1/25. Large annular purpura and paraneoplastic purpura in a patient with sjogren's syndrome and cervical cancer.We report a 79-year-old female with anaphylactoid purpura on her legs and unusual large annular purpura on the trunk. Histopathological characteristics of leukocytoclastic vasculitis were observed in the upper and middle dermis of both types of skin lesions. She was complicated by sjogren's syndrome and advanced cervical cancer. The annular purpura spontaneously resolved in a week and did not recur. However, the anaphylactoid purpura relapsed more frequently and spread more widely following the elevation of her serum SCC antigen levels from the onset of purpura until her death. We consider that the characteristic annular configuration was caused by the complication of sjogren's syndrome and that the recurrent anaphylactoid purpura indicated paraneoplastic vasculitis primarily caused by the tumor specific protein immune complexes. Complication by Sjogren's syndrome many also play a role in the development of allergic vasculitis.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
2/25. Posttransfusion purpura secondary to an alloantibody reactive with HPA-5a (Br(b)).BACKGROUND: Posttransfusion purpura (PTP) is characterized by severe thrombocytopenia following blood transfusion that results from alloimmunization to platelet-specific alloantigens. Most cases involve antibodies against HPA-1a in homozygous HPA-1b persons. CASE REPORT: A patient developed PTP after cardiopulmonary bypass associated with a platelet-specific antibody with strong reactivity against HPA-5a (Br(b)). Geno-typing confirmed that the patient was homozygous for HPA-5b. CONCLUSION: This is the first well-documented occurrence of PTP associated with isolated allosensitization to HPA-5a or Br(b). The case highlights the importance of maintaining a high level of suspicion for PTP in the appropriate clinical setting, even in an atypical patient.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
3/25. Post-transfusion purpura: case report.Post-transfusion purpura (PTP) is a rare bleeding disorder of platelet alloimmunization that perhaps occurs as an anamnestic reaction. Most commonly, it is observed in PlA1-negative subjects previously sensitized with PlA1 platelet antigen either through PlA1-positive pregnancy or PlA1-positive transfusion. PTP appears with sudden severe thrombocytopenia, purpura, and often life-threatening hemorrhage within 5-10 days of blood transfusion. It is believed to be self-resolving. Yet inactivity risking dangerous bleeding can be disastrous. Treatment with intravenous immunoglobulin, corticosteroids, exchange transfusion, and plasmapheresis has been reported with variable success. No single modality, however, is effective in all cases. Not more than 150 cases of PTP seem to have been reported. We present two such cases. Both were multiparous PlA1-negative women given a blood transfusion for the first time. Corticosteroid therapy failed in both. One responded to intravenous immunoglobulin, while for the other plasmapheresis was the only life-saving modality. One of them subsequently required a blood transfusion for surgical intervention, which could be given uneventfully.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
4/25. autoantibodies and the spectrum of sjogren's syndrome.In studies reported recently, the sera of patients with sjogren's syndrome were found to contain precipitating antibodies to nuclear antigens that can be identified by immunodiffusion analysis. These precipitating autoantibodies have been termed SS-A and SS-B antibodies. We show that identification of these autoantibodies helped in establishing the diagnosis of sjogren's syndrome in 12 of 30 patients in whom the diagnosis had not been considered at the time of the physician's initial examination. The reasons for this were related to lack of spontaneous complaints of keratoconjunctivitis sicca and xerostomia and prominence of symptoms associated with arthritis, myalgia, pulmonary fibrosis, and cardiac disease. This study re-emphasizes the importance of multisystem disease in sjogren's syndrome and shows that specific serologic assays for autoantibodies aided in diagnosis.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
5/25. Post-transfusion purpura in a patient with HPA-1a and GPIa/IIa antibodies.Post-transfusion purpura is a rare bleeding disorder characterized by severe and sudden thrombocytopenia within 3-12 days after blood transfusion. Typically, preformed antibodies directed against human platelet antigens, especially HPA-1a, are associated with the clinical symptoms. A 46-year-old female presenting to the hospital with acute progressive kidney insufficiency and anaemia received two units of packed red blood cells (RBC) within 2 days. On day 7, platelet count felt from 414 to 189 x 10(9) L(-1) and 1 day later dropped to 4 x 10(9) L(-1). Four platelet concentrates were applied without success. After serological confirmation of an HPA-1a antibody, the patient was treated with intravenous gamma immunoglobulin (ivIgG), and the platelet count increased to normal values on day 17. In addition to the persisting HPA-1a alloantibody, an antibody reactive with GPIa/IIa of HPA-5a- and HPA-5b-positive platelets was detected during the acute phase of thrombocytopenia. After complete remission, the patient was transfused with four units of packed RBC from HPA-1a-negative donors, and platelet counts remained normal.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
