“…Some patients, however, have lesions limited to skin without any systemic involvement. A cutaneous variant of ATLL has been proposed to term this subgroup [60,61], and the cutaneous lesions are sometimes indistinguishable from those of mycosis fungoides and Sézary syndrome clinically and histologically. ATLL cells express chemokine receptors and adhesion molecules, including CCR1, CCR4, CCR7, CCR8, and CD62L (L-selection).…”
Section: Advances In the Pathogenesis Of Htlv-1 Positive Disordersmentioning
“…Some patients, however, have lesions limited to skin without any systemic involvement. A cutaneous variant of ATLL has been proposed to term this subgroup [60,61], and the cutaneous lesions are sometimes indistinguishable from those of mycosis fungoides and Sézary syndrome clinically and histologically. ATLL cells express chemokine receptors and adhesion molecules, including CCR1, CCR4, CCR7, CCR8, and CD62L (L-selection).…”
Section: Advances In the Pathogenesis Of Htlv-1 Positive Disordersmentioning
“…Of note, there is a cutaneous type of ATLL with lesions that persist without the development of extracutaneous involvement. 11 Cutaneous ATLL may in fact have a better prognosis than 'classical' ATLL. 9 Fig.…”
Follicular mucinosis is currently recognized as a histopathological finding characterized by the accumulation of mucin within follicular epithelium and is commonly associated with follicular mycosis fungoides (MF). We report the finding of follicular mucinosis in a cutaneous nodule of human T-lymphotropic virus type 1 (HTLV-1) associated adult T-cell leukemia/lymphoma (ATLL). The patient was a 69-year-old female of Caribbean descent with a history of ATLL who presented with erythematous nodules on the chest and abdomen. Histopathologic examination showed a pan-dermal infiltrate of medium-to-large sized atypical lymphocytes extending into follicular epithelium where they associated with large mucin deposits. Immunohistochemical stains showed that the atypical lymphocytes were positive for CD3, CD4 and CD25 and negative for CD30. Cutaneous lesions of ATLL, which often present histopathologically as an epidermotropic lymphoma with Pautrier-type collections, are often difficult to distinguish from MF. Until recently, lymphoma-associated follicular mucinosis seemed specific to MF and Sézary syndrome (SS), being reported only once in a lesion of ATLL. We report a second case of ATLL-associated follicular mucinosis to increase awareness of this possible association, and briefly review the literature of follicular mucinosis-associated hematologic malignancies, ultimately cautioning against the interpretation of all cutaneous lymphoma-related follicular mucinosis as MF/SS.
“…Skin involvement is reported variably in 43-72% of patients. There is a cutaneous type of ATLL with lesions that persist without the development of extracutaneous involvement [16,17]. The skin lesions of ATLL are often clinically non-speci ic, and include papules, nodules, plaques, tumors, ulcerative lesions and erythroderma.…”
Primary cutaneous lymphomas (PCLs) are the second most common group of extranodal non-Hodgkin lymphomas (NHL) with an estimated annual incidence of 1/100.000. Interferons (IFNs) are used in mono or combination therapy for cutaneous lymphomas especially for cutaneous T-cell lymphomas (CTCL) for years. IFN-α is the most widely-used type for cutaneous lymphomas. IFN-α has been shown to be a highly active agent in CTCL with response rates ranging from 40% to 80%. In this review, the current information about PCLs and IFNs treatment is summarized.
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