1/23. Heavy transfusions and presence of an anti-protein 4.2 antibody in 4. 2(-) hereditary spherocytosis (949delG).BACKGROUND AND OBJECTIVE: A patient with hereditary spherocytosis (HS) was found not to have red cell membrane protein 4.2. This rare form of HS, or 4.2 (-) HS, stems from mutations within the ELB42 or the EPB3 genes. The patient had long suffered from a gastric ulcer and impaired liver function. He had had several dramatic episodes of gastrointestinal tract bleeding and had received numerous transfusions. An antibody against a high frequency, undefined antigen was found, creating a transfusional deadlock. We elucidated the responsible mutation and searched for an anti-protein 4.2 antibody. DESIGN AND methods: Red cell membranes were analyzed by SDS-PAGE and by Western blotting. Nucleotide sequencing was performed after reverse transcriptase-polymerase chain reaction (RT-PCR) and nested PCR. RESULTS: The not previously described mutation was a single base deletion: 949delG (CGCAECC, exon 7, codon 317) in the homozygous state. It was called protein 4.2 Nancy. The deletion placed a non-sense codon shortly downstream so that no viable polypeptide could be synthesized. The patient carried a strong antibody against protein 4.2 as shown by Western blotting. INTERPRETATION AND CONCLUSIONS: The manifestations resulting from the mutation described were compared with the picture of HS stemming from other ELB42 gene mutations. We discuss the mechanism through which the anti-protein 4.2 antibody developed. There was no way to establish or to rule out whether the antibody participated in the transfusional deadlock found in our patient.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
2/23. A case report of a hemolytic transfusion reaction caused by anti-Holley.Clinical and laboratory investigation of a black male patient, who received emergency transfusion of blood incompatible for a high incidence antigen, provided evidence for the pathogenetic importance of high titer anti-Holley antibodies with poor avidity. The red blood cells of the patient and two siblings were remarkable in being negative for two high incidence antigens, Hy and hrS, and weak for a third, Gy.- - - - - - - - - - ranking = 2keywords = antigen (Clic here for more details about this article) |
3/23. Immunological studies in familial primary biliary cirrhosis.Tests for cell-mediated immunity and presence of autoantibodies were performed in a mother and daughter with primary biliary cirrhosis. lymphocytes transformation to phytohemagglutinin, delayed cutaneous response to one or more skin test antigens, and percentage of peripheral T and B lymphocytes were normal in both patients. Although successful in the mother, dinitrochlorbenzene sensitization was not achieved in the daughter. histocompatibility antigens 1 and 8, elevated levels of IgM, and antibodies to mitochondria, smooth muscle, and skeletal muscle were present in both patients. However, the clinical course was more severe in the daughter who developed portal hypertension with bleeding esophageal varices requiring portacaval anastomosis. Except for intermittent pruritus, the mother has remained asymptomatic.- - - - - - - - - - ranking = 2keywords = antigen (Clic here for more details about this article) |
4/23. Allergic colitis presenting in the first day of life: report of three cases.Allergic colitis can occur within hours of birth and should be considered in the differential diagnosis of any newborn in whom hematochezia develops. This diagnosis should be considered after excluding infectious and anatomic disorders common to this age group. The diagnosis is supported by the healthy appearance of affected infants and specific proctosigmoidoscopic and histopathologic findings. Infants with allergic colitis usually respond to withdrawal of the offending antigen, by the use of hydrolyzed cow's milk protein formula or more elemental formulas, or if the infant has been breast fed, by the strict removal of the offending antigen from the breast-feeding mother's diet.- - - - - - - - - - ranking = 2keywords = antigen (Clic here for more details about this article) |
5/23. Esophagatis and perinatal cytomegalovirus infection.A 6-week-old male infant presented to hospital with a large upper gastrointestinal hemorrhage. Endoscopic biopsies of his esophagus and duodenum showed cytomegalovirus (CMV) inclusions and CMV early antigen was detected by immunohistochemistry. There were no other signs of congenital CMV infection. This case adds to the clinical spectrum of perinatal CMV infection and may be more common than is currently recognized.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
6/23. Systemic lupus erythematosus complicated by cytomegalovirus-induced hemophagocytic syndrome and colitis.Here, we report a case of systemic lupus erythematosus (SLE) complicated by cytomegalovirus (CMV)-induced hemophagocytic syndrome (HPS) and colitis. A 44-year-old woman with SLE was treated with corticosteroid and cyclophosphamide for lupus nephritis. Although her lupus nephritis improved, fever, progressive pancytopenia and intestinal bleeding were observed. A bone marrow aspiration showed an increase in mature histiocytes with hemophagocytosis. In addition, a colonoscopy showed hemorrhagic colitis with ulcer and the biopsy specimen from the colon revealed typical CMV cells with CMV inclusions confirmed by immunohistochemistry. Furthermore, a large number of CMV antigen-positive leukocytes was detected, suggesting an active CMV infection. CMV infection is serious in compromised hosts. Therefore clinicians should be aware of the clinical settings in which this infection can arise and the target organs potentially affected in order to initiate the appropriate intervention.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
