An unusual case of autoimmune hemolytic anemia with reticulocytopenia, erythroid dysplasia, and an IgG2 autoanti-U

BACKGROUND: Autoantibodies with anti-U specificity, usually in combination with autoantibodies of other specificities, have occasionally been identified in association with autoimmune hemolytic anemia. A case of life-threatening autoimmune hemolytic anemia, characterized by several atypical features, including apparent intravascular hemolysis associated with an IgG2 anti-U, reticulocytopenia, and bone marrow dyserythropoiesis is described. CASE REPORT: A 36-year-old man with a severe case of acute-onset autoimmune hemolytic anemia was admitted to another hospital; he had a hematocrit of 15 percent, elevated bilirubin and lactate dehydrogenase, and positive direct and indirect antiglobulin tests. He received 7 units of incompatible red cells without improvement in hematocrit, and he was transferred to University Hospitals of Cleveland (OH). He was jaundiced and became syncopal in the sitting position. His serum was reddish pink; he had a hematocrit of 11.8 percent and a reticulocyte count of 2.5 percent. No spherocytes were observed in the peripheral blood smear. Shortly after admission, the hematocrit fell to 6.9 percent. He was given 3 units of “least-incompatible” red cells and was started on prednisone, with little improvement. An IgG2 autoanti-U was detected in his serum. Seven units of U- red cells were transfused over the next 4 days. The hematocrit improved to 23 percent and continued to rise without further transfusion. A bone marrow examination, initially revealing erythroid hyperplasia accompanied by dyserythropoiesis, became morphologically normal. Drug studies failed to show evidence of drug-related hemolysis. He remains well 2 years after discharge without evidence of recurrent hemolysis. CONCLUSION: Severe life-threatening autoimmune hemolytic anemia, in this instance induced by an autoanti-U, may be associated with IgG2 autoantibody and characterized by apparent intravascular hemolysis and bone marrow dyserythropoiesis. Early treatment with U- blood, in addition to steroids, may be beneficial.     

Fluconazole resistant opportunistic oro-pharyngeal candida and non-candida yeast-like isolates from HIV infected patients attending ARV clinics in Lagos, Nigeria

Background

Oro-Pharyngeal Candidiasis (OPC) continues to be considered the most common opportunistic fungal disease in HIV/AIDS patients globally. Azole antifungal agent has become important in the treatment of mucosal candidiasis in HIV patients. Presently, antifungal drug resistance is fast becoming a major problem particularly with the immune depleted population.

Objectives

This study was designed to investigate the: existence of OPC, species distribution fluconazole susceptibility profile of yeast cells isolated from oral specimens of HIV/AIDS patients from Lagos Nigeria, between Oct. 2004 and June, 2005.

Methodology

The venous blood samples were screened for HIV antibodies using the Cappillus HIV I and II test kit (Trinity Biotech Plc UK), and Genie II HIV I and II EIA kit (Bio-Rad France). The positive results were subsequently confirmed at the laboratory attached to each of the clinics, using the Nigerian Federal Ministry of Health approved algorithm. The samples from 213 (108 females and 105 males) HIV positive patients were plated onto SD agar. The isolates were identified by morphotyping, microscopy and speciated using germ tube test and battery of biochemical sugar fermentation and assimilation tests. Fluconazole agar diffusion susceptibility testing was carried out on each isolates.

Results

Seventy-four (34.7%) isolates were recovered including one person with double isolates. Only 70(94.6%) of the isolates could be adequately speciated. Candida albicans 30 (40.5%) was the most frequently isolated species, the rest were non-albicans species, with the frequency of C. tropicalis › C. Krusei › C. glabrata and C. neoformans for species for species having up to 4 isolates. Four (30.8%) out of 13 isolates of C. tropicalis showed germ tube formation. While one C. albicans was germ-tube negative. Out of the 74 isolates tested for fluconazole sensitivity, 58(78.4%) were sensitive, MIC d″ 8µg/ml, 9(12.1%) were susceptible Dose Dependant (SDD), MIC 16–32 µg/ml and 7(9.5%) were resistant, MICs e″ 64µg/ml. Among the C. albicans isolates, 26(86.7%) were sensitive to fluconazole. The rank of susceptibility was C. albicans > C. tropicalis > C. Krusei for the most prevalent species.

Conclusion

We conclude that fluconazole resistant strains of oro-pharyngeal yeast-like cells exist in about 9.5% of HIV/AIDS patients with the above stated species distribution. We therefore, highlight the need for routine antifungal susceptibility testing on HIV patients with cases of initial or repeat episodes of OPC.     

