Maternal allo-antibody production is stimulated when fetal red blood cells are positive for an antigen absent on the mother's red cells. The maternal IgG antibodies produced will pass through the placenta and attack fetal red cells carrying the corresponding antigen. Allo-immune hemolytic disease of the fetus and newborn caused by anti-E rarely occurs.We report two cases of anti-E hemolytic diseases in neonates. One of the neonates had severe hemolysis presenting with severe anemia, thrombocytopenia, and conjugated hyperbilirubinemia, while the other had moderate anemia and unconjugated hyperbilrubinemia. Although both the neonates were treated by phototherapy and intravenous immunoglobulin, one of them received double volume exchange transfusion.There appeared to be an increase in the occurrence of hemolytic disease of the fetus and newborn caused by Rh antibodies other than anti-D. In this case report, both patients presented with anemia and hyperbilirubinemia but were successfully treated, with a favorable outcome.

Acinetobacter infections are fast emerging as a major nosocomial threat across the globe. With a predilection for blood stream infections in critically ill, immunocompromised patients, their presence is now being felt in febrile neutropenics with underlying malignancies and marrow failure. We aimed through this study to ascertain the current circulating pathogens and levels of antimicrobial resistance in blood stream infections in febrile neutropenia patients, with specific emphasis on elucidating acinetobacter and pseudomonas infections. Clinical and laboratory records of all consecutive neutropenic patients with underlying haematological malignancies and marrow failure, admitted to our AIIMS, New Delhi from April 2009 to March 2010 were analysed for blood stream infections, pathogen profiles and antimicrobial resistance. All clinical and microbiological variables were statistically analysed to elucidate potential risk factors, infection patterns and drug resistance trends. Of the 1,165 blood cultures investigated, 105 episodes of blood stream infections were microbiologically confirmed in febrile neutropenia patients. Gram-negative infections (n = 78, 72.9%) dominated with acinetobacter spp (n = 20, 18.7%) emerging as the most common pathogen. Acinetobacter and pseudomonas together were responsible for 42.9% of all blood stream infections. Both acinetobacter and pseudomonas displayed very high resistance to all five major classes of antibiotics, including multidrug resistance (90.0% and 76.9%) and ESBL production (90.0% and 84.6%), respectively. Comparison of infection patterns and resistance levels with reports over the past decade from this centre and other centres across the globe, revealed a striking increase in multidrug resistant acinetobacter blood stream infections in these patients. Multidrug resistant acinetobacter Infections are a fast emerging threat in febrile neutropenia patients and at this centre in general. Similar early trends from some Indian centres and neighbouring developing countries suggest grave concern. These emerging circulating pathogens and drug resistance patterns demand to systematically evaluate antibiotic and hospital infection policies and to sensitise all clinicians to curb this pathogen capable of rapid nosocomial spread.

Atypical hemolytic uremic syndrome (aHUS), although rare, is the most common cause of acute renal failure (ARF) in children and has poor prognosis. We present a single centre experience of aHUS.Thirty six children (29 males, 7 females), with mean age 7.9 years, presented with ARF, 2 children also had tonic-clonic type convulsions. Their hematology examination revealed hemolytic anemia with serum create-nine (SCr) of 5.54 mg/dL. Acute HUS was observed in 75%, acute-on-chronic HUS in 19.4%, and patchy cortical necrosis (PCN) in 5.6% biopsies. A mean of 5.4 plasma exchanges (PE) were carried out. Supportive management of anti-hypertensives and prednisone was also given. Recovery end points were establishment of urine output and improvement of SCr and hematological profile.Hematology and renal function profile improved variably in all children, 5.6% died, relapse was observed in 80.5% over a mean of 70 days; 13.9% children are doing well over a mean follow-up of 268.8 days. Thus, poor prognosis was observed in 86.1% children. Children with acute or chronic HUS and PCN did not recover. Six children who recovered had acute HUS.aHUS in Indian children occurs at an older age of around 8 years and chronic/irreversible changes on histopathology examination are harbingers of poor prognosis. PE is life-saving; however, further research for developing strategies to improve long-term survival is needed.

