclosporin A daily. Afterward, we immunostained skin samples with antibodies against TCR-ß and δ and found positive TCR-δ and negative TCR-ß. Therefore, we corrected the diagnosis to CGD-TCL, although the clinical course and the presence of dermatomyositis were reminiscent of SPTCL.
RESUMO
BACKGROUND: Subcutaneous panniculitis-like T-cell lymphoma (SPTL) as strictly defined by World Health Organization-European Organization for Research and Treatment of Cancer classification is a rare cytotoxic α/ß T-cell lymphoma, characterized by primary involvement of subcutaneous tissue mimicking panniculitis. OBJECTIVES: To describe the clinicopathologic, immunophenotypic, and molecular features of SPTL. METHODS: A 10-year retrospective study of 18 patients diagnosed with SPTL was thoroughly reviewed according to clinicopathology, immunophenotype, and T-cell receptor (TCR) gene rearrangement. RESULTS: Of the 18 patients, 16 patients were definitely diagnosed with SPTL. The median age was 26 years (ranged 14-53 years) with female predominance. Most patients presented with prolonged fever and subcutaneous nodules and/or plaques, usually located on lower extremities. 37.5% of patients had hemophagocytic syndrome. The main histopathology was lobular panniculitis with rimming of atypical lymphocytes highlighted by CD3+, CD8+, Beta-F1+, granzyme B+, and Ki-67 (50%-90%). Monoclonal TCR gene rearrangement was found in 50% of patients and upper extremities involvement indicated a poor prognosis. CONCLUSION: The correlation between clinicopathologic and immunophenotypic study is the most helpful method to give a precise diagnosis of SPTL. Rimming of CD8+ atypical lymphocytes highlighted by high Ki-67 index is highly specific for the diagnosis of SPTL.
Assuntos
Rearranjo Gênico do Linfócito T , Linfoma de Células T , Proteínas de Neoplasias , Paniculite , Receptores de Antígenos de Linfócitos T alfa-beta , Neoplasias Cutâneas , Adolescente , Adulto , Feminino , Humanos , Linfoma de Células T/genética , Linfoma de Células T/metabolismo , Linfoma de Células T/patologia , Masculino , Pessoa de Meia-Idade , Proteínas de Neoplasias/genética , Proteínas de Neoplasias/metabolismo , Paniculite/genética , Paniculite/metabolismo , Paniculite/patologia , Receptores de Antígenos de Linfócitos T alfa-beta/genética , Receptores de Antígenos de Linfócitos T alfa-beta/metabolismo , Estudos Retrospectivos , Neoplasias Cutâneas/genética , Neoplasias Cutâneas/metabolismo , Neoplasias Cutâneas/patologia , Centros de Atenção TerciáriaRESUMO
Cutaneous γ/δ T-cell lymphoma (CGD-TCL) is a recent entity described in the newly revised World health organization-European organization for research and treatment of cancer classification of cutaneous lymphomas. Only a few cases have been reported, of which two pediatric cases. A 15 years old child with a 6 months history of polyadenopathy, cutaneous lesions, general edema and deterioration of general condition was hospitalized. Results from laboratory testing, cutaneous histopathology and immunohistochemistry showed a primary CGD-TCL. Staging was completed by a total body computed tomography. Therapy was planified with SMILE protocol. It is a highly aggressive tumor resistant to chemotherapy, immunotherapy, and radiation therapy. The GDTCL is characterized by a worse prognosis with a median survival of 15 months. Early diagnosis is essential and aggressive therapy is necessary.
Assuntos
Linfoma Cutâneo de Células T/diagnóstico , Neoplasias Cutâneas/diagnóstico , Adolescente , Evolução Fatal , Humanos , Linfoma Cutâneo de Células T/imunologia , Masculino , Receptores de Antígenos de Linfócitos T gama-delta , Neoplasias Cutâneas/imunologiaRESUMO
In the WHO-EORTC (World Health Organization-European Organization for Research and Treatment of Cancer) classification, the primary cutaneous B-cell lymphomas were divided into 4 groups: primary cutaneous marginal zone B-cell lymphoma, primary cutaneous follicle center cell lymphoma, primary cutaneous diffuse large B-cell lymphoma, other, and primary cutaneous diffuse large B-cell lymphoma, leg type (PCLBCL LT). PCLBCL LT is known to have more aggressive clinical behavior and a worse prognosis than the other groups of primary cutaneous B-cell lymphomas. We report here on a case of PCLBCL LT in a 55-year-old male who presented witha multiple erythematous nodules on the right leg. After chemotherapy, the skin lesions had almost cleared, but afterward the tumor was revealed to have metastasized to the brain during 1 year.
