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.

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Background: Thalassemias and hemoglobinopathies are highly prevalent in India. Identification of these disorders is important for epidemiologic purposes and for prevention of thalassemia major and clinically severe hemoglobinopathies. Objectives: The aim of this study was to determine the prevalence of thalassemias and hemoglobinopathies in patients of a tertiary care hospital of West Bengal. Materials and Methods: A prospective study was undertaken in which 90,210 cases were included over a period of 8 years. Clinical history and family history were obtained from each patient. The venous blood samples were analyzed for complete blood count, liver function tests, serum iron, ferritin, cobalamin and folate levels. High-performance liquid chromatography (HPLC) was performed on the samples with Biorad Variant using beta thalassemia short program. Confirmatory tests were done whenever required. Results: Normal hemoglobin (Hb) pattern was observed in 79,897 (88.57%) cases and abnormalities were detected in 10,313 (11.43%) patients. β (beta) thalassemia trait was the most common abnormality found in 3870 (4.29%) patients. HbE trait was found in 2418 (2.68%) cases, and then Eβ thalassemia in 1406 (1.56%) patients and β thalassemia major/intermedia in 1135 (1.26%) cases. Other variants detected included sickle cell trait, HbE disease, sickle cell disease, sickle β thalassemia, HbD-Punjab trait, double heterozygous state of HbS and HbE, double heterozygous state of HbS and HbD, Hb Lepore, HbJ-Meerut and HbH. Conclusion: Premarital and antenatal screenings are important measures to prevent birth of children with severe Hb disorders. HPLC is a rapid and reliable technique for identification of various Hb fractions.

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Tuberculosis (TB) is a mycobacterial infectious disease that can affect any organ system. The wide varieties of manifestations that TB can present with make the disease a challenge for treating physicians. Anemia is commonly seen in patients with TB, and it is usually anemia of chronic disease. However, the occurrence of autoimmune hemolytic anemia in the setting of TB infection is rare.

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Primary renal Burkitt lymphoma (BL) is an unusual occurrence in children. Although the prognosis of this tumor is poor, early diagnosis on biopsy confirmed by immunohistochemistry (IHC) and followed by chemotherapy improves survival of such patients. We present a case of 4-year-old male child with bilateral renal lump suggestive of lymphoma on computerized tomography. Renal biopsy and subsequent IHC was suggestive of bilateral BL in the kidney; following which the child was started on FAB LMB 96 based treatment protocol.

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Background: Despite the confirmed favorable prognosis of childhood t(8;21) acute myeloid leukemia (AML), recent reports suggest heterogeneity in survival outcomes in this subtype of AML may be influenced by ethnicity. Therefore, we aimed to assess the outcome of childhood t(8;21) AML in an Arab population to evaluate if survival outcomes were inferior and determine the predictive relevance of additional cytogenetic abnormalities. Methods: This multicenter retrospective study analyzed 175 de novo AML children of 14 years of age or younger consecutively diagnosed between January 2005 and December 2012. Survival outcomes were analyzed and patients with t(8;21) were stratified on the basis of karyotype into sole and additional cytogenetic groups. Results: A total of 33 (18.9%) patients had t(8;21) AML. Complete remission (CR) was achieved in 31 (93.9%) patients. The 5-year overall survival, event-free survival, cumulative incidence of relapse (CIR), and remission death rates were 59.9 ± 9.2, 45.6 ± 9.1, 36.4, and 9.1%, respectively. Despite the administration of hematopoietic stem-cell-transplant salvage therapy in first relapse, five out of 11 (45.5%) relapsed patients died of disease. Subanalysis of sole vs. additional cytogenetic abnormalities revealed no significant difference in outcome. Conclusion: In the present study, childhood t(8;21) AML was associated with inferior survival and resistance to salvage therapy compared to reports from international groups. The inferior outcomes were unrelated to additional cytogenetic abnormalities. Further detailed genetic studies are warranted to unmask the biological and clinical differences between racial/ethnic groups. Given the high CR rate of childhood t(8;21) AML, further modification of postremission therapy to improve the CIR rate is needed.

