1/127. central nervous system T-cell lymphoproliferative disorder in a patient with chronic active Epstein-Barr virus infection.PURPOSE: central nervous system (CNS)-T cell lymphoproliferative disorder (T-LPD) developing during the course of chronic active Epstein-Barr virus (CAEBV) infection is reported. patients AND methods: CAEBV was diagnosed in a 14-month-old boy with fever, cytopenia, hepatosplenomegaly, and abnormal high titers of anti-Epstein-Barr virus (EBV) antibodies. At 8 years of age, he had a splenectomy because of progressive disease. RESULTS: After 27 months of clinical remission, muscle weakness and paresthesia developed. magnetic resonance imaging of his brain showed spotty T2 prolongation in left parietal, bilateral frontal, and temporal white matter with meningeal enhancement. brain biopsy revealed the cerebral infiltration of CD3 , CD4 , CD8-, CD45RO , CD56-, and EBV-encoded rna 1 cells. CONCLUSIONS: The CNS involvement of EBV-associated T-LPD is a rare but serious complication in CAEBV without known underlying immunodeficiency.- - - - - - - - - - ranking = 1keywords = splenomegaly (Clic here for more details about this article) |
2/127. Chronic urticaria as a presenting sign of hairy cell leukemia.Chronic urticaria is a common clinical disorder that is idiopathic in over 75% of cases. Less commonly, urticaria may be the presenting manifestation of an allergic or infectious disease, endocrinopathy, inherited syndrome, or autoimmune disorder. Rarely, urticaria may be a sign of underlying malignancy, including leukemia. C.C. is a 48-year-old white female who was referred for evaluation of recurrent urticaria for 3 years. The pruritic, erythematous wheals were pinpoint, and appeared to be precipitated by heat, stress, and effort. Prick tests were negative except to D. pteronyssinus. CBCs over the past 5 years revealed WBCs of 2,300-5,000 cells/mm3. skin biopsy revealed interstitial edema with infiltration of eosinophils and mast cells consistent with urticaria. The impression was probable cholinergic urticaria, for which hydroxyzine was prescribed with fair symptomatic control. One year later, she presented with bright red blood per rectum. Repeat physical examination revealed lymphadenopathy and splenomegaly. Subsequent laboratory studies showed pancytopenia. endoscopy was normal except for small, nonbleeding hemorrhoids. bone marrow biopsy revealed histologic evidence of hair, cell leukemia that was treated with 2-chlorodeoxyadenosine. Upon initiation of chemotherapy her pruritus and urticaria subsided. Recent CBC revealed Hgb 9.2 g/dL, platelets 290,000 cells/mm3, and WBC 4,100 cells/mm3. Peripheral blood smear showed no hairy cells.- - - - - - - - - - ranking = 1keywords = splenomegaly (Clic here for more details about this article) |
3/127. Acute hemorrhage into the peritoneal cavity--a complication of chronic pancreatitis with pseudocyst: a case report from clinical practice.Acute hemorrhage due to a pseudocyst of the pancreas is a dangerous complication of chronic pancreatitis (CP). Without operative treatment, mortality is as high as 90%. Immediate recognition of this complication as well as urgent operative treatment allowing the survival of 70% of patients is imperative. Described is the case of a patient with CP and pseudocyst in which hyperamylasemia and unclarified anemia developed following sudden abdominal pain. The suspicion of hemorrhage into the peritoneal cavity was confirmed by selective visceral angiography showing hemorrhage from the splenic artery in the region of the hilus of the spleen. Operative treatment was successful. During the procedure, a ligature was applied to the hemorrhaging splenic artery and a splenectomy was carried out with 2500 ml of bloody contents being removed from the abdominal cavity. Acute hemorrhage into the peritoneal cavity as a complication of chronic pancreatitis with pseudocyst (CPP) requires immediate identification, confirmation by visceral angiography, and urgent operative treatment.- - - - - - - - - - ranking = 0.14964546575805keywords = spleen (Clic here for more details about this article) |
4/127. Surgical approaches for pancreatic ascites: report of three cases.Pancreatic ascites can occur in association with the rupture of a pseudocyst or the disruption of a pancreatic duct during the natural course of chronic pancreatitis. We report herein the successful treatment of three patients with pancreatic ascites by performing a surgical procedure after 4-6 weeks of total parenteral nutrition (TPN) proved ineffective. The principles of our surgical procedure for pancreatic ascites are as follows: (1) minimum pancreatic tissue is resected; (2) surgical intervention to repair leaking sites is not necessary; (3) pancreatic duct drainage is facilitated by an intestinal Roux-en-Y loop; (4) An external drainage tube is inserted through the Roux-en-Y loop into the main pancreatic duct. All three patients who underwent our surgical procedure had a good outcome. Although the mean follow-up time is still only 18.3 months, their condition has improved, with no evidence of recurrent ascites. Thus, our surgical procedure should be considered as an appropriate treatment for pancreatic ascites because it can be applied for all types of leakage, including leakage from the posterior wall of pancreas; it preserves pancreatic function, especially endocrine function; and it enables preservation of the spleen.- - - - - - - - - - ranking = 0.14964546575805keywords = spleen (Clic here for more details about this article) |