INTRODUCTION:

Recently, a consensus report for microscopic schistocyte determination was prepared by International Council for Standardization in Hematology (ICSH). ICSH focused on diagnosis of thrombocytopenic purpura (TTP)/hemolytic uremic syndrome (HUS). We aimed to reanalyze schistocytes according to ICSH recommendations, to study diseases other than TTP/HUS related to the schistocytes, and to compare the percentage of schistocytes among the various diseases.

METHODS:

We retrieved all reported cases of peripheral blood (PB) smear in a single institution during 6 years. Schistocytes on 282 PB smears showing previous peripheral schistocytes and hemoglobin ≤10 g/dL were recounted according to ICSH recommendations.

RESULTS:

The schistocytes were frequently observed in patients with microangiopathic hemolytic anemia (MAHA), metastatic carcinoma, sepsis, chronic renal failure, preterm infant, and infection. Only two among 34 patients categorized as MAHA were diagnosed as TTP/HUS. Schistocytes were observed with other morphological changes in 169 of 170 cases with schistocyte ≤1% and in 102 of 112 with schistocyte >1%. The median schistocyte percentages of patients with hematologic malignancy, megaloblastic anemia, acute renal failure, and preterm infant were 1.20%, 1.30%, 1.35%, and 1.70%, respectively.

CONCLUSION:

Schistocytes were observed above 1% in many diseases other than TTP /HUS. Therefore, it is important to understand that schistocytes could be seen in various diseases, and in these cases, schistocytes were usually detected together with other red blood cell morphologic changes. These data support ICSH recommendation that a schistocyte count should be considered clinically meaningful if schistocytes represent the main morphological abnormality in the PB smear.

Historically, many children and adolescents with sickle cell disease (SCD) were underweight. Treatment advances like hydroxyurea have been associated with improved growth. We hypothesized that increased hemoglobin (Hb) levels would be associated with increased weight status of children with SCD.Investigators at 6 institutions conducted a retrospective chart review of all patients aged 2 to 19 years of age for the calendar years 2007-2009. Height, weight, baseline Hb levels, demographic information, and select comorbidities were recorded from the most recent clinic visit. Overweight and obesity were defined as ≥85th and ≥95th BMI percentiles for age and gender, respectively, and underweight was defined as <5th BMI percentile.Data were collected on 675 children and adolescents in 3 New England states. In this sample, 22.4% were overweight or obese, whereas only 6.7% were underweight. Overweight or obese status was associated with sickle genotypes other than Hb SS or Hb Sβ(0) disease, and were associated with higher baseline Hb levels. Underweight individuals were more likely to be male, older, and have had at least 1 SCD-related complication. After adjusting for demographic factors, any SCD-related complication, SCD-directed treatments, and obesity-related conditions, there was a 36% increased odds of overweight/obesity for each 1 g/dL increase in baseline Hb levels.Nearly one-quarter of children and adolescents with SCD in New England are overweight or obese. Longitudinal studies are needed to determine the impact of elevated BMI on the morbidity and mortality of both children and adults with SCD.

Accessibility to iron chelators including deferoxamine and deferasirox remains obscured in many developing countries. To provide an alternative, the government pharmaceutical organization of Thailand (GPO) manufactured deferiprone which has similar bioequivalent to the standard product. Seventy-three pediatric patients with severe β thalassemias, age range 3.2-19 years, were recruited to a 1-year multicenter prospective, single arm, open label, dose escalating Phase III study of deferiprone to determine its clinical efficacy and safety. Sixty-four patients (87.6%) completed the study with good compliance (>94%). Average deferiprone dose was 79.1±4.3 mg/kg/day. Overall, mean serum ferritin (SF) levels at 1 year were not significantly changed from baseline. However, 45% of patients (response group) had SF reduced >15% from baseline at 1 year with a median reduction of 1,065 ng ml(-1) . Baseline SF was the major factor that predicts clinical efficacy; patients with baseline SF>3,500 ng ml(-1) had the most significant fall of SF at 1 year. A subgroup analysis by MRI-T2* confirmed that the response group had higher baseline liver iron and deferiprone could significantly reduce liver iron overload and normalize levels of ALT at 1 year. Although, gastrointestinal irritation (20.5%) was the most common drug-related adverse events (AEs) followed by transaminitis (16.4%) and neutropenia (6.8%), all patients were well tolerated. There was no mortality and agranulocytosis found in this trial. Monotherapy of deferiprone with appropriate dose adjustment and monitoring for adverse events appeared to be an effective chelation therapy in some patients with good compliance and acceptable safety profiles.