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Middle East respiratory syndrome corona virus (MERS-CoV) is becoming a major health concern in Saudi Arabia resulting in an associated high case fatality. The rapid deterioration of vital organs functions and high mortality rate seen in MERS-CoV has been observed previously in Epstein-Barr virus (EBV), severe acute respiratory syndrome caused by a coronavirus (SARS-CoV) and avian and bird flu-related hemophagocytic lymphohistiocytosis (HLH). There are many reports of successful treatments for severe cases of EBV-HLH using HLH-94/2004-type immunochemotherapy with etoposide as a pro-apoptotic agent. The use of immunochemotherapy in severe MERS-CoV-related HLH merits an urgent investigation in a well-designed clinical trial.

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Severe aplastic anemia (SAA) is a serious bone marrow disease that needs a comprehensive and service-intense treatment with either bone marrow transplantation (BMT) or immunosuppressive therapy (IST); both are difficult to optimally offer in resources-limited countries. Here, we report the outcome of IST using horse Antithymocyte globulin (ATG) in 18 (7 children; 11 adults) patients with SAA referred to our center in west India. Only 18 patients out of 102 diagnosed as AA in 2 years could receive IST, largely due to costs restraints. Although CR was seen in 30% in adults and 33% in pediatric cases, but overall 50% cases were able to enjoy transfusion-independence, requiring no further treatment. Treatment related mortality occurred in 6.2%, relapse in 6.2% and 6.2% had clonal evolution. This makes IST a valuable option for managing SAA in absence of bone marrow transplantation.

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Context: Pancytopenia is not a disease but a clinico-hematological entity encountered in clinical practice. A prompt intervention is required to avoid complications, which can occur in these patients. The relevant clinical history and complete hematological workup is required to treat patient of pancytopenia. The severity of pancytopenia and the underlying pathology determines the management and prognosis. Aims: To diagnose different conditions producing pancytopenia on the basis of clinical, hematological and bone marrow studies. To estimate the frequency of different diseases producing pancytopenia. Subjects and Methods: The study was conducted in Department of Hematology of our college in capital city of India. A prospective study was carried out among 100 consecutive patients with pancytopenia. Blood samples of the patients were analyzed for red cells, white cells and platelets morphology along with presence and absence of immature cells and abnormal cells. In bone marrow examination, morphology of all cells lineage, cellularity, parasite and abnormal cells were scrutinized. Trephine biopsy was done where ever indicated. Results: The age of patients ranged from 5 to 80 years. 74% of patients in this study were vegetarian. The most common cause of pancytopenia was megaloblastic anemia (66%) followed by aplastic anemia (18%), malaria (6%), kala-azar (4%), acute myeloid leukemia (2%), multiple myeloma (2%), myelodysplastic syndrome (1%), and tuberculosis (1%). Conclusions: The present study concludes that detailed primary hematological investigations along with bone marrow examination in cytopenic patients is helpful for understanding disease process, to diagnose or to rule out the causes of pancytopenia. It is also useful in planning further investigations and management.

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Background and Objective: Recently totally implanted vascular access devices (TIVADs) have played an essential role in the management of pediatric patients. The purpose of this study was to describe the indications, techniques and complications of the procedures. Design and Setting: A retrospective study of all patients' files underwent TIVADs procedures between 2009-2012, in a single service providing institute in Saudi Arabia (King Saud Medical city-Riyadh). Patients and Methods: Fifty four TIVADs were inserted in fifty one patients (thirty males and twenty one females). Age ranged six months and twelve years (average 5.6 years). All TIVADs were implanted by pediatric consultant surgeons under general anesthesia. Seldinger technique used in fifty procedures and open approach in four patients. Results: The main indications for TIVADs use were acute lymphocytic leukemia forty six (90%), congenital nephrotic syndrum two (4%), hemophilia two (4%) and solid tumor one (2%). TIVADs were in situe for a total of 35222 days (average 652 days/catheter). Over all complications rate was (30%). Conclusion: TIVADs provided a simple almost pain free venous system access, allow children to conduct a normal life style and sport activities. We believe this study is one of very few reviews originating from developing countries with results comparable to those of world literature.

