Background. Cutaneous CD8+ epidermotropic cytotoxic T-cell lymphoma is a recently described rare primary cutaneous lymphoma exhibiting aggressive clinical behavior. Only about twenty cases have been described in the l...Background. Cutaneous CD8+ epidermotropic cytotoxic T-cell lymphoma is a recently described rare primary cutaneous lymphoma exhibiting aggressive clinical behavior. Only about twenty cases have been described in the literature. Below we report a case involving unusual association of cutaneous vasculitis and lymphoproliferation. Case report. A 42-year-old senegalese man was hospitalized for cutaneous nodular lesions, which rapidly spread and became necrotic and ulcerated, he had recent weight loss with fever and multiple enlarged lymph nodes. Cutaneous histological analysis showed epidermotropic dermal infiltrate comprising medium and large cd8+ cytotoxic t-cells of unusual angiocentricity with cutaneous vasculitis and fibrinoid necrosis, the patient died 4 months after initiation of treatment with multi-agent chemotherapy. Discussion. This patient presented the characteristics of primary Cutaneous CD8+ epidermotropic cytotoxic T-cell lymphoma described by Berti. The clinical findings in most cases consist of nodular and ulcerative cutaneous lesions. Histologically, the cutaneous infiltrate is composed of pleomorphic lymphocytes with marked and constant epidermotropism. Immunohistochemistry shows lymphocytes expressing a CD8+ phenotype and cytotoxic proteins, which probably accounts for the local and systemic aggressiveness of the disease, as well as the angio destructive nature of the infiltrate and the necrotic lesions.展开更多
Adult T-cell leukemia/lymphoma (ATLL) is a malignant proliferation of mature helper T lymphocytes, and is caused by human T-lymphotropic virus type I (HTLV-I); an HTLV-I infection endemic in the Caribbean, south-weste...Adult T-cell leukemia/lymphoma (ATLL) is a malignant proliferation of mature helper T lymphocytes, and is caused by human T-lymphotropic virus type I (HTLV-I); an HTLV-I infection endemic in the Caribbean, south-western Japan, South America and Africa. Seroepidemiological studies suggest that it is also endemic in Brazil. Although carriers of HTLV-I show polyclonal integration of virus in T lymphocytes, only patients with ATLL of various subtypes show monoclonal integration of HTLV-I in tumor cells. Cutaneous T-cell lymphomas (CTCL) are a group of primary cutaneous lymphoproliferative diseases unknown etiology. The two most common presentations of CTCL are mycosis fungoides (MF) and Sézary syndrome (SS). However, both CTCL categories can easily resemble ATLL. Therefore, in HTLV-I endemic areas, differentiation between ATLL and CTCL must be performed, as they have different prognoses and treatment approaches.展开更多
Adult T-cell leukaemia/lymphoma is alymphopro life rativedis ord eraetiologi call yassociated with human T-cell lymphotropic virus type I infection. A cutan eous lesion often develops in the disease, and in rare cases...Adult T-cell leukaemia/lymphoma is alymphopro life rativedis ord eraetiologi call yassociated with human T-cell lymphotropic virus type I infection. A cutan eous lesion often develops in the disease, and in rare cases, is even the only m anifestation. Here we report a rare case of ‘cutaneous’adult T-cell leukaemia /lymphoma with neither atypical cells in the peripheral blood nor lymph node inv olvement. All nodular lesions were completely eliminated after local electron be am irradiation (20 Gy/nodule in total). To evaluate whether or not there were re sidual lymphoma cells in the skin, we performed PCR to detect clonal T cell rece ptor γgene rearrangements. The sample from the nodule before irradiation showed evidence of a rearranged band, which was not detected at the same site after tr eatment nor in any peripheral blood. The findings suggest that this procedure is useful for the evaluation of therapeutic effects and the early detection of lym phoma recurrence.展开更多
Background: Erythroderma is a diffuse, inflammatory skin reaction that, in rare instances, is associated with hematologic maligancies such as cutaneous T-cell lymphoma (erythrodermic mycosis fungoides) or T-cell leuke...Background: Erythroderma is a diffuse, inflammatory skin reaction that, in rare instances, is associated with hematologic maligancies such as cutaneous T-cell lymphoma (erythrodermic mycosis fungoides) or T-cell leukemia (S′ ezary syndrome or adult T-cell leukemia/lymphoma). Observations: We screened 30 patients with erythroderma (20 patients with erythroderma of known etiology and 10 patients with idiopathic erythroderma) for the presence of circulatingmonoclonal T-lymphocyte populations using T-cell receptor (TCR)-γ genespecific polymerase chain reaction and automated capillary DNA electrophoresis. Moreover, the phenotypic analysis of peripheral blood CD4+ lymphocytes was performed using the following surface markers: CD3, CD7, CD8, CD25, CD26, CD27,CD28, CD29,CD30, CD45RO,CD45RA,CD56, CD134,HLA-DR, TCRα β , TCRγ δ , and cutaneous lymphocyte antigen (CLA). In 5 patients with idiopathic erythroderma we detected T-cell clones in peripheral blood (in 1 case, associated with the presence of the same clone in the skin) and a 2-fold increase in the proportion of CD3+ CD4 + CD7-CD26-cells. Cell depletion studies indicated that the monoclonal T cells were present within the CD4 + CD7- cell population. Clinically, all patients had chronic, recalcitrant erythroderma but none developed any hematological malignancy during their lifetimes or fulfilled the criteria for cutaneous lymphoma or S′ ezary syndrome. Conclusions: A proportion of patients with chronic erythroderma present with the monoclonal expansion of CD4 + CD7-CD26- lymphocytes in their blood. This condition represents a probably benign T-cell dyscrasia, or one of very low malignancy. Alongside monoclonal gammapathy of undetermined significance (MGUS) and monoclonal (B-cell) lymphocytosis of undetermined significance (MLUS), we propose using monoclonal T-cell dyscrasia of undetermined significance (MTUS) to underline a conceptual similarity between this disorder and the more common types of lymphocytic dyscrasia.展开更多
