1/123. 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.- - - - - - - - - - ranking = 1keywords = hemolytic (Clic here for more details about this article) |
2/123. Molecular exclusion of haemoglobin SD disease by prenatal diagnosis.The most common Hb D variant, Haemoglobin D (Hb D) los angeles is caused by a glutamic acid to glutamine substitution at codon 121 of the beta globin gene. Although asymptomatic in the heterozygous form, inheritance together with an Hb S allele can result in a severe disease similar to sickle-cell anaemia that is referred to as Hb SD disease. prenatal diagnosis for Hb SD disease was requested by an at-risk couple of Irish/English descent. prenatal diagnosis was performed on dna isolated from chorionic villi at 12 weeks' gestation using dot blot and allele-specific oligonucleotide hybridization for the HbS mutation, and two independent approaches, restriction fragment analysis and ARMS (amplification refractory mutation system) for the detection of the Hb D los angeles mutation. The fetus was found to be heterozygous for the HbS mutation, but did not inherit the HbD mutation. Thus, a reliable and rapid prenatal diagnosis for the Hb SD disease can be achieved by molecular diagnosis.- - - - - - - - - - ranking = 2.4545236561154keywords = anaemia (Clic here for more details about this article) |
3/123. Large cerebral vessel disease in sickle cell anaemia.An 18 year old male with documented sickle cell disease was admitted to the hospital for the final time in coma. cerebral angiography revealed multiple stenotic lesions of the large cerebral vessels. The pathology of this large vessel involvement is demonstrated and the potential contribution of large as opposed to small cerebral vessel disease in the neurological manifestations of sickle cell anaemia is discussed.- - - - - - - - - - ranking = 12.272618280577keywords = anaemia (Clic here for more details about this article) |
4/123. Delayed hemolytic transfusion reaction presenting as a painful crisis in a patient with sickle cell anemia.CONTEXT: patients with sickle cell anemia (SCA) are frequently transfused with red blood cells (RBC). Recently we reported that the calculated risk of RBC alloimmunization per transfussed unit in Brazilian patients with SCA is 1.15%. We describe a delayed hemolytic transfusion reaction (DHTR) presenting as a painful crisis in a patient with SCA. CASE REPORT: A 35-year-old Brazilian female with homozygous SCA was admitted for a program of partial exchange transfusion prior to cholecystectomy. Her blood group was O RhD positive and no atypical RBC alloantibody was detected using the indirect antiglobulin technique. Pre-transfusional hemoglobin (Hb) was 8.7 g/dL and isovolumic partial exchange transfusion was performed using 4 units of ABO compatible packed RBC. Five days after the last transfusion she developed generalized joint pain and fever of 39 degrees C. Her Hb level dropped from 12.0 g/dL to 9.3 g/dL and the unconjugated bilirrubin level rose to 27 mmol/L. She was jaundiced and had hemoglobinuria. Hemoglobin electrophoresis showed 48.7% HbS, 46.6% HbA1, 2.7% HbA2, and 2.0% HbF. The patient's extended RBC phenotype was CDe, K-k , Kp(a-b ), Fy(a-b-), M N s , Le(a b-), Di(a-). An RBC alloantibody with specificity to the Rh system (anti-c, titer 1:16.384) was identified by the indirect antiglobulin test. The Rh phenotype of the RBC used in the last packed RBC transfusion was CcDEe. The patient was discharged, asymptomatic, 7 days after admission.- - - - - - - - - - ranking = 0.71428571428571keywords = hemolytic (Clic here for more details about this article) |
5/123. Sickle cell disease and nitrous oxide-induced neuropathy.We report three cases of peripheral neuropathy in patients with sickle cell disease. All had a history of frequent painful crises and regular attendance at our Accident and Emergency department where nitrous oxide analgesia was administered for prolonged periods. All three patients (one male and two females) presented with difficulty in walking associated with paraesthesiae, and neurological examination revealed signs compatible with a peripheral sensorimotor neuropathy, later confirmed by nerve conduction studies. serum vitamin B12 levels were mildly reduced in two patients and very low in one patient (< 10 ng/l). Haemoglobin levels in all the patients were unchanged compared with their steady-state levels but one had developed a macrocytosis (103 fl). Schilling tests were normal in two patients, and two patients had negative gastric parietal antibodies. All three patients were given intramuscular vitamin B12 in addition to avoiding further exposure to nitrous oxide, and their neurological symptoms improved completely. As nitrous oxide is known to cause a neuropathy similar to that seen in pernicious anaemia, we postulate that nitrous oxide analgesia combined with low B12 levels was the cause of the marked neuropathy in these patients. As a result of our observations and the probable association, we now do not use nitrous oxide analgesia in the management of patients with sickle cell disease.- - - - - - - - - - ranking = 2.4545236561154keywords = anaemia (Clic here for more details about this article) |