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Context : Various hemoglobinopathies are one of the major public health problems of India. Aims: High performance liquid chromatography (HPLC) is a speedy and accurate tool for diagnosis of various hemoglobinopathies. In present study about 7,000 cases have been studied for identification of various hemoglobin (Hb) disorders in western India. Settings and Design : Study is conducted from May 2010 till April 2013 for various hemoglobinopathies and variants. The geographical distribution of all cases included states of western India. Materials and Methods: Complete blood count (CBC) is done on CELL DYN 3700 analyzer and then HPLC is performed on BIO-RAD 'VARIANT II' (beta thalassemia short program) in samples received in our laboratory. Simple Statistical analysis is done with help of Microsoft Office Excel 2007. Results: A total of 7,261 cases were included in our study, out of these 1,615 (22.24%) cases showed abnormal Hb fractions. The major abnormality observed was of high HbA2, a cutoff value of > 3.9% was considered for diagnosis of beta thalassemia trait (BTT). A total of 839 cases (11.55%) of BTT were diagnosed. Other hemoglobinopathies were also identified in varying proportions. Conclusions: HPLC is simple, accurate, and superior technique combined with complete automation makes it an ideal method for diagnosis of hemoglobinopathies.

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Background / Objective: The presence of persistent high fetal hemoglobin (HbF) in sickle cells disease (SCD) patients may be a modulator of clinical and biochemical features. This study seeks to test the hypothesis that high level of HbF may regulate the levels of calcium, magnesium, zinc, and copper in SCD patients in a steady clinical state. Materials and Methods: Serum calcium, magnesium, zinc, and copper were assayed in 100 SCD patients in steady clinical state and 50 control subjects using the colorimetric method while blood HbF was determined by alkaline denaturation method. Results: Twenty-five percent of the study group had high (>5%) HbF, while 75% had low (<4.9%) HbF levels. HbF (P < 0.001), serum copper (P < 0.001), and calcium (P = 0.002) were significantly higher in SCD patients compared with controls, while zinc and magnesium were significantly lower (P < 0.001) in SCD patient compared with controls. Serum calcium (P = 0.01) and copper (P = 0.118) were lower in SCD patients with high (≥5%) HbF while magnesium and zinc were significantly higher (P < 0.001) in SCD patients with high HbF compare with those with low (≤4.9). HbF correlated negatively with calcium (r = −0.25, P = 0.011) and copper (r = -0.11, P = 0.287) while magnesium (r = 0.60, P = 0.001) and zinc (r = 0.57, P < 0.001) correlated positively on HbF levels. Conclusion: HbF levels may have modulated the levels of these elements in SCD patients. It is suggested that HbF may be estimated along with hemoglobin electrophoresis in diagnosis, clinical management, and predicting clinical course of SCD patients. Nutritional studies may be routinely conducted in this group of patients for better management.

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Context: Oral anticoagulant therapy (OAT) is one of the most widely used therapies. Being on such regimens requires high degree of compliance and adequate knowledge to avoid serious complications. Aims: This study aims to assess compliance with and awareness about OAT among Saudi patients, and their willingness to use the point-of-care (POC) international normalized ratio (INR) testing devices for self-monitoring. Settings and Design: This cross-sectional study was conducted at a tertiary hospital in Riyadh, Saudi Arabia, over 6 months. Subjects and Methods: A face-to-face interview has been carried out for all patients based on the questionnaire carried out for all patients based on the questionnaire. Results were analyzed according to demographics, adherence, knowledge, and INR control. Statistical Analysis Used: Statistical Package for the Social Sciences version 19 software (SPSS Inc., Chicago, IL, USA) was used. Results: One hundred sixty-two patients were interviewed, of which females (69.1%) exceeded males (30.1%). Most of them were on warfarin (80.2%), received education by their physicians. In general, patients had poor knowledge and medium adherence (53.1%) (scored < 50%). About 24% of the poor knowledge group (PKG) were highly adherent compared to 14.5% of the fine knowledge group (FKG). However, 53.2% of FKG had a controlled INR where this percentage reduces to 27% in PKG. The most incorrect answered question in both groups was related to warfarin-drug-interactions (75.3%). The majority (74.7%) was eager to make use of the POC-INR devices. Conclusions: The participants' knowledge was generally poor but level of knowledge did not play a role in compliance. Regardless, an education program should be accommodated to help patients in improving their medication control and reducing clinical visits. The majority was willing to adopt (POC) INR devices that will certainly help them in managing their treatment and potentially reducing adverse clinical outcomes.

