1/528. Anti-s antibody-associated delayed hemolytic transfusion reaction in patients with sickle cell anemia. PURPOSE: signs and symptoms of delayed hemolytic transfusion reaction (DHTR) may resemble those of vaso-occlusive crises in patients with sickle cell anemia (SCA). The diagnosis of DHTR therefore presents a challenge to the clinician when treating such patients. The current study describes a patient with SCA and DHTR secondary to red cell anti-s antibody, manifesting as painful extremeties, severe hemolytic anemia, and acute oliguric renal failure. patients AND methods: A 17-year-old patient with homozygous hemoglobin S presented 8 days after partial exchange transfusions with severe anemia and signs and symptoms resembling vaso-occlusive crisis. Clinical course was complicated by intravascular hemolysis and acute renal failure. RESULTS: Anti-s antibody was detected in the eluate. diagnosis of DHTR was made. Treatment included single volume whole blood exchange transfusion and continuous veno-venous hemofiltration with dialysis. CONCLUSIONS: The possibility of DHTR should be considered in a patient with SCA with hemolytic anemia. Acute renal failure is a rare complication of anti-s antibody-associated DHTR. Such reactions can be successfully managed with exchange transfusion and continuous hemofiltration with dialysis. ( info) |
2/528. Does coagulation have a causative role in eclampsia? Alternations in the coagulation mechanism were looked for in a population of eclamptic women, most of when were young, nulliparous, and without evidence of chronic vascular disease, and all of whom survived. thrombocytopenia was identified in 29% of these women. A prolonged plasma thrombin time was demonstrated in 51% yet elevated fibrinogen-fibrin degradation products in serum were uncommon, as was fibrin monomer in plasma. Overt microangiopathic hemolysis was rare. It is concluded that disseminated intravascular coagulation, when it does occur in eclampsia, is the consequence of the disease rather than the cause. Moreover, endothelial damage, rather contents, probably initiates the thrombocytopenia and other coagulation changes. ( info) |
3/528. Complement sensitivity of erythrocytes in a patient with inherited complete deficiency of CD59 or with the Inab phenotype. We investigated the complement sensitivity of erythrocytes from three patients, one with inherited complete deficiency of CD59, one with the Inab phenotype, and one with paroxysmal nocturnal haemoglobinuria (PNH). The complement lysis sensitivity units on the erythrocytes were 11.7, 4.6, and 47.6 for inherited CD59 deficiency, Inab phenotype, and PNH, respectively. Two-colour flow cytometric analysis showed that the erythrocytes from the three patients consisted of a single population negative for CD59, negative for decay accelerating factor (DAF), and negative for both proteins, respectively. In addition, only the Inab phenotype patient had no haemolysis in vivo. These facts suggest that CD59 deficiency plays a more important role than DAF deficiency in complement-mediated haemolysis in vitro and in vivo, and that deficiency of both proteins, but not CD59 or DAF alone, causes complement sensitivity corresponding to that of PNH III erythrocytes in vitro. ( info) |
4/528. hemolysis associated with 25% human albumin diluted with sterile water--united states, 1994-1998. Since 1994, a shortage of 5% human albumin, a product used off-label during therapeutic plasma exchange (TPE), has existed in the united states. Because of this shortage, hospital pharmacists may prepare 5% solution of human albumin by diluting 25% human albumin with 0.9% NaCl or, when sodium load is a concern, 5% dextrose. However, if sterile water alone is used as the diluent, the osmolarity (tonicity) of the albumin solution is reduced and may cause hemolysis in recipients. This report describes two of 10 episodes of hemolysis (one fatal) among persons who received 25% human albumin diluted with sterile water and emphasizes that sterile water alone should not be used to dilute albumin. ( info) |