6/25. Post-transfusion purpura: a challenging diagnosis.BACKGROUND: Post-transfusion purpura is a rare syndrome characterized by severe thrombocytopenia and bleeding caused by alloimunization to human platelet specific antigens following a blood component transfusion. The suggested incidence is 1:50,000-100,000 transfusions, most often occurring in multiparous women. The diagnosis is not easy because these patients, who are often critically ill or post-surgery, have alternative explanations for thrombocytopenia such as infection, drugs, etc. OBJECTIVES: To describe patients with initially misdiagnosed PTP and to emphasize the diagnostic pitfalls of this disorder. patients AND RESULTS: During a period of 11 years we diagnosed six patients with PTP, four women and two men. The incidence of PTP was approximately 1:24,000 blood components transfused. We present the detailed clinical course of three of the six patients in whom the diagnosis was particularly challenging. The patients were initially misdiagnosed as having heparin-induced thrombocytopenia, systemic lupus erythematosus complicated by autoimmune thrombocytopenia, and disseminated intravascular coagulation. A history of recent blood transfusion raised the suspicion of PTP and the diagnosis was confirmed by appropriate laboratory workup. CONCLUSIONS: PTP seems to be more frequent than previously described. The diagnosis should be considered in the evaluation of life-threatening thrombocytopenia in both men and women with a recent history of blood transfusion.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
7/25. Posttransfusion purpura due to an alloantibody reactive with glycoprotein Ia/IIa (anti-HPA-5b).A 38-year-old woman (JT) was diagnosed with posttransfusion purpura and significant posthysterectomy vaginal bleeding 9 days after the transfusion of 2 U of packed red blood cells. Analysis of JT's serum by a monoclonal antibody-antigen capture enzyme-linked immunosorbent assay method showed the presence of anti-HPA-5b (anti-Bra) antibodies directed against an epitope on platelet glycoprotein (GP) la of the GPIa/IIa complex. The patient's serum immunoprecipitated two proteins from 125I-labeled HPA-5b positive platelets that migrated under both nonreducing and reducing conditions on sodium dodecyl sulfate polyacrylamide gels at molecular weights characteristic of GPIa (150 Kd and 165 Kd, respectively) and GPIIa (120 Kd and 145 Kd, respectively). These bands were not precipitated when 125I-labeled HPA-5b negative platelets were used. Platelet typings performed on JT and her three children showed that the patient was HPA-5b negative and one of her children was HPA-5b positive. Platelets obtained from one of the donors who provided blood for the inciting transfusion also typed as HPA-5b positive. These findings demonstrate that posttransfusion purpura may be induced by antibodies directed against an alloantigenic epitope, namely HPA-5b (Bra), located on GPIa/IIa. Moreover, clinically significant bleeding can be associated with antibody reactions directed against this GP complex.- - - - - - - - - - ranking = 2keywords = antigen (Clic here for more details about this article) |
8/25. Posttransfusion purpura associated with alloantibody specific for the platelet antigen, Pen(a).Posttransfusion purpura (PTP) and severe thrombocytopenia occurred 9 days after transfusion of red blood cells to a 48-year-old, multiparous Navajo woman. The platelet count rose to hemostatic levels after treatment with prednisone and three plasma exchange transfusions. Serologic studies showed that the patient's serum contained the potent antibody reactive with platelets from nearly all normal subjects, but nonreactive with autologous platelets obtained after recovery. This antibody was found to be specific for a high-frequency, platelet-specific antigen, designated Pen(a),implicated previously as an immunogen in neonatal alloimmune thrombocytopenic purpura. An exchange of serum showed that Pena is identical with an alloantigen designated Yuk(b) by Japanese workers. We conclude that PTP can occur in association with alloimmunization against Pen(a) (Yuk(b).- - - - - - - - - - ranking = 6keywords = antigen (Clic here for more details about this article) |
9/25. Human parvovirus (HPV/B19) infection with purpura.A 33-year-old man complained of purpura (petechial hemorrhage) in chelidons, poples, axillae, and bilateral chest in addition to other symptoms such as lumbago, arthralgia, muscular pain, and fever. On the next day of the onset, human parvovirus (HPV/B19) antigen and HPV/B19 dna were detected in his serum, and twelve days later IgM antibody to HPV/B19 became detectable. This case supports the relationship between purpura and HPV/B19 infection.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
10/25. Post-transfusion purpura and isoimmune neonatal thrombocytopenia in the same family.Post-transfusion purpura and isoimmune neonatal thrombocytopenia are rare and unusual syndromes leading to severe thrombocytopenia. In both disorders the PLA1 platelet antigen is involved in the pathogenesis. A 41-year-old woman with an obstetrical history of isoimmune neonatal thrombocytopenia in two of her children developed post-transfusion purpura following a transfusion of packed red blood cells. Despite the apparent link in these two disorders associated with the PLA1 platelet antigen, this is the first reported case of post-transfusion purpura and isoimmune neonatal thrombocytopenia occurring in the same family.- - - - - - - - - - ranking = 2keywords = antigen (Clic here for more details about this article) |
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