1/8. Acute onset hemoglobinemia and/or hemoglobinuria and sequelae following Rh(o)(D) immune globulin intravenous administration in immune thrombocytopenic purpura patients.Rh(o)(D) immune globulin intravenous (anti-D IGIV) was licensed by the united states food and drug administration (FDA) in March 1995 to treat patients with immune thrombocytopenic purpura (ITP). Anti-D IGIV induces extravascular hemolysis, an expected adverse reaction that is consistent with the presumed mechanism of action. Between licensure and April 1999, the FDA received 15 reports of hemoglobinemia and/or hemoglobinuria following anti-D IGIV administration that met the case definition for this review. The mechanism responsible for hemoglobinemia and/or hemoglobinuria is unexplained. review of these reports was prompted by the seriousness and the unexpectedness of treatment-associated sequelae experienced by 11 patients. Of these patients, 7 developed sufficient onset or exacerbation of anemia that orders were written for packed red blood cell transfusions, although only 6 patients were transfused. Eight patients experienced the onset or exacerbation of renal insufficiency, and 2 patients underwent dialysis. One patient died due to complications of exacerbated anemia. Six patients experienced 2 to 3 sequelae. Absent validated incidence data, a 1.5% estimated incidence rate from published clinical trial data and a 0.1% estimated reporting rate from FDA and drug utilization data were calculated for reported cases of hemoglobinemia and/or hemoglobinuria. This review presents the first case series of anti-D-IGIV-associated hemoglobinemia and/or hemoglobinuria and provides pretreatment and posttreatment clinical and laboratory findings of the case series patients. The primary purpose of this review is to increase awareness of this potentially serious occurrence among physicians and health care professionals who manage ITP patients treated with anti-D IGIV, thereby enabling prompt recognition and treatment of sequelae. (Blood. 2000;95:2523-2529)- - - - - - - - - - ranking = 1keywords = physician (Clic here for more details about this article) |
2/8. disseminated intravascular coagulation associated with acute hemoglobinemia or hemoglobinuria following Rh(0)(D) immune globulin intravenous administration for immune thrombocytopenic purpura.The Food and Drug Administration (FDA) licensed Rh(o)(D) immune globulin intravenous (anti-D IGIV) on March 24, 1995, for treatment of immune thrombocytopenic purpura (ITP). A previous review described data on 15 patients who experienced acute hemoglobinemia or hemoglobinuria following anti-D IGIV administration for ITP or secondary thrombocytopenia. Eleven of those patients also experienced clinically compromising anemia, transfusion with packed red blood cells, renal insufficiency, dialysis, or death. That review suggested that patients receiving anti-D IGIV be monitored for those and other potential complications of hemoglobinemia, particularly disseminated intravascular coagulation (DIC). Through November 30, 2004, the FDA received 6 reports of DIC associated with "acute hemolysis" (or similar terms), 5 of which involved fatalities. The attending or consulting physicians assessed that acute hemolysis or DIC caused or contributed to each death. This review presents the first case series of DIC associated with acute hemoglobinemia or hemoglobinuria following anti-D IGIV administration for ITP. The purpose of this review is to increase awareness among physicians and other health care professionals that DIC may be a rare but potentially severe complication of anti-D IGIV treatment. Increased awareness of DIC as a diagnostic possibility may enable prompt recognition and medical intervention in affected patients.- - - - - - - - - - ranking = 2keywords = physician (Clic here for more details about this article) |
3/8. Neonatal thrombocytopenic purpura: case reports and methodological approach.Six emblematic cases of neonatal thrombocytopenic purpura (NTP) due to different etiologic factors are described. Various methods for the detection of platelet and leukocyte antibodies are considered: platelet suspension and lymphocyte immunofluorescence tests (PSIFT and LIFT), solid-phase platelet antibody detection test (Capture-P), immune-enzymatic assay on platelet suspension (ELISA) and on solid-phase platelets (Capture-P-ELISA), lymphocytotoxicity (Lctx). Furthermore, chloroquine stripping of hla antigens and monoclonal antibody (MoAb) treatment of target platelets specific antigens are described. The technical characteristics and advantages of each method are discussed. The authors stress that a step-by-step investigation employing an integrated system that includes various methods can correctly supply the pathogenetic information necessary for the physician to undertake the best therapeutical approach.- - - - - - - - - - ranking = 1keywords = physician (Clic here for more details about this article) |