Role of interventional radiography in the treatment of the hepato-pancreato-duodenal area,complicated by obstructive jaundice

We assessed the role of interventional radiological procedures used in the treatment of patients with hepatopancreatobiliary cancer complicated by obstructive jaundice. Between 1990 and 2000, 71 patients underwent percutaneous transhepatic biliary drainage (PTBD): external drainage--18 (group A); external-internal drainage or stenting (group B), and external-internal drainage or stenting plus chemoinfusion or chemoembolization of the hepatic artery--15 (group C). Mean survival (M(SD) calculated for patients who died was 2.1(0.5 mo for group A; (pb,c(0.01), 7.9(6.7 mo (group B), and 16.6(14.8 mo for group C (NS with B). The actual one-year survival was 10, 25, and 45%, respectively. External-internal PTBD and stenting are safe effective palliative procedures for patients with malignant obstructive jaundice. Survival in patients with hepatopancreatobiliary cancer doubled after chemoinfusion or chemoembolization, without grave complications or lethality.     

The incidence of painful crisis in homozygous sickle cell disease: correlation with red cell deformability

To determine whether the vasoocclusive severity of homozygous sickle cell (SS) disease is influenced by cellular dehydration, we correlated the incidence of painful crisis with steady-state measurements of red cell hydration. Sixteen children with SS disease were followed for 3.3 to 8 years (mean, 6.8 years), and a single crisis rate was calculated for each patient. At the time of well visits, cellular hydration was assessed by measuring cell deformability, the percentage of red cells with a density greater than or equal to 1.1056 g/mL, and the percentage of irreversibly sickled cells (ISC). The incidence of painful crisis showed a strong positive correlation with Omax, a deformability measurement reflecting cellular hydration (r = .84, P less than .002), and with hemoglobin concentration (r = .59, P = .04). That is, higher crisis rates were observed in patients with less dehydrated, more deformable red cells and also in patients with higher hemoglobin concentrations. Furthermore, cell deformability and hemoglobin concentration were independent predictors of the incidence of painful crisis, which is consistent with separate effects of these two red cells parameters on vasoocclusive severity.     

Liver transplantation for hepatic cirrhosis in cystic fibrosis

Five children with cystic fibrosis complicated by hepatic cirrhosis received liver grafts. They all had portal hypertension with varices and three had variceal bleeding; respiratory function was only moderately impaired, but four were colonised with pseudomonas and one with aspergillus. Liver transplantation was well tolerated and there was no increase in respiratory or other early postoperative complications. Four of the children were fully well from 14 to 35 months after transplantation; the most recently transplanted had problems from a biliary stricture. In spite of the need for immunosuppression there was no increase in infection and respiratory function improved or remained stable. Once the children were stabilised after transplantation their nutrition and general health were greatly improved.     

High dose vitamin A supplementation in the course of pneumonia in Vietnamese children

We carried out a randomized, placebo-controlled, double-blinded trial to evaluate the effect on morbidity of high dose oral vitamin A, given on hospital admission to 592 children aged 1–59 months with moderate and severe pneumonia. Severely underweight children were not included, but 45% were moderately underweight. The vitamin A and placebo groups were comparable in baseline characteristics. Four patients died. Among all of the surviving children, no differences were found regarding mean time for normalization of fever, respiratory rate and time of hospitalization. Stratification for moderate malnutrition, degree of pneumonia, age and sex revealed moderately malnourished vitamin A-supplemented children to have a shorter time of hospitalization ( p = 0. 04), due to an effect in females aged > 12 months ( p = 0. 02) and females with very severe pneumonia ( p = 0. 048). This study indicates that, in developing countries like Vietnam, supplementation with vitamin A in children with pneumonia could shorten the recovery rate in the ones that are undernourished, especially females > 1 y old.     

Ooplasmic injection of elongating spermatids for the treatment of non- obstructive azoospermia

We applied the technique of ooplasmic elongating spermatid injection to the treatment of non-obstructive azoospermia. Mature oocytes were injected with elongating spermatids isolated from testicular biopsy material obtained from 13 non-obstructed azoospermic men. Seventy-three oocytes were successfully injected with elongating spermatids and were then cultured for 36 h. At 13 h post-injection 68 oocytes were found to be activated and 52 of them were fertilized. Forty-one 2- to 4-cell stage embryos developed from normally fertilized oocytes were transferred. At least two embryos were transferred to each female partner. Two pregnancies were achieved. Elongating spermatid injection may have a role in the treatment of non-obstructive azoospermia.      

Alpha globin gene number: population and restriction endonuclease studies

Restriction endonuclease analysis was used to test a proposed genetic model using alpha-globin gene number to account for the observed distributions of the proportions of hemoglobin (Hb) S in sickle cell trait. In a subsample of specimens collected during a population survey in India, these studies confirmed that the postulated genotype was present in 22 of the 23 individuals examined. In the study population, the number of alpha-globin genes explains about 90% of the variance in the proportion of HbS in sickle cell trait (r2 = 0.895, p less than 10(- 10)).