Rangelia vitalii is a tick-transmitted piroplasm that causes both hemolytic and hemorrhagic disease in dogs in Brazil.The aim of this study was to evaluate the response of the bone marrow in dogs experimentally infected with R vitalii during the acute stage of the disease.For this study, 2 groups of a total of 12 young dogs were used. Group A was composed of healthy dogs (n = 5), and group B consisted of animals infected with R vitalii (n = 7). Blood samples were collected on days 0, 10, 20, and 30 post-inoculation and stored in EDTA tubes for a full hematology profile, including a reticulocyte count. On days 10 and 20, bone marrow samples were collected, stained, and examined.In infected dogs anemia was identified on days 10 and 20 post-inoculation (P < .01), and on day 20 reticulocytosis was present. Infected dogs had leukopenia due to neutropenia and eosinopenia, along with lymphocytosis and monocytosis, when compared with control animals. In bone marrow, the myeloid:erythroid ratio was significantly decreased (P < .05) in infected dogs due to increased erythroid precursors.Dogs experimentally infected with R vitalii develop regenerative extravascular hemolytic anemia accompanied by erythroid hyperplasia in the bone marrow. During the acute phase of the disease, leukopenia due to neutropenia and eosinopenia suggests intense tissue recruitment of these cells in response to the endothelial damage caused by this parasite.

Alpha thalassaemia is a common monogenic disorder found in most parts of the world but the severe form is restricted in its distribution while the mild deletional form of alpha- thalassaemia has a wider geographical distribution.One hundred and fifty one Nigerians which included 29 patients with unexplained recurrent haemolytic anaemia had their a-thalassaemia status determined by gap PCR after being screened using red cell indices.Only the -alpha(3.7) deletion occurs among Nigerians with a gene frequency of 0.21. Forty-two per cent of the study population are heterozygotes, 9% are homozygotes while 49% are normal for this deletion. The mean haematocrit for the study population is 37.5%: homozygote, heterozygotes and normal subjects had a mean haematocrit of 37.3%, 37.5% and 37.7% respectively. The mean mean corpuscular haemoglobin (MCH) and haematocrit are significantly lower for patients than controls (24 +/- 2.9 pg vs. 26.6 +/- 2.5 pg (p = .000), 29% vs. 40% (p = .000) respectively). Forty three percent of both patients and controls had MCH of less than 27 pg but only 10% of patients had MCH that is greater than 27pg. Homozygote patients had a higher mean haematocrit than patients who are normal for the deletion (34.7% vs. 25.4%, p = 0.06) but homozygote patients had a lower mean haematocrit than homozygote controls (34.7% vs. 38.5%, p = 0.4).This would suggest that alpha-thalassaemia is not responsible for the recurrent haemolytic anaemia observed in these patients and that the high prevalence of microcytosis among the normal populace needs further exploration.

Beta-thalassaemia is a congenital disorder caused by point mutations in a haemoglobin beta-globin chain. The heterozygous form produces microcytosis and normal iron levels, however, haemoglobin electrophoresis shows elevated amounts of haemoglobin A2 and eventually foetal haemoglobin F as well.Between 2005-2011, in three centres in Slovakia, carriers of beta-thalassaemic genes or other haemoglobinopathies were searched for. Diagnosis was performed by haematologists whereby the family history was evaluated, together with the overall clinical condition, blood count and blood smear, iron parameters, haemolysis and haemoglobin electrophoresis testing. A proportion of patients was examined by molecular genetic methods.A clinical suspicion of the heterozygous form of beta-thalassaemia was documented in 402 patients (21.9%) out of a total of 1,834 examinations. From these patients, 87 underwent molecular genetic testing and mutations of beta globin genes were identified in 70 of them, where the most frequent mutations were IVS 2.1 (28.5%), IVS 1.110 (25.6%) and IVS 1.1 (11.3%). Evidence of haemoglobin S (sickle cell anaemia) was also notable in one case (patient of African origin). Unusually high levels of haemoglobin F (6-21%) were found in 23 adult subjects.The study showed that there is a higher number of heterozygotes for beta-thalassaemia and rarely haemoglobinopathies. It is necessary to continue in search of pathological gene carriers in Slovakia.