6/123. Hereditary congenital cataracts associated with sickle cell anaemia in a Nigerian family.Pedigrees of hereditary disorders are rarely reported from sub-Saharan countries because of difficulties inherent in obtaining reliable family histories, case tracing and follow-up. This report relates to a four-generation family in northern nigeria with dominantly inherited cataracts associated with recessively inherited sickle cell anaemia. It highlights the need for genetic counselling services in clinical practice in the region.- - - - - - - - - - ranking = 12.272618280577keywords = anaemia (Clic here for more details about this article) |
7/123. An acute hemolytic transfusion reaction due to anti-IH in a patient with sickle cell disease.BACKGROUND: A hemolytic transfusion reaction (HTR) due to anti-IH is reported in a patient with sickle cell disease (SCD). CASE REPORT: An 18-year-old woman with SCD and a complete phenotype on file had been identified as group B-positive with negative antibody-screening tests and had received 1 unit of packed RBCs. Ten days later, she was readmitted in painful crisis with a Hb of 4.2 g per dL. Antibody-screening tests and panel cells were positive at all test phases with a negative autocontrol, which suggested alloantibodies. Phenotypically matched group O RBCs were issued emergently. After the transfusion of 100 mL, the patient had an HTR with chills, fever, and tachycardia and laboratory findings of hemoglobinemia, hemoglobinuria, and negative DATs. A high-titer, IgM anti-IH with a high thermal amplitude (reactive with group O, but not group B RBCs at 37 degrees C) was identified. Autologous RBCs appeared to have normal I antigen expression, but less H antigen than pooled group B RBCs. She was given group B RBCs, uneventfully, by use of a blood warmer. CONCLUSIONS: This is a rare case of anti-IH as the cause of a HTR, as a serologic problem that may be seen in SCD, and as an autoantibody that may mimic an alloantibody. Ironically, this HTR resulted from the effort to provide phenotypically matched RBCs, which necessitated the selection of group O RBCs.- - - - - - - - - - ranking = 0.71428571428571keywords = hemolytic (Clic here for more details about this article) |
8/123. A 'made in one piece' skeleton in a 22-year-old man suffering from sickle cell anaemia.A 22-year-old African male with known sickle cell anaemia was referred by a Congolese medical centre with a request to improve his poor physical condition. He was unable to walk, stand or sit because his large joints and his spine were either ankylosed or very rigid. Radiographs showed joint fusion from the third to the fifth cervical vertebrae, of both hips, of the left knee, and a bilateral osteonecrosis of the humeral head. There was no scintigraphic evidence for an active osteomyelitis (99mTc-MDP (methyldiphosphonate) bone scan, Tc monoclonal antigranulocyte scan and 99mTc sulphur colloid scan). To improve his mobility the right femoral head was resected in June 1997; 14 days later the left femoral head was resected. Four months after the resection of the right hip, a right uncemented total hip prosthesis was implanted on this side. One month later the same type of hip arthroplasty was performed on the left side. During the postoperative rehabilitation period the patient regained autonomy. We have found no previous reports of such severe and multiple joint complications in a single patient suffering from sickle cell anaemia.- - - - - - - - - - ranking = 14.727141936692keywords = anaemia (Clic here for more details about this article) |
9/123. methylene blue-induced hyperbilirubinemia in neonatal glucose-6-phosphate dehydrogenase (G6PD) deficiency.methylene blue continues to be used in the gravid female. We report three premature neonates exposed to methylene blue that experienced severe hemolytic reactions requiring exchange transfusions. Two neonates were subsequently diagnosed with G6PD deficiency. Continued caution is warranted prior to the use of methylene blue in the gravid female.- - - - - - - - - - ranking = 0.14285714285714keywords = hemolytic (Clic here for more details about this article) |
10/123. Aplastic crisis caused by parvovirus B19 in an adult patient with sickle-cell disease.We describe a case of aplastic crisis caused by parvovirus B19 in an adult sickle-cell patient presenting with paleness, tiredness, fainting and dyspnea. The absence of reticulocytes lead to the diagnosis. Anti-B19 IgM and IgG were detected. Reticulocytopenia in patients with hereditary hemolytic anemia suggests B19 infection.- - - - - - - - - - ranking = 0.14285714285714keywords = hemolytic (Clic here for more details about this article) |
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