5/528. Nonhemolytic IgG anti-IT. Nonhemolytic, IgG, anti-IT autoantibodies were found in the sera of three Caucasian patients, none of whom had Hodgkin's disease. Each antibody reacted by indirect antiglobulin test. Two of the three also reacted in albumin at 37 C, and one of these was moderately enhanced by papain. As judged by transfusion responses, reticulocyte counts, hematocrit stability, and one hour 51Cr red blood cell survivals, none of the antibodies were considered to be hemolytic. When tested with anti-IgG serum, cells from all three had a positive direct antiglobulin test. Anti-IT antibody was eluted from their cells. Ii status of the patients' cells differed from normal in each case. These data suggest greater variation in the disease association, serologic reactivity, and clinical significance, of anti-IT than has been evident from previous studies. ( info) |
6/528. Lessons to be learned: a case study approach: prolonged methaemoglobinaemia due to inadvertent dapsone poisoning; treatment with methylene blue and exchange transfusion. The authors present a case of methaemoglobinaemia of acute onset, with an unusually protracted course. The long persistence of this disorder led to a search for the cause which was eventually traced to medication with dapsone. The latter was found to be inappropriately being taken by the patient instead of an antispasmodic that had been prescribed for a spinal condition; this was because the tablets had been incorrectly labelled and dispensed in a pharmacy. The patient took increasing doses of the presumed 'antispasmodic' tablets as they seemed to lack clinical effect, thus further exacerbating the toxic consequences. Moreover, the patient brought his wrongly labelled tablets into hospital and was allowed to use them there, contrary to normal hospital policy. As treatment for the methaemoglobinaemia both bolus and continuous infusions of methylene blue were used, which probably contributed to the severe haemolysis which followed. Furthermore, the development of a rare side effect of dapsone toxicity, namely that of a sensorimotor neuropathy, is reported. ( info) |
7/528. hemosiderin deposition on the renal cortex by mechanical hemolysis due to malfunctioning prosthetic cardiac valve: report of MR findings in two cases. We present two cases of mechanical hemolysis due to malfunctioning prosthetic cardiac valves in which MRI was characteristic. The signal intensity of the renal cortex was much lower than that of the medulla on T2-weighted images due to deposition of hemosiderin in proximal convoluted tubules by intravascular hemolysis. These MR findings are identical to those in paroxysmal nocturnal hemoglobinuria. ( info) |
8/528. Anaesthetic management of a patient with erythropoietic protoporphyria for ventricular septal defect closure. Erythropoietic protoporphyria (EPP) is due to a deficiency in ferrochelatase required for haem synthesis. We describe the anaesthetic management of a seven-year-old with EPP undergoing closure of a haemodynamically significant ventricular septal defect. Photosensitivity in EPP patients is triggered at wavelengths near 400 nm and light-excited porphyrins generate free radicals and singlet oxygen that lead to erythrocyte deformity and haemolysis. Stimuli that could trigger a porphyric crisis during anaesthesia and surgery were reduced by avoiding exposure to the sensitive 400 nm spectrum and using light sources covered with yellow acrylate filters in the operating room. Anaesthetic agents not previously associated with porphyric crisis were chosen. Whole blood priming of the extracorporeal circuit was performed to ensure adequate haemoglobin concentrations during the perioperative period. ( info) |
| Acute bacterial endocarditis in the absence of underlying heart disease is rare. We report the occurrence of endocarditis caused by group A beta-hemolytic streptococcus (GABHS), following varicella, in a 5-month-old child without heart disease. In addition to this child, seven other children with endocarditis caused by GABHS have been reported since 1966, six of whom did not have preexisting heart disease. In one of these children, GABHS endocarditis was preceded by varicella. These cases indicate that GABHS is capable of causing endocarditis in the absence of heart disease, and they provide further evidence that varicella is an important risk factor for invasive GABHS infections. ( info) |
10/528. Spontaneous resolution of hemolysis after partial coil occlusion of ductus arteriosus. hemolysis occurred in a 2-year-old girl following partial coil occlusion of a moderate persistent ductus arteriosus. Spontaneous resolution of hemolysis occurred 2 months later, even though a small residual shunt persisted. ( info) |