文摘Background. Cutaneous CD8+ epidermotropic cytotoxic T-cell lymphoma is a recently described rare primary cutaneous lymphoma exhibiting aggressive clinical behavior. Only about twenty cases have been described in the literature. Below we report a case involving unusual association of cutaneous vasculitis and lymphoproliferation. Case report. A 42-year-old senegalese man was hospitalized for cutaneous nodular lesions, which rapidly spread and became necrotic and ulcerated, he had recent weight loss with fever and multiple enlarged lymph nodes. Cutaneous histological analysis showed epidermotropic dermal infiltrate comprising medium and large cd8+ cytotoxic t-cells of unusual angiocentricity with cutaneous vasculitis and fibrinoid necrosis, the patient died 4 months after initiation of treatment with multi-agent chemotherapy. Discussion. This patient presented the characteristics of primary Cutaneous CD8+ epidermotropic cytotoxic T-cell lymphoma described by Berti. The clinical findings in most cases consist of nodular and ulcerative cutaneous lesions. Histologically, the cutaneous infiltrate is composed of pleomorphic lymphocytes with marked and constant epidermotropism. Immunohistochemistry shows lymphocytes expressing a CD8+ phenotype and cytotoxic proteins, which probably accounts for the local and systemic aggressiveness of the disease, as well as the angio destructive nature of the infiltrate and the necrotic lesions.
文摘Adult T-cell leukemia/lymphoma (ATLL) is a malignant proliferation of mature helper T lymphocytes, and is caused by human T-lymphotropic virus type I (HTLV-I); an HTLV-I infection endemic in the Caribbean, south-western Japan, South America and Africa. Seroepidemiological studies suggest that it is also endemic in Brazil. Although carriers of HTLV-I show polyclonal integration of virus in T lymphocytes, only patients with ATLL of various subtypes show monoclonal integration of HTLV-I in tumor cells. Cutaneous T-cell lymphomas (CTCL) are a group of primary cutaneous lymphoproliferative diseases unknown etiology. The two most common presentations of CTCL are mycosis fungoides (MF) and Sézary syndrome (SS). However, both CTCL categories can easily resemble ATLL. Therefore, in HTLV-I endemic areas, differentiation between ATLL and CTCL must be performed, as they have different prognoses and treatment approaches.
文摘Adult T-cell leukaemia/lymphoma is alymphopro life rativedis ord eraetiologi call yassociated with human T-cell lymphotropic virus type I infection. A cutan eous lesion often develops in the disease, and in rare cases, is even the only m anifestation. Here we report a rare case of ‘cutaneous’adult T-cell leukaemia /lymphoma with neither atypical cells in the peripheral blood nor lymph node inv olvement. All nodular lesions were completely eliminated after local electron be am irradiation (20 Gy/nodule in total). To evaluate whether or not there were re sidual lymphoma cells in the skin, we performed PCR to detect clonal T cell rece ptor γgene rearrangements. The sample from the nodule before irradiation showed evidence of a rearranged band, which was not detected at the same site after tr eatment nor in any peripheral blood. The findings suggest that this procedure is useful for the evaluation of therapeutic effects and the early detection of lym phoma recurrence.
文摘Background: Erythroderma is a diffuse, inflammatory skin reaction that, in rare instances, is associated with hematologic maligancies such as cutaneous T-cell lymphoma (erythrodermic mycosis fungoides) or T-cell leukemia (S′ ezary syndrome or adult T-cell leukemia/lymphoma). Observations: We screened 30 patients with erythroderma (20 patients with erythroderma of known etiology and 10 patients with idiopathic erythroderma) for the presence of circulatingmonoclonal T-lymphocyte populations using T-cell receptor (TCR)-γ genespecific polymerase chain reaction and automated capillary DNA electrophoresis. Moreover, the phenotypic analysis of peripheral blood CD4+ lymphocytes was performed using the following surface markers: CD3, CD7, CD8, CD25, CD26, CD27,CD28, CD29,CD30, CD45RO,CD45RA,CD56, CD134,HLA-DR, TCRα β , TCRγ δ , and cutaneous lymphocyte antigen (CLA). In 5 patients with idiopathic erythroderma we detected T-cell clones in peripheral blood (in 1 case, associated with the presence of the same clone in the skin) and a 2-fold increase in the proportion of CD3+ CD4 + CD7-CD26-cells. Cell depletion studies indicated that the monoclonal T cells were present within the CD4 + CD7- cell population. Clinically, all patients had chronic, recalcitrant erythroderma but none developed any hematological malignancy during their lifetimes or fulfilled the criteria for cutaneous lymphoma or S′ ezary syndrome. Conclusions: A proportion of patients with chronic erythroderma present with the monoclonal expansion of CD4 + CD7-CD26- lymphocytes in their blood. This condition represents a probably benign T-cell dyscrasia, or one of very low malignancy. Alongside monoclonal gammapathy of undetermined significance (MGUS) and monoclonal (B-cell) lymphocytosis of undetermined significance (MLUS), we propose using monoclonal T-cell dyscrasia of undetermined significance (MTUS) to underline a conceptual similarity between this disorder and the more common types of lymphocytic dyscrasia.
