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    • tropisetron br Case report Skin examination revealed multipl

    2018-10-25


    Case report Skin examination revealed multiple asymptomatic, scaly, wrinkling, mottled reddish and hypopigmented patches over the left forearm, left flank, both buttocks and thighs (Figure 1A, B and C). About 15% of the total body surface area was involved. Additionally, one reddish papule located within reddish patches over the left flank was also noted (Figure 1B). Review of the patient\'s history over the previous 10 years indicated absence of fever, chills, or body weight loss. There were no palpable lymph nodes or hepatomegaly. Results of a complete blood count, liver function, and lactate dehydrogenase were within normal limits. The peripheral blood did not show atypical lymphocytes. Chest x-ray revealed no significant findings. Total body positron emission tomography indicated no abnormal uptake in visceral organs. Histopathology of a biopsy specimen from a reddish patch over the left flank demonstrated mild infiltration of atypical cerebriform lymphocytes at the dermal-epidermal junction with focal epidermotropism (Figure 2A and B). The tropisetron were positively stained with CD3, CD4, and CD8, but negative for CD20 and CD30 (Figure 2C–F). Another skin biopsy was taken from the reddish papule over the left flank, and the histopathological examination showed diffuse intradermal lymphocytic infiltration with large pleomorphic and hyperchromatic lymphocytes (Figure 3A–C). The cells were positively stained with CD3 and CD4, and focally with CD8 (Figure 3D and E), but negative for CD20. Of all infiltrating atypical lymphocytes, about 60% were large cells. They were strongly positive for CD30 (more than 75% of large cells) (Figure 3F), but negative for anaplastic lymphoma kinase and CD68. Polymerase chain reaction of skin biopsy from the reddish patch over the left flank showed clonality of the T-cell receptor γ gene rearrangement.
    Discussion Mycosis fungoides (MF) is the most common type of cutaneous T-cell lymphomas. Generally, the clinical manifestation shows three phases- patch, plaque and tumor stage, and typically runs an indolent course with a long evolution over years to decades. However, with disease progression, a small number of cases develop a morphologic change (large cell transformation), especially more common in the advanced stage of disease. The diagnosis of large cell transformation (LCT) in MF always relies on histologically defined criterion: the presence of large cells exceeding 25% of the total lymphoid infiltrate or forming microscopic nodules. Mycosis fungoides with LCT usually portends an aggressive course and shortened survival. Diamandidou et al reported shorter median survival for patients with LCT in MF (37 months), compared with nontransformed MF (163 months). Arulogun et al also indicated a lower 5-year survival rate for LCT group than nontransformed group of MF patients, 32.5% versus 53.6%. Although the molecular mechanism for LCT has not been fully demonstrated, molecular studies have confirmed that large cells are derived from the original T-cell clone in MF. Around 30% to 50% of MF patients with LCT showed CD30-positive cells. Although the histological criterion for the diagnosis of LCT in MF has been well defined, making a definite differential diagnosis is still sometimes challenging. MF with focal CD30-positive LCT should be differentiated from other CD30 positive lymphoproliferative disorders, including primary cutaneous anaplastic large cell lymphoma, lymphomatoid papulosis (LyP), and borderline cases. Primary cutaneous CD30+ lymphoproliferative disorders typically have an excellent prognosis with a disease specific 5-year survival of 85% to 100%. However, CD30 positive LCT in MF has worse outcome with a 5-year survival of 28.6% to 44.4%. Meanwhile, the clinical manifestations are obviously different between CD30 positive LCT in MF and primary cutaneous CD30 positive lymphoproliferative disorders. Coexisting MF and LyP is found in 9% of patients with LyP, and represents 3% of MF patients. Many studies show MF with concurrent LyP leads an indolent course and has a favorable prognosis. The clinical course of LyP with concurrent MF is also similar to that of LyP, and the skin lesions tend to regress spontaneously and then recur. By contrast, CD30+ LCT in MF rarely show spontaneous remission. Besides, LyP could arise from normal skin and/or previous MF lesions, while CD30+ LCT always develops upon pre-existent MF lesions. The coexistence of MF and CD30-positive anaplastic large cell lymphoma (ALCL) has also been reported previously, but, as shown in these cases, the two diseases invariably appear in separate locations and run independent clinical course. The diagnosis of CD30 positive LCT in MF should be made when newly-onset papules or nodules arising from the prior MF lesions are seen clinically, and cerebriform T lymphocytes admixing with <75% of CD30 positive large cells are shown histopathologically. If >75% of large cells have CD30 expression, it is very difficult to distinguish CD30+ LCT in MF from MF associated with CD 30+ lymphoproliferative disorder. In such a situation, Vergier et al indicated the clinical evolution may help us discriminate between these two entities.