1/17. 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 = fat (Clic here for more details about this article) |
2/17. A patient with diabetes insipidus and periorbital swellings; erdheim-chester disease.erdheim-chester disease is a rare multisystem disease in which a progressive xanthogranulomatous infiltration of several tissues can be seen. We describe a woman, known to have diabetes insipidus for ten years, with periorbital, retroperitoneal, mediastinal, axillar and inguinal involvement. On histological examination a granulomatous infiltration of fatty tissue and striated muscle was seen, consisting of Touton giant cells, histiocytes with foamy cytoplasm and lymphocytes. Immunohistochemical staining with CD-1a and S-100 was negative and on electron microscopy no Langerhans granules were seen. These findings led to the diagnosis of erdheim-chester disease. She had a good response on steroids. Because of some similar clinical features of Langerhans cell histiocytosis and erdheim-chester disease, a histiocyte disorder seems the most probable cause.- - - - - - - - - - ranking = 1keywords = fat (Clic here for more details about this article) |
3/17. Pseudotumoral bilateral involvement of the breast in erdheim-chester disease: CT appearance.We report a case of pseudotumoral involvement of the breast in erdheim-chester disease. CT shows an enlargement of both breasts with inhomogeneous structure, microcalcifications, and foci of fatty density.- - - - - - - - - - ranking = 1keywords = fat (Clic here for more details about this article) |
4/17. pleural effusion cytology in a case of cytophagic histiocytic panniculitis (subcutaneous panniculitic T-cell lymphoma). A case report.BACKGROUND: Cytophagic histiocytic panniculitis (CHP) presents with subcutaneous panniculitis associated with hemophagocytic syndrome. Many cases of CHP are now being classified as a natural disease progression of subcutaneous panniculitic T-cell lymphoma (SPTL). There have been no cytologic reports dealing with pleural aspirates in cases of CHP or SPTL. CASE: A pleural aspirate obtained from a 19-year-old female revealed lymphoma cells and hemophagocytic histiocytes. A skin biopsy specimen showed the presence of CD8-positive lymphoma cells in fat lobules associated with cytologically benign histiocytes with erythrophagocytosis and lymphophagocytosis. CONCLUSION: Hemophagocytic histiocytes were seen in the pleural effusion from a patient with SPTL.- - - - - - - - - - ranking = 1keywords = fat (Clic here for more details about this article) |
5/17. Hemophagocytic syndrome after living-related liver transplantation.Hemophagocytic syndrome (HPS) is a life-threatening hematological disorder in immunocompromised patients. Although the number of patients with HPS following liver transplantation is scarce the outcome is usually fatal. We report a patient who developed HPS following living-related liver transplantation (LRLT) and was treated successfully by a combination of intravenous (IV) immunoglobulin (Ig) and granulocyte colony-stimulating factor (GCSF).- - - - - - - - - - ranking = 1keywords = fat (Clic here for more details about this article) |
6/17. Massive bone marrow crystal-storing histiocytosis in a patient with IgA-lambda multiple myeloma and extensive extramedullary disease. A case report.We report a case of multiple myeloma (MM) displaying an unusual course, with metastatic spread in uncommon sites (gastroduodenal and upper respiratory tract, breast, skin and liver) and with a fatal outcome. In our patient this plasma cell neoplasm was associated with a rare condition named crystal-storing histiocytosis (CSH), resulting from the storage in reactive histiocytes of crystalline immunoglobulin inclusions. These crystal-forming paraprotein components are secreted by the neoplastic plasma cells and give rise to the crystalline material of the histiocytes only after their ingestion and degradation by the same histiocytes. In this disorder crystal-storing cells may be present in various tissues (in our case mainly in bone marrow), often with functional alterations of the involved organs. In our opinion the association of this "atypical" MM with CSH is to be considered an uncommon event.- - - - - - - - - - ranking = 1keywords = fat (Clic here for more details about this article) |
7/17. Histiocytic cytophagic panniculitis: report of a case in a 12-year-old girl.We report a 12-year-old girl with nodular skin lesions, fever, pancytopenia, coagulation defects and a fatal outcome within 4 months. Histopathology revealed proliferation of histiocytes with haemophagocytosis in skin, perivisceral fat and haemopoietic organs. The symptoms were most consistent with cytophagic histiocytic panniculitis.- - - - - - - - - - ranking = 2keywords = fat (Clic here for more details about this article) |
8/17. Orbital and eyelid involvement with erdheim-chester disease. A report of two cases.erdheim-chester disease is an idiopathic condition characterized by infiltration of the heart, lungs, retroperitoneum, bones, and other tissues by a fibrosing xanthogranulomatous process composed of xanthomatous histiocytes and Touton giant cells. This condition is often fatal, with death due to cardiomyopathy, severe lung disease, or chronic renal failure. Ocular findings with this potentially fatal disease are rare. We report the clinical and histopathologic findings in two cases of bilateral xanthelasmas and bilateral orbital infiltrates in association with erdheim-chester disease. The first patient was a 38-year-old man with cardiovascular and renal disease and severe retroperitoneal fibrosis. The massive orbital infiltration produced bilateral blindness. The second patient was a 77-year-old man with severe cardiovascular disease and retroperitoneal fibrosis. The diagnosis was confirmed in both patients with retroperitoneal and orbital biopsies. Both patients had the unusual occurrence of bilateral xanthelasmas with bilateral, diffuse orbital masses, eye findings that should alert the clinician to the possibility of this serious systemic disease.- - - - - - - - - - ranking = 2keywords = fat (Clic here for more details about this article) |
9/17. Unexpected infant death attributable to cardiac tumor or cardiomyopathy. Immunohistochemical and electron microscopical findings in three cases.The pathological findings, including immunohistochemical and electron microscopical findings, in three infants who died unexpectedly of cardiac tumor or cardiomyopathy are reported. The first was a 13-month-old boy with tuberous sclerosis and multiple rhabdomyomas of the heart, who presented with a postpartal cardiac murmur and moderate cardiomegaly. The further history was unknown. The rhabdomyoma nodules were composed of spider cells containing small amounts of desmin and myosin as well as isolated myofibrils. Microscopically small glioma nodules contained high amounts of GFAP. The second case, a boy 4 months of age, died of a large benign fibrous histiocytoma of the heart after an uneventful history. Tumor cells contained alpha-1-anti-chymotrypsin and lysozyme. The third case, a girl 2 months of age, died unexpectedly of histiocytoid cardiomyopathy. The affected cells contained fat droplets, glycogen granules, many leptomer myofibrils and small amounts of myosin and desmin.- - - - - - - - - - ranking = 1keywords = fat (Clic here for more details about this article) |
10/17. Cytophagic histiocytic panniculitis evolving into total lipodystrophy.A 15-year-old boy had persistent fever with severe neutropenia, thrombocytopenia, coagulation disorder, and marked elevation of lactate dehydrogenase values. A diagnosis of cytophagic histiocytic panniculitis was made after repeated skin biopsies. Three years after onset, he gradually lost 20 kg in body weight, and both skin and bone marrow specimens revealed degenerative changes of fatty tissue without any inflammatory cells. These findings suggest that total lipodystrophy syndrome results from the inflammatory destructive process of adipose tissue. To our knowledge, our report is the first to show, in a series of histologic studies, that an inflammatory destructive process may be involved in some cases of total lipodystrophy syndrome.- - - - - - - - - - ranking = 73.59176868115keywords = adipose, fat (Clic here for more details about this article) |
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