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Purpose: Although few previous studies suggested an increased prevalence of obstructive sleep apnea (OSA) among children with sickle cell disease (SCD) compared to others, there are little data regarding this association in adults. In this study, we aimed to investigate the prevalence of OSA among the patients with SCD and its risk factors. Materials And Methods: This cross-sectional, observational study was conducted at a specialty hematology clinic. From December 2013 to July 2014, all the patients with SCD and chronic anemia were approached. Data were collected on the risk of OSA using the Berlin questionnaire, and the data on daytime sleepiness were collected using the Epworth sleepiness scale (ESS); medical history of the patients was also obtained. The objective diagnosis of OSA using polysomnography was performed in a subsample of the study population. Results: The study included 106 patients, 44.3% with SCD and 55.7% with other types of anemia being used as a comparison group. Among the patients with SCD, 14.9% were at a high risk of OSA, and 23% were confirmed to have OSA, whereas 10.1% and 16.7% of those in the comparison group did, respectively. However, there were no statistically significant differences between the two groups. Mean scores of risk factors for OSA among the patients with SCD with OSA were 28 years for age, 22.1 kg/m²for body mass index, 14.5 inches for neck circumference, 122 mmHg for systolic blood pressure, and 6.67 for ESS score. Conclusion: Although not different from chronic anemia, the OSA is a common, coexisting disease in the patients with SCD. Furthermore, it is suspected that the traditional screening tools may miss potential cases of OSA in the SCD population.

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Cluster of differentiation (CD) 56, a member of the immunoglobulin superfamily, and an isoform of neural cell adhesion molecule (NCAM), was the first cell adhesion molecule to be identified. NCAM (CD56) plays an important role both in human health and in disease. Human NCAM gene is located on chromosome 11q23. CD56 antigen is a 175–185-kD cell surface glycoprotein expressed on all subsets of human natural killer (NK) cells except a small minority of CD56⁻ NK-cell, on subsets of CD4⁺/CD8⁺ T-cells, interleukin-2-activated thymocytes, bone marrow macrophages, osteoclasts, and on adrenal gland and neural tissues. NCAM is important in calcium independent cell-cell interactions that mediate homotypic and heterotypic cell-cell and cell-matrix adhesions. At least 27 alternatively spliced NCAM mRNAs are produced giving a wide diversity to NCAM isoforms sharing a similar structural organization. NCAM in the cerebellum and cerebral cortex mediates homophilic adhesion of neural cells, and plays an important role in brain development, emotions, and memory functions. While CD56⁺ NK-cells play an important role in defense against infections, tumor remission, normal pregnancy and graft rejection. Malignancies expressing CD56 are usually aggressive, with more potential for metastasis and extramedullary/central nervous system involvement, and may respond to new CD56-linked targeted therapies.

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Context: Role of youngsters in voluntary blood donation (VBD) is crucial to meet the demand of safe blood. Therefore understanding the various factors contributing to knowledge, attitude, and practice of VBD among college students is important. Aims: The present study aims to assess the level of knowledge, attitude, and practice regarding blood donation among the health care students. Settings and Design: Cross-sectional study done at Anand from July 2012 to December 2013. Subjects and Methods: A cross-sectional study was conducted among 1000 health care medical students at Anand, Gujarat, India using a structured survey questionnaire. Statistical Analysis Used: The data were tabulated, and master chart was prepared. Analysis was performed by using the percentage. Results: Overall knowledge on blood donation among respondents was 37.3%, majority of the participants (78.7%) never donated blood in which 43.7% of the nondonors had negative attitude like blood donation leads to weakness (10.2%), anemia (25.5%), and 8.0% of nondonors felt that blood donation leads to reduced immunity. Majority of nondonors showed a positive attitude (53.1%) by expressing their willingness to donate blood if they were asked to donate blood (35.3%), about 9.8% of the nondonors don't know the importance of blood donation and 11.2% of nondonors don't know where to donate blood. In this study, 11.5% of students had donated blood only once, in which 38.8% of blood donors said no privacy was provided while donation, few (12.2%) had experienced some discomfort after donation and 50.4% of respondent said that they have not tried again. Conclusions: The present study recommends that even health care students' needs to be educated about the importance, awareness and motivation of blood donation on a regular basis and addressing the problems faced, while blood donation will strengthen the recruitment and retention of blood donors to donate blood on a regular basis.