4/8. Autoimmune disorders complicating adolescent Hodgkin's disease.Four adolescents with Hodgkin's disease also developed autoimmune diseases. There were two idiopathic thrombocytopenic purpura (ITP), one polymyositis, and one scleroderma. The first two patients developed ITP in the absence of a spleen, and with their Hodgkin's disease in remission. The first patient with Hodgkin's disease has been continuously free of cancer for over five years. The second patient was a 17-year-old male whoe Hodgkin's disease recurred, but whose disease was in remission at the time the ITP occurred. The polymyositis occurred in an 18-year-old youth when he was in his initial remission for his Hodgkin's disease, but his disease subsequently recurred two years later. This youth presented with Coombs positive autoimmune hemolytic anemia. The polymyositis did not respond to therapy, and he is left with severe muscle wasting and weakness; however, the polymyositis is now quiescent. The scleroderma occurred in an 18-year-old female who had been continuously free of Hodgkin's disease for eight years. The scleroderma did not respond to drug therapy and she now has moderate skin changes, but remains in continuous remission of her Hodgkin's disease. Although there are a few reports of Hodgkin's disease and concurrent autoimmune disorders, physicians dealing with cancer in adolescents should be aware of this association.- - - - - - - - - - ranking = 1keywords = physician (Clic here for more details about this article) |
5/8. Acute idiopathic thrombocytopenic purpura.A 35-year-old man had hemorrhagic bullae of the buccal and sublingual mucosa as the first sign of acute idiopathic thrombocytopenic purpura. Twenty-four hours later the more typical cutaneous manifestations of petechiae of the lower extremities were present. Although it is unusual for the initial symptoms of this disease to be limited to the oral region, the physician and dentist should be keenly aware of the clinical appearance described in this article, as it may be the only clue of an underlying systemic disease.- - - - - - - - - - ranking = 1keywords = physician (Clic here for more details about this article) |
6/8. Leukocytoclastic vasculitis following staphylococcal protein a column immunoadsorption therapy. Two cases and a review of the literature.BACKGROUND: Protein A immunoadsorption is a novel therapy for the treatment of diseases mediated by pathogenic autoantibodies. This procedure consists of circulating patients' plasma through a column containing staphylococcal protein a, which binds to the Fc portion of IgG, enabling removal of IgG. Presently, protein A immunoadsorption is used in the treatment of idiopathic thrombocytopenic purpura, but may be more widely used as an immunomodulator in human immunodeficiency virus infection and metastatic carcinoma. OBSERVATIONS: We present two histologically documented cases of leukocytoclastic vasculitis in the setting of protein A immunoadsorption. This potentially severe adverse effect is probably more common than the literature reflects and should be recognized by physicians who are treating patients with protein A column pheresis. CONCLUSIONS: The pathogenesis of protein A therapy-associated leukocytoclastic vasculitis remains unclear. Further study of vasculitis in the setting of protein A column pheresis may lead to modifications of this therapy, resulting in fewer adverse effects. Protein A-associated leukocytoclastic vasculitis may serve as a useful model of the relation of immune complexes and vasculitis.- - - - - - - - - - ranking = 1keywords = physician (Clic here for more details about this article) |
7/8. Thrombocytopenic purpura. Importance of early diagnosis.Thrombotic thrombocytopenic purpura and hemolytic uremic syndrome are important diagnostic considerations for family physicians because therapy can be lifesaving. Treatment with plasmapheresis is usually successful. Therefore, early diagnosis is essential. A patient with microangiopathic hemolytic anemia is described. Diagnosis of Hodgkin's disease was confirmed after the initial episode. The case highlights the important role of family physicians in the diagnosis and treatment of uncommon hematologic syndromes.- - - - - - - - - - ranking = 2keywords = physician (Clic here for more details about this article) |
8/8. Management of isoimmune neonatal thrombocytopenia.Two infants, who presented at birth with isoimmune thrombocytopenic purpura, are the basis for this report. The problems confronting the physician in treating an affected infant, as well as in the management of subsequent pregnancies after an infant with isoimmunization has been delivered, are discussed. In view of the small but serious risk of intracranial hemorrhage during the birth process in these infants, delivery by cesarean section is advocated for all pregnancies known to be at risk i.e., after a previous infant has been shown to be affected.- - - - - - - - - - ranking = 1keywords = physician (Clic here for more details about this article) |