1/5. Cytophagic histiocytic panniculitis improved by combined CHOP and cyclosporin A treatment.In a 31-year-old Japanese man with cytophagic histiocytic panniculitis (CHP) remission was achieved by a combination of combined chemotherapy CHOP and cyclosporin A treatment. He was admitted to our hospital in January 1994 with recurrent high fever of 40.2 degrees C and tender and violaceous subcutaneous nodules on his trunk, arms and legs. He developed pancytopenia, hemorrhagic diathesis, liver dysfunction. Histological examination of the biopsied subcutaneous nodule revealed a lobular panniculitis with fat necrosis and a massive infiltration of histiocytes phagocytosing nuclear debris. He was treated initially with 40 mg/day prednisolone. However, following a reduction in prednisolone dosage, his symptoms reappeared. CHOP (cyclophosphamide, doxorubicin, vincristine and prednisolone) therapy was then initiated. Three courses of CHOP treatment alleviated his symptoms and cyclosporin A was used to maintain his condition for 15 months. His medication was then discontinued and he has been in complete remission for 10 months. Combined treatment of cyclosporin A and CHOP combined chemotherapy was shown to be effective for this patient with severe CHP.- - - - - - - - - - ranking = 1keywords = diathesis (Clic here for more details about this article) |
2/5. Fatal cytophagic histiocytic panniculitis.Individual cases of so-called Weber-Christian disease with a bleeding diathesis have been reported for several years. These were originally diagnosed as Weber-Christian disease, but have been recategorized on review as a chronic, visceral, and cutaneous histiocytic (cytophagic) panniculitis, progressing to liver dysfunction and jaundice and a terminal hemorrhagic diathesis. We report here a rare catastrophic form of systemic panniculitis in an adolescent girl. Despite compelling clinical evidence, the diagnosis was made only on postmortem biopsies.- - - - - - - - - - ranking = 2keywords = diathesis (Clic here for more details about this article) |
3/5. Cytophagic histiocytic panniculitis with fever, cytopenia, liver failure, and terminal hemorrhagic diathesis.We have seen five adult patients with a clinical picture of recurrent histiocytic, cytophagic panniculitis, cytopenia, abnormal liver function tests, and a terminal, febrile bleeding diathesis. Originally thought to have Weber-Christian disease, these patients, we believe, represent a unique syndrome: lobular, histiocytic, cytophagic panniculitis. Erythrophagocytosis and cytophagocytosis are readily observed, but the cells do not show malignant features. histiocytosis can be found at times in the bone marrow, lymph nodes, liver, spleen, and serosal tissues, as well as in the skin and subcutaneous tissues. The terminal hemorrhage in these patients is characterized by features of pancytopenia, liver failure, and intravascular coagulation. This disease may be separated from malignant histiocytosis by the chronic course, the primary involvement of the adipose tissue, and the benign histiocytes in the infiltrate. It has some similarities to other regional histiocytoses such as sinus histiocytosis, intestinal histiocytosis, and splenic histiocytosis.- - - - - - - - - - ranking = 5keywords = diathesis (Clic here for more details about this article) |
4/5. Successful treatment of cytophagic histiocytic panniculitis by cyclosporin A: a case report.This is a report of a case, 7 1/2 year-old-boy having chronic febrile and recurrent crops of painful subcutaneous nodules on lower extremities, which had previously been diagnosed as Weber-Christian disease, which progressed to have cytophagic histiocytic activity in the skin, bone marrow with abnormal liver function and hemorrhagic diathesis. He was subsequently treated with corticosteroid without good response. After he was diagnosed as having cytophagic histiocytic panniculitis, cyclosporin A was administered intravenously in an initial dosage of 1 mg/kg/day and in oral maintenance dose of 10 mg/kg/day with a successful response and the patient completely recovered within 6 months with mild hypertension as an adverse effect.- - - - - - - - - - ranking = 1keywords = diathesis (Clic here for more details about this article) |
5/5. Hemorrhagic diathesis in Weber-Christian disease.A case of a 21-year-old woman with Weber-Christian disease, associated with consumption coagulopathy and adequately controlled over the past year or more by corticosteroid and heparin therapy, has been described. Through the study on this case, it was concluded that a hemorrhagic diathesis complicating Weber-Christian disease, at least in advanced cases, was characterized by consumption coagulopathy associated with enhanced blood coagulability which arises from hyperlipemia due to abnormal lipid metabolism and further, that the consumption coagulopathy was modified by diminished synthesis of clotting factors and activation of the fibrinolytic system due to the secondary hepatic impairment. It might also be likely that systemic vascular changes contribute to the activation of the clotting system as a trigger of clotting factor consumption. The complex pathophysiology of a hemorrhagic diathesis in this disease seemed to be brought about by a complex combination of such a variety of factors. In addition, decreased erythrocyte sedimentation rate observed characteristically during hemorrhage might probably have a direct correlation with hypofibrinogenemia due to consumption coagulopathy in advanced cases.- - - - - - - - - - ranking = 6keywords = diathesis (Clic here for more details about this article) |