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Aim: Acute myeloid leukemia (AML) is a type of malignancy that is associated with a malignant alteration of normal hematopoietic stem cells in the bone marrow. The aim of this study was to study the demographics and pathological subtypes of AML, evaluate the response and outcome to different treatment modalities. Methods: This was a retrospective study of adult patients diagnosed with AML at King Abdulaziz Medical City - Riyadh, between 2006 and 2013. Data were retrieved from patients' files, electronic medical files and laboratory information system. Results: 91 patients were included in the study with a male dominance. M1 was the most common French-American-British subtype with 23 (32%) cases. Patients with intermediate-risk AML were the most common subgroup with 41 (48%) cases followed by high and low-risk subgroups, 29 (33%) and 16 (19%), respectively. 74 patients were treated with intensive chemotherapy, and 17 were on palliative chemotherapy or best supportive treatment. Remission rate was found to be 84% in patients who received induction chemotherapy while 41% of them relapsed. 93% of low-risk patients underwent complete remission (CR) compared to intermediate and high-risk patients (79% and 87% respectively), but it was not statistically significant (P = 0.4). The median follow-up was 19 months, with overall survival (OS) of 46% for all groups. The low-risk patients had the highest OS 57% compared to intermediate and high risk (52% and 36%, respectively), but it was not statistically significant (P = 0.3). 18 patients had been treated with allogeneic stem cell transplant and at a median follow-up of 17 months posttransplant the OS was 72%. Conclusion: This study shows M1 subtype to be the most common of AML in this population. In addition, the CR was better with similar survival rate as compared to other local and internationally published experiences. These results, albeit with its limitations, need to be confirmed in a prospective clinical trial or national disease registry.

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Objectives: Many Iranian studies have reported the high prevalence of osteoporosis and low bone mass in patients with thalassemia. Considering the importance of prevention in lowering the risk of osteoporosis and its complications in patients with thalassemia major (TM), the present study was conducted to assess the knowledge, attitude and preventive practice (KAP) of a group of TM patients regarding the importance of calcium and Vitamin D supplementation and its role in preventing osteoporosis. Materials and Methods: The cross-sectional study was conducted on all adult beta-TM patients (200) who visited the Specific Disease Center, in the Iranian Capital, Tehran. A four-section questionnaire on demographic, lifestyle, and socioeconomic information as well as their KAP about the importance of calcium and Vitamin D in preventing osteoporosis in TM patients was filled out for each patient. Results: About 63%, 62%, and 33% of those with poor knowledge, attitude and practice towards the importance of calcium and Vitamin D were not physically active, correspondingly. There was no association between age and gender and knowledge or attitude score. Conclusion: While a large number of the studied population consumed Ca and Vitamin D supplementation, their poor knowledge about the importance of these supplements points out the need for educating TM patients and their families about the importance of calcium and Vitamin D supplementation in preventing osteoporosis.

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Background: Tumor lysis syndrome (TLS) comprises a number of metabolic and renal abnormalities of variable spectrum. It is associated with lymphoproliferative disorder e.g., acute lymphoblastic leukemia (ALL), non-Hodgkin lymphoma (NHL). The aim of this study to evaluate the incidence, severity and outcome in Saudi children with ALL. Methods: Medical records of Children age <14 years and diagnosed with ALL were reviewed. We adopted the Hande and Garrow definitions for tumor lysis Syndrome. All patients received hydration, alkalinization with sodium bicarbonate and allopurinol as prophylaxis. Results: Seventy-four patients developed TLS (19% of all patients). Boys were affected than girls with ratio of 2.4:1. LTLS occurred in 93.3% and CTLS in 6.7%. Hyperphosphatemia was found in 94.6% of the patients. Hyperkalemia occurred only in 17 patients (23%). Hypokalemia was found in significant number of patients (12%). Lymphomatous presentation was seen in 60 patients (82%). Hemodialysis was required in 5 patients (6.6%) and 4 of them had hyperleukocytosis. There were no deaths encounterd due to TLS. Male sex, lymphomatous presentation, high LDH, high pretreatment WBC and central nervous system involvement were found to have statistical significance as risk factors for TLS development. Conclusion: TLS is a common event in childhood ALL. Spontaneous TLS indicate highly proliferating tumor and possible delayed presentation to our institute. Prophylactic measures are important to improve the outcome particularly when initiated as soon as the diagnosis is established.

