A 32-year-old man presented with a 5-year history of cutaneous nodules on his head and a diffuse, lichenified eruption. Histopathologic examination showed an atypical lymphocytic infiltrate. Immunophenotyping studies determined that the lymphocyte population to be CD4-positive, with partial loss of CD3 and CD7, and immunogenotyping studies showed a clonal rearrangement of the T-cell receptor. A positron-emission tomography scan showed increased uptake in cervical, axillary, and inguinal lymph nodes. A diagnosis of peripheral T-cell lymphoma was made, and the patient is undergoing chemotherapy. 相似文献
An 88-year-old man from the Dominican Republic with a history of gastric adenocarcinoma was admitted with one month of fatigue, anorexia, weight loss, and abdominal pain. The dermatology department was consulted to evaluate an asymptomatic, shiny, firm, red nodule on the lower left chest, with an expanding rim of erythema. Skin biopsies were performed from the nodule and surrounding rim of erythema, which were both diagnostic of peripheral T-cell lymphoma (PTCL). This case is a unique example of PTCL with erysipelaslike spread. 相似文献
An unusual case of subcutaneous panniculitis-like T-cell lymphoma is presented involving multiple organ systems, which eventually culminated in rapid demise from the haemophagocytic syndrome, after an initial protracted course. A 44-year-old man presented in April 2001 with bronchiolitis obliterans organising pneumonia that initially responded well to corticosteroids. However, the condition relapsed on attempted prednisone withdrawal in January 2002 and the patient was noted to have developed truncal subcutaneous nodules. Initial skin biopsy revealed lobular panniculitis, with negative microbiological culture. In July 2002, mononeuritis multiplex was diagnosed after the patient presented with paresthesiae and was treated with pulse cyclophosphamide therapy. By November 2002 there was ulceration of the subcutaneous nodules. Repeat skin biopsy revealed subcutaneous panniculitis-like T-cell lymphoma. The clinical manifestations were supportive of an unifying diagnosis of malignancy involving pulmonary, cutaneous and nervous systems. Combination chemotherapy with fludarabine, mitoxantrone and dexamethasone was commenced. However, the patient deteriorated, with fevers, weight loss, pancytopenia and laboratory features consistent with the haemophagocytic syndrome. Despite maximal supportive therapy the patient succumbed to his disease. 相似文献
The concept of subcutaneous T-cell lymphoma defines a reduced group of primary cutaneous lymphomas characterized morphologically by a prominent or exclusive subcutaneous tissue involvement. Subcutaneous panniculitic-like T-cell lymphoma is a rare subtype of primary cutaneous T-cell lymphoma clinically mimicking panniculitis. The clinical course is usually protracted with recurrent cutaneous lesions but rarely with early extracutaneous dissemination. The clinical, histopathologic, immunophenotypic, and evolutive features of this heterogeneous and rare group of primary cutaneous lymphomas are reviewed. 相似文献
We reported on a case of subcutaneous panniculitis-like T-cell lymphoma (SPTCL) with multiple erythematous nodular lesions on the extremities, trunk and face. Histological examination of an excised lesion revealed a dense infiltrate of markedly atypical T-lymphoid cells expressing the CD8+ phenotype located in the subcutaneous tissue with histiocyte-phagocytizing apoptotic cells. The 'bean-bag' histiocytic cells, the characteristic finding of SPTCL, are considered to be products of haemophagocytosis. In our case the 'bean-bag' cells were produced by phagocytosis of apoptotic bodies, as confirmed by electron microscopy. It is suspected that 'bean-bag' cells are related not to haemophagocytosis but to phagocytosis of apoptotic cells in the CD8+ T-cell type of SPTCL. 相似文献
A 22‐year‐old man had a subcutaneous nodule on the left thigh for 3 months. Physical examination showed an ulcerative nodule on the left thigh ( Fig. 1 ). He suffered from a fever for 1 week. Laboratory tests revealed a white blood cell count of 7180/mm3, with 22.4% neutrophils, 68.5% lymphocytes, 8.4% monocytes, 0.3% eosinophils, and 0.4% basophils. Hemoglobin was 9.0 g/dL, hematocrit was 28.1%, and the platelet count was 202 × 109/L. Analysis of serum showed the following abnormal results: aspartate aminotransferase, 102 IU/L (normal, 13–37); alanine aminotransferase, 77 IU/L (normal, 7–43); alkaline phosphatase, 1296 (normal, 70–270); LDH, 1105 (normal, 120–520). Results of other laboratory tests including serum electrolytes, blood and tissue culture, and viral markers for hepatitis were negative or normal. Ultrasonography and computed tomography of abdomen and pelvis showed hepatosplenomegaly with ascites. Whole body bone scan showed an increased long bone activity of the left leg. Bone marrow aspiration was negative for neoplastic cells. Histologic examination from the thigh nodule revealed subcutaneous lymphoid infiltrates with involvement of the deep dermis. Examination at high power magnification showed small, medium, and large‐sized, pleomorphic lymphoid cells with hyperchromatic nuclei, nuclear debris, and phagocytic macrophages. Fat necrosis, partial rimming of fat spaces and clustering around blood vessels by pleomorphic lymphoid cells were also seen ( Fig. 2 ). The immunohistochemical studies on paraffin sections revealed that most of the infiltrated lymphoid cells positively stained for CD56 ( Fig. 3 ), granzyme B, TIA‐1, cytoplasmic CD3, and CD45RO, whereas CD4, CD8, CD20, and CD30 showed no immunoreactivity. Clonality of the infiltrate was analyzed by polymerase chain reaction (PCR). Nested PCR was performed using FR3A and VLJH as primers for IgH gene and Vγ – Jγ for T‐cell receptor γ chain gene. Polymerase chain reaction analysis showed a distinct band for TCR gene, confirming T‐cell monoclonality of infiltrating lymphocytes. EBV‐encoded small nuclear RNA was evident in lesional tissue by in situ hybridization. He was treated with two cycles of chemotherapy with CHOP‐BV (Cytoxan 735 mg, adriamycin 45 mg, vincristine 1.45 mg, prednisolone 75 mg, bleomycin 15 mg, VP‐16 150 mg). He died 5 days after chemotherapy. Figure 1 Open in figure viewer PowerPoint An ulcerative nodule on the left thigh 相似文献
BACKGROUND: Cutaneous lymphomas have many morphologic forms and clinical features. Lymphoma presents rarely with a constellation of nodules, panniculitis, and localized lipoatrophy. The histopathologic similarities of lymphoma and connective tissue disease panniculitis may create a diagnostic challenge. METHODS: We retrospectively reviewed the case of a 47-year-old man who presented 15 years earlier with recurrent fevers, fatigue, tender subcutaneous nodules, and facial, trunk, and extremity lipoatrophy. RESULTS: Initial biopsy of a cutaneous nodule showed lymphohistiocytic panniculitis without atypical inflammatory cells. Serologic tests showed negative connective tissue serologies, yet the initial clinical impression was most consistent with lupus panniculitis. Initially, the patient was treated with oral prednisone; later, steroid-sparing agents were used with modest improvement evidenced by resolution of his systemic symptoms and stabilization of the cutaneous findings. A decade later, more pronounced facial lipoatrophy, new facial nodules, and posterior thigh pain developed. Laboratory testing showed leukopenia, elevated liver and muscle enzymes, hypertriglyceridemia, and a low level of high-density lipoprotein. Biopsy of a new chin nodule indicated peripheral T-cell lymphoma, whereas an evaluation for systemic malignant involvement was negative. The patient was started on chemotherapy, which resulted in stabilization of the lipoatrophy and decreasing size and frequency of the cutaneous nodules, but the posterior thigh pain persisted. CONCLUSIONS: We report a rare case of lymphoma presenting as nodules and profound lipoatrophy, which exemplifies the complexity of lymphomas. Profound lipoatrophy and panniculitis may be an unusual and diagnostically challenging presentation of cutaneous lymphoma. 相似文献
Subcutaneous panniculitis-like T-cell lymphomas (SPTLs) represent a rare, difficult-to-diagnose, and poorly characterized subtype of cutaneous T-cell lymphomas (CTCLs) affecting younger people more than the other CTCL forms. We performed a thorough clinical, immunohistological, and molecular analysis of nine Finnish SPTL patients. Specifically, we performed single-cell comparative genomic hybridization (CGH) from laser microdissected, morphologically malignant SPTL cells, as well as loss of heterozygosity (LOH) and fluorescence in situ hybridization (FISH) analysis for the NAV3 (neuron navigator 3) gene. CGH revealed large numbers of DNA copy number changes, the most common of which were losses of chromosomes 1pter, 2pter, 10qter, 11qter, 12qter, 16, 19, 20, and 22 and gains of chromosomes 2q and 4q. Some of the DNA copy number aberrations in SPTL, such as loss of 10q, 17p, and chromosome 19, overlap with those characteristic of common forms of CTCL (mycosis fungoides (MF) and Sezary syndrome (SS)), whereas 5q and 13q gains characterize SPTL. Allelic NAV3 aberrations (LOH or deletion by FISH), previously found in MF and SS, were identified in 44% of the SPTL samples. This study demonstrates that SPTL is also moleculocytogenetically a uniform entity of CTCL and supports the current World Health Organization-European Organization for Research and Treatment of Cancer (WHO-EORTC) classification defining SPTL as a subgroup of its own. 相似文献