7/23. Hepatitis B associated fulminant polyarteritis nodosa: successful treatment with pulse cyclophosphamide, prednisolone and lamivudine following emergency surgery.For hepatitis b virus associated polyarteritis nodosa, alpha interferon and plasma exchanges have been proposed to be the first-line treatment. We report a case of hepatitis B surface antigen (HBsAg)-positive fulminant polyarteritis nodosa with predominant gastrointestinal involvement who showed good response to pulse cyclophosphamide, prednisolone, and lamivudine therapy. The patient, a 22-year-old man, presented with a short history of epigastric pain. Initial upper gastrointestinal endoscopy revealed gastritis and duodenal erosions. His pain did not respond to H2-receptor antagonists. He had slightly impaired liver function tests, and was HBsAg and hepatitis B e antigen (HBeAg) positive. Around 3 weeks after initial presentation, he developed massive gastrointestinal haemorrhage requiring resuscitation and emergency laparotomy. Microscopic examination of the resection specimens revealed necrotizing vasculitis of small and medium-sized arteries in the submucosa compatible with polyarteritis nodosa. The patient was treated with pulse cyclophosphamide and prednisolone, with lamivudine being added when he showed an acute rise in liver enzymes. He subsequently developed HBeAg seroconversion, and remained well 18 months after cessation of all immunosuppressives. We believe that the efficacy of pulse cyclophosphamide, prednisolone, and lamivudine in the treatment of hepatitis b virus associated polyarteritis nodosa, especially in comparison with interferon and plasma exchanges, deserves further evaluation.- - - - - - - - - - ranking = 2keywords = antigen (Clic here for more details about this article) |
8/23. Severe intestinal bleeding caused by intestinal metastases of a primary angiosarcome of the thyroid gland.A 75 year old male presented with gastrointestinal bleeding after resection of both upper lobes of the lungs because of metastases. One year ago an angiosarcoma was the reason for a complete removal of the thyroid gland. In esophago-gastro-duodenoscopy we found multiple hemorrhagically stained polyploids lesions in the postbulbar duodenum and jejunum. colonoscopy showed isolated polyploid lesions of the right flexura. Because of persistent gastrointestinal bleeding a diagnostic laparotomy was done. Intraoperative intestinoscopy demonstrated multiple bleeding metastasis. To remove many of the bleeding lesions two longer intestinal segments of the jejunum and ileum were resected. The histology of the metastases showed arrangements of polygonal cells with prominent nucleoli and atypical mitosis. immunohistochemistry identified CD 31, vimentin and factor viii associated antigen. There was an erosion of the superficial intestinal mucosal cells with resulting hemorrhage; same histology had been found in the thyroid gland and the right upper lobe of lung. Eight days after surgery the patient died because of respiratory and circulatory insufficiency.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
9/23. Anti-factor V auto-antibody in the plasma and platelets of a patient with repeated gastrointestinal bleeding.Development of autoantibody against coagulation factor V (FV) is a rare clinical condition with hemorrhagic complications of varying severity. The aim of this study was to establish the pathomechanism of an acquired FV deficiency and characterize the FV inhibitor responsible for the clinical symptoms. A 78-year-old female was admitted to hospital with severe gastrointestinal bleeding. General clotting tests and determination of clotting factors were performed by standard methods. FV antigen and FV containing immune complexes were measured by ELISA. The FV molecule was investigated by Western blotting and by sequencing the f5 gene. The binding of patient's IgG to FV and activated FV (FVa) was demonstrated in an ELISA system and its effect on the procoagulant activity of FVa was tested in clotting tests and in a chromogenic prothrombinase assay. Localization of the epitope for the antibody was performed by blocking ELISA. FV activity was severely suppressed both in plasma and platelets. FV antigen levels were normal by ELISA using polyclonal anti-FV antibody or monoclonal antibody against the connecting region of FV, but depressed when HV1 monoclonal antibody against the C2 domain in the FV light-chain was used as capture antibody. The FV molecule was found intact. An IgG reacting with both FV and FVa was present in the patient's plasma and its binding to FV was inhibited by HV1 antibody. FV-containing immune complexes were detected in the patient's plasma and platelet lysate. The patient's IgG inhibited the procoagulant function of FVa. An anti-FV IgG was present in the patient's plasma and platelets. The autoantibody reacted with an epitope in the C2 domain of FV light chain and neutralized the procoagulant function of FVa.- - - - - - - - - - ranking = 2keywords = antigen (Clic here for more details about this article) |
10/23. Multidisciplinary treatment of synchronous primary rectal and prostate cancers.BACKGROUND: A 58-year-old Caucasian man with a history of irritable bowel syndrome and occasional rectal bleeding presented with a 4-week history of progressive, bright red blood per rectum. A digital rectal examination revealed a 3 cm distal, midrectal mass. Laboratory tests showed an elevated serum prostate-specific antigen of 32 ng/ml but other physical and medical examinations were unremarkable. INVESTIGATIONS: digital rectal examination, colonoscopy, rectal mass biopsy, endorectal ultrasound, transrectal ultrasound-guided prostate biopsy, CT scan and MRI. diagnosis: Clinical stage III (T3N1M0), moderately differentiated adenocarcinoma of the rectum and clinical stage II (T1cN0M0) adenocarcinoma of the prostate. MANAGEMENT: Intensity-modulated radiation therapy, chemoradiation, chemotherapy, hormone therapy and surgery.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
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