5/127. Three generations of hereditary chronic pancreatitis.The patient was a 22 year-old male. Hereditary chronic pancreatitis was suspected as a diagnosis since his mother's uncle had been operated on for chronic pancreatitis 14 years previously at the age of 64 years and his mother had been operated on for chronic pancreatitis with calculi 5 years previously at the age of 40 years. Surgery was needed, since: 1) he had experienced abdominal pain for 8 years; 2) endoscopic retrograde cholangiopancreatography (ERCP) revealed a marked irregular dilatation in the main pancreatic duct and a marked irregular dilatation and protein plugs in the ductule of the tail of the pancreas; and, 3) pancreatic functional diagnostic (PFD) test examination showed a 75% decrease in exocrine function. If a surgical procedure had not been performed, the patient would likely have experienced calculi formation in the pancreas and a further decrease in exocrine function. Since the patient was very young and had many protein plugs in the dilated ductule of the tail of the pancreas, we decided to perform a spleen-preserving Puestow's procedure with removal of the tail of the pancreas. Clinical and pathological findings of hereditary pancreatitis are reviewed.- - - - - - - - - - ranking = 0.14964546575805keywords = spleen (Clic here for more details about this article) |
6/127. Post-traumatic pseudocyst of the spleen: sclerotherapy with ethanol.We report a case of successful percutaneous treatment of a chronic post-traumatic splenic pseudocyst using alcohol as the sclerosing agent. A 26-year-old man presented with a symptomatic cystic mass located in the spleen. Aspiration of 300 ml of fluid was only temporarily effective, and therefore a drainage catheter was placed 3 days later. After histopathologic and microbiologic exclusion of a malignant or infectious origin, local sclerotherapy with alcohol was performed because of recurrence after percutaneous drainage. This therapy was repeated six times within 2 weeks. Two weeks later, the remaining volume was determined to be 16 ml. Six months after treatment the cyst was no longer visible. To our knowledge this is the first case of a chronic post-traumatic splenic cyst treated with alcohol. Percutaneous sclerotherapy of a symptomatic post-traumatic splenic pseudocyst may be an alternative to surgical treatment.- - - - - - - - - - ranking = 0.74822732879023keywords = spleen (Clic here for more details about this article) |
7/127. anemia due to massive chronic foetomaternal hemorrhage.We report a case of massive chronic foetomaternal hemorrhage. The labor course was uncomplicated. The newborn presented with pallor. tachypnea, and moderate hepatosplenomegaly. The initial hemoglobin was 6.5 g/dl. The Kleihauer-Betke stain on a maternal blood sample was 12%, which is equivalent to 540 ml of fetal blood in the maternal circulation. A clot in the umbilical vein was demonstrated sonographically. The possible association of foetomaternal hemorrhage with umbilical vein thrombosis is discussed.- - - - - - - - - - ranking = 1keywords = splenomegaly (Clic here for more details about this article) |
8/127. Cytogenetic studies of the spleen in chronic granulocytic leukaemia.Using a technique of short-term in vitro culture, cytogenetic studies were performed on splenic tissue from 12 patients with chronic granulocytic leukaemia. In eight patients the disease was in its chronic phase and had been treated with busulphan (seven cases) or splenic irradiation (one case). In five of these patients, small numbers of dividing cells possessing the philadelphia (Ph1) chromosome were observed; in one, only Ph1 -negative metaphases, presumably those of lymphocytes, occurred, and in two patients no dividing cells were obtained. In four patients chronic granulocytic leukaemia had undergone metamorphosis to an acute phase: in two of these patients no dividing cells were observed in splenic cultures with or without added phytohaemagglutinin: this result may have been attributable to prior cytotoxic therapy. In cultures from the other two patients in metamorphosis, almost all dividing cells were Ph1 -positve and many cells possessed two Ph1 chromosomes. Full cytogenetic analysis in one of these cases showed that the spleen contained several closely related cell lines, apparently reflecting progressive tumour cell evolution.- - - - - - - - - - ranking = 0.74822732879023keywords = spleen (Clic here for more details about this article) |
9/127. Systemic AL amyloidosis in gaucher disease. A case report and review of the literature.Chronic gaucher disease [GD] in association with systemic AL amyloidosis is extremely rare. We describe a 46-year-old Greek male with chronic GD confirmed by low glucocerebroside activity in fibroblasts and N370S/L444P mutations at the cerebrosidase gene, who also had systemic AL amyloidosis diagnosed by bone marrow diffuse plasmacytosis, serum monoclonal IgA-lambda, severe total proteinuria with monoclonal IgA-lambda, Bence-Jones-lambda and amyloid deposits in bone marrow, liver, spleen and kidney biopsy specimens. Treatment with melphalan and prednizolon has dramatically decreased both levels of serum M-IgA and proteinuria and also improved the clinical symptoms of amyloidosis. He died from restrictive cardiac disease 30 months after the diagnosis of amyloidosis. Previously reported cases of GD in association with AL amyloidosis are reviewed.- - - - - - - - - - ranking = 0.14964546575805keywords = spleen (Clic here for more details about this article) |
10/127. Cryptococcal meningitis in an hiv negative patient with systemic sarcoidosis.A case of cryptococcus neoformans meningitis is described in an hiv negative patient with undiagnosed systemic sarcoidosis. The patient presented with signs of meningitis together with generalised lymphadenopathy and hepatosplenomegaly. Cryptococcal meningitis was diagnosed on lumbar puncture. She was treated with intravenous amphotericin b but died within two weeks of admission. Necropsy revealed lesions in the lungs, liver, spleen, lymph nodes, small intestine, and bone marrow consistent with sarcoidosis. Microscopically the lesions contained non-caseating epithelioid cell granulomas typical of sarcoidosis. No Schaumann or Hamazaki-Wesenberg bodies were identified. cryptococcus neoformans meningitis is generally associated with immunosuppressive disorders. As T cell abnormalities have been described in sarcoidosis, this could have been a case of opportunistic infection. Although rare, sarcoidosis merits consideration in patients with cryptococcal disease in the absence of hiv infection.- - - - - - - - - - ranking = 1.149645465758keywords = splenomegaly, spleen (Clic here for more details about this article) |
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