Recent advances in prenatal diagnosis and multidisciplinary management has improved the reproductive outlook for carriers of hemophilia and their offspring. Pre-pregnancy planning allows the woman and her partner to explore reproductive options and prepares the family for the potential delivery of an affected male infant. Non-invasive methods of prenatal diagnosis are advancing, with the aim of providing a definitive test for hemophilia. Carriers of hemophilia have an increased risk of primary and secondary postpartum hemorrhage. The affected male offspring is potentially at risk of cranial bleeding during delivery. A multidisciplinary approach provides advanced planning for the optimum mode of delivery and provision of appropriate haemostatic cover to reduce the risk of bleeding complications. This review summarises current recommendations for pregnancy management in carriers of hemophilia, from the initial pre-pregnancy counseling through to delivery and care of the neonate.

Background: The clinical manifestations of vaso-occlusion result from a dynamic combinations of abnormalities in hemoglobin (Hb) structure and functions, red blood cell membrane integrity, erythrocyte density, endothelial activation, microvascular tone, inflammatory mediators and coagulation factors. Objective: The objective of the following study is to determine the changes in plasma concentration of fibrinolytic proteins among sickle cell anemia (SCA) patients in two clinical states; steady state and vaso-occlusive crises (VOC) and compare with HbAA controls and also to determine if any, the clinical relevance of these proteins in evaluating these patients in the different clinical state. Materials and Methods: A total of 25 (14M: 11F) HbSS subjects in VOC, 24 (13M: 11F) HbSS subjects in a steady state and 30 (17M: 13F) healthy HbAA volunteers matched for age and sex with the subjects were recruited for the study. Hematological parameters, i.e., full blood count, fibrinolytic proteins concentration including plasma concentration of D-dimer, plasminogen, fibrinogen (FBG), tissue plasminogen activator (tPA) and fibrinopetide-A were determined. Result: Plasma fibrinopeptide A (FPA) concentration of 680.99 ΁ 411.37 ng/ml for SCA subjects in VOC and 449.67 ΁ 310.01 ng/ml in steady state subjects were significantly increased (P < 0.001) compared with 163.52 ΁ 86.26 ng/ml for HbAA controls. However, there were no statistically significant changes in the plasma concentrations of D-dimer, FBG, plasminogen and tPA among the subjects in any of the clinical states (steady state and VOC) and controls studied. Conclusion: The significant increase in FPA concentration observed among subjects compared to controls may have been confounded by hyperleucocytosis especially polymorphonuclear leucocytes commonly associated with SCA. Thus, the clinical relevance of these plasma proteins in evaluating these patients in either of the clinical states of this disorder is doubtful.

Context: Manual examination of peripheral blood smear in diagnosis of anemia has taken a backseat with the advent of automated counters. Though a lot of studies have been done to assess the efficacy and significance of red blood cell parameters in different hematological conditions fewer efforts have been made to standardize the visual examination of peripheral blood smears for diagnosing anemias. Aims: Standardization and grading of abnormal red cell morphology in peripheral blood smear and counter based red cell indices in cases of anemia of various etiologies. Settings and Design: Cross-sectional study of one year duration conducted in the Hematology laboratory, in a tertiary care hospital in North India. Materials and Methods: In 60 anemic patients, automated counts and peripheral blood smear were prepared and evaluated by three observers, according to a red cell morphology grading guide. Statistical Analysis Used: ANOVA, Tukey post hoc test were used. Results: Objective grading of peripheral blood smears in cases of anemia have a good inter observer correlation and hence have reduced subjective variation. Manual parameters like microcytosis, macrocytosis and hypochromia expressed as a percentage, have shown significant correlation, with their corresponding automated parameters, and the regression model so generated may provide a novel way for quality control of automated counters, if calculated for different models. Conclusions: Even in the age of molecular analysis, the blood smear remains an important diagnostic tool and sophisticated modern investigations of hematologic disorders should be interpreted in the light of peripheral blood features as well as the clinical context.

Background: Data on frequency distribution of ABO Rh blood group in pediatric dengue patients are not available. Dengue disease an emerging arthropod borne infection of public health concern belongs to Flaviviridae. The studies defining the relationship between blood groups and dengue disease and its severity are limited. Blood group antigens are generally known to act as receptors for various etiological agents. Hence, we hypothesized there will be an association between blood group and dengue disease and its severity and conducted this case-control study. Study Design: Hospital-based case-control study. Materials and Methods: Study was conducted in department of pediatrics, 244 pediatric subjects (age group 1 - 13 years) were enrolled divided into cases who were admitted cases of dengue (119, 65 males, 64 females) and controls who were attending outpatient department for various other ailments. (125, 67 males, 58 females) and demographic data (age, gender, blood group, and dengue infection status) were collected from them. The risk of acquiring dengue disease and severity and its association with factors such as blood group, gender were analyzed statistically. Results: The data of this study showed a possible association between blood groups of the study population with dengue infection. We observed that dengue infections were higher in individuals with O positive blood group 42.8% when compared with controls 32%. (P = 0.043) But blood groups were not associated with severity of infection. These data present further evidence for the association of the blood groups, gender to susceptibility to dengue infection. Further studies are needed to confirm these findings. Conclusion: Dengue disease is more common with blood group O. But severity of the disease is not associated with any blood groups.

Background: Susceptibility of sickle cell disease subjects to various infectious agents is on the increase, but information relating SCD to allergic condition is scarce. Hence, assessment of immunoglobulin status in SCD children may provide useful information to improve management of SCD children. Objectives: To determine the levels of immunoglobulin classes (IgG, IgA, IgM, and IgE) in Nigerian HbSS children below 5 years of age compared with sex- and age-matched HbAA children. Materials and Methods: Blood samples were collected from a total of 45 children less than 5 years of age who were recruited into the study as follows: 26 HbSS and 19 HbAA subjects for the estimation of serum immunoglobulin levels using enzyme-linked immunosorbent assay (ELISA) techniques and determination of genotype using electrophoresis technique. Results: IgG concentration was nonsignificantly higher (P = 0.997) in HbSS children (1022.56 ± 148.97 ng/ml) compared to HbAA children (933.68 ± 106.10 ng/ml). IgA (P = 0.906) and IgM (P = 0.986) concentrations were nonsignificantly lower in HbSS children (255.07 ± 133.71 ng/ml) compared to HbAA children. IgE was significantly higher (P = 0.000***) in HbSS (108.67 ± 69.22 IU/ml) compared to HbAA (24.51 ± 17.58 IU/ml) children. Conclusion: SCD children in steady state have adequate levels of Ig classes. Non-specific elevation of IgE levels may be a factor of inflammatory response in SCD children, and this may be proposed for reduced allergic reaction among SCD children.

Hemophagocytic lymphohistiocytosis (HLH) is a rare condition of unregulated immune function. Prompt clinical detection and treatment of the condition is important for survival. Its association with hepatitis A is rare and only limited to case reports. We present a 4-year-old boy with hepatitis A associated HLH and treated successfully with steroid monotherapy. A review of literature has been done for hepatitis A associated secondary HLH.

The hallmark of hemophilia A is prolonged bleeding and re-bleeding. We report a case of 28-year-old male with hemophilia A who after road accident presented with acute subdural hematoma which after evacuation re-bled to extradural hematoma. Prompt diagnosis and treatment with Factor VIII infusions perioperatively and meticulous hemostasis are the key to the management of such cases.