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Background and Objectives: Donor screening process is one of the most important steps in protecting the safety of blood supply. Donors who do not meet specified criteria are deferred either temporarily or permanently. These criteria are designed to protect both donors and patient safety. Due to the varied rates and reasons for deferrals in the existing literature, we aimed to evaluate the patterns and prevalence of deferrals in our institution. Materials and Methods: This retrospective study was conducted at a Tertiary Care Hospital, Karnataka, Southern India, to evaluate the various reasons for blood donor deferral from January 2011 to January 2014. Demographic data of blood donors was obtained through the blood bank database and secondary measures such as the type of deferral (permanent/temporary, pathogenic/nonpathogenic, and harmful to donor/recipient) were assessed. Results: A total of 54,653 subjects presented to our blood bank during this period out of which 2935 (5.6%) were deferred. The deferral to donor percentage was higher in females (36.54%) than males (3.64%). Low hemoglobin was the major deferral criterion seen in our participants (48.1%) followed by hypertension (16.4%), underweight (8.9%). Low pulse rate and fasting donor were the least prevalent reasons. A total of 36.8% of reasons for deferral were harmful to donors, 88.2% were nonpathogenic, and 98.1% were temporary causes. Conclusion: Variations in donor deferral may be attributed to different donor selection criteria in different regions and centers. Hence, it is important to know the common causes of donor deferral in a region so that measures may be taken to improve the donor pool.

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Introduction: Sickle cell disease (SCD) is an inherited genetic disorder characterized by various complications, including thrombosis. Increased levels of circulating microvesicles (MVs) and tissue factor (TF)- bearing MVs have been reported in SCD. Objectives: The present study compares the levels of circulating MVs and TF- bearing MVs in steady state SCD children with age- and gender- matched healthy controls using an indirect ELISA. Citrated whole blood was collected from 54 SCD patients homozygous for sickle haemoglobin (HbSS) (aged from 2 to 12 years-old) and 34 healthy controls. Results: SCD patients showed significantly higher levels of MVs in their plasma as compared to the controls (P = 0.0095). Although the TF activity on MVs was low in both groups, there was a significant difference between them P <0.05). A strong correlation between the level of MVs and TF-MVs in the patient group was also noted. Conclusion: This suggests their involvement in the hypercoagulable state in the study group of patients. Further studies are recommended to elucidate the functional activity of MVs and TF-MVs, as well as the size and origin of MVs in the plasma.

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Background: Donor reaction has the most negative impact on the blood donor return rate. Amelioration of some adverse events has the potential to improve return rate. This study was initiated with the aim to determine the frequency of various adverse reactions. Materials and Methods: The study was conducted over a period of 7 years and 1 month, from July 1, 2007, to August 31, 2014. The donor population analyzed consisted of 27,664 donors (26,557 males and 1107 females). Results: Only 195 donors (0.7%) suffered some adverse reactions. One hundred and fifty-eight (0.57% of the subjects) had mild reactions (agitation, sweating, giddiness, pallor, cold feeling, sense of weakness, nausea), and only 37 (0.13% with 10 females and 27 males) had more severe reactions (including vomiting, fainting, loss of consciousness, and convulsive syncope). Conclusions: Although the number of donors who developed adverse reactions during or immediately after the blood donation was very low, still it is very important to reduce risks to a minimum so that donor return rate could be maintained.

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The updated WHO classification system for myelodysplastic syndromes (MDS) includes some new entities, in particular "refractory cytopenias with unilineage dysplasia (RCUD)". The following review article presents a short overview regarding all subvariants of MDS but with special emphasis on the rare subtypes of MDS that can be exclusively recognized histomorphologically: (1) Hypoplastic MDS (MDS-hypo), (2) Fibrotic MDS (MDS-F) and MDS with associated systemic mastocytosis (SM-AHNMD).

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Hemophilia is an X-linked heritable coagulopathy with an overall prevalence of approximately 1 in 10,000 individuals. The most common form is factor VIII deficiency (hemophilia A), which comprises approximately 80% of cases. Factor IX deficiency (hemophilia B), comprises approximately 20% of cases. In patients with hemophilia, regular replacement therapy with clotting factor concentrates (prophylaxis) is effective in preventing recurrent bleeding episodes into joints and muscles. However, despite this success, intra-articular and intramuscular bleeding is still a major clinical manifestation of the disease. Bleeding most commonly occurs in the knees, elbows, and ankles, and is often evident from early childhood. The pathogenesis of hemophilic arthropathy is multifactorial, with changes occurring in the synovium, bone, cartilage and blood vessels. Recurrent joint bleeding causes synovial proliferation and inflammation (hemophilic synovitis) that contribute to end-stage degeneration (hemophilic arthropathy); with pain and limitation of motion severely affecting patients' quality of life. The author conducted a comprehensive review and synthesis of the relevant literature. