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Rare Reports (rare + report)
Selected AbstractsSafety and efficacy of solvent/detergent-treated antihaemophilic factor with an added 80 °C terminal dry heat treatment in patients with haemophilia AHAEMOPHILIA, Issue 3 2000J. S. Powell Plasma-derived factor VIII concentrates remain an important resource for haemophilia A patients. To improve the safety of these preparations, various methods of viral removal and inactivation have been used that are designed to eliminate both enveloped and non-enveloped viruses. There have been rare reports that some viral inactivation processes altered the immunogenicity of some concentrates, leading to the development of factor VIII inhibitors in previously treated haemophilia A patients. This study evaluated the safety, efficacy and lack of neo-antigenicity of a highly purified factor VIII preparation which undergoes both solvent/detergent treatment and final dry heat treatment at 80 °C for 72 h. The study included: (i) a single blind, single-dose crossover pharmacokinetic study in 18 previously treated patients, comparing sibling lots of the unheated preparation (KoateŽ -HP) and the heat-treated preparation (KoateŽ -DVI), and (ii) an extended home treatment programme for 36 patients at two haemophilia treatment centres primarily to assess immunogenicity. Clinical parameters were assessed at regular intervals. The results confirm that KoateŽ -HP and KoateŽ -DVI are bioequivalent, and that KoateŽ -DVI is safe and efficacious for treatment of acute bleeding episodes and for surgery. Furthermore, the heat-treated preparation is not associated with the development of inhibitors in previously treated patients. [source] Fatal Subcutaneous Panniculitis-Like T-Cell Lymphoma (Sptcl) with Interface Change and Dermal Mucin, A Dead-Ringer for Lupus ErythematosusJOURNAL OF CUTANEOUS PATHOLOGY, Issue 1 2005L. Ma We report a 48-year-old male who presented with ulcerated plaques and nodules of the lower extremities. Skin biopsies revealed a dense lymphocytic infiltrate involving the dermis and the subcutis in a lobular and septal pattern. No overt cytological atypia was present. Notably, several features resembling lupus erythematosus were present, including vacuolar interface change and abundant dermal mucin deposition. The patient developed pulmonary nodules, and a lung biopsy showed a perivascular and interstitial lymphoid infiltrate without overt atypia. The cutaneous and pulmonary lymphoid infiltrates showed similar immunohistochemical profiles: CD3+CD4,CD8+/,CD56+. Monoclonal rearrangements of T-cell receptor gamma gene with similar migration patterns were identified from both locations. The patient developed fatal hemophagocytic syndrome, involving liver, spleen, lymph nodes, and bone marrow. This case is amongst rare reports of subcutaneous panniculitis-like T-cell Lymphoma (SPTCL) with systemic involvement. [source] Primary Cutaneous Carcinosarcoma (PCCS0 Aka Metaplastic Carcinoma)JOURNAL OF CUTANEOUS PATHOLOGY, Issue 1 2005Ramin Ram We describe a case of PCCS in a 73 year-old male who presented with a rapidly growing tumor of 6 months duration on the right ear. Clinical examination revealed a 5 × 4 × 4-cm ulcerated, crusted, exophytic tumor on the superior aspect of the helix. Histologically, the mass revealed a biphasic tumor with malignant epithelial and mesenchymal components. The epithelial component consisted of a few foci of basal cell carcinoma (BCC). The majority of the tumor was composed of osteogenic sarcoma and malignant fibrous histiocytoma (MFH). An intricate transition of BCC into sarcoma was noted in a few foci. Atypical mitosis and necrosis were common. The BCC and tumor giant cells in the MFH area showed positive immunoreaction for cytokeratin and CD68 respectively. The tumor was negative for S-100 protein, HMB-45, muscle actins, chromogranin, and synaptophysin. In conclusion, a PCCS growing predominantly as osteosarcoma and MFH is reported. Although the exact histogenesis of PCCS is unknown, primitive mesenchymal cells of the dermis, surrounding the follicular units, are capable of pluripotent differentiation and are likely the source of origin of the sarcoma. The known aggressive biological behavior warrants wide excision and given the rare reports of metastasis and death, regular follow-up is required [source] Multiple trichoepitheliomas in a mother and daughterAUSTRALASIAN JOURNAL OF DERMATOLOGY, Issue 4 2003Kerry Crotty SUMMARY A 38-year-old mother and her 8-year-old daughter presented with multiple facial papules. In both cases, the papules had started to develop at about the age of 4,5 years. Biopsy of one of the mother's papules revealed a trichoepithelioma. With time the lesions had become less obvious in the mother, so she and her daughter elected to have no treatment. Both mother and daughter were otherwise well. However, there have been rare reports of multiple trichoepitheliomas being associated with systemic conditions. Multiple trichoepitheliomas are inherited in an autosomal dominant manner and have been mapped to chromosome 9p21. [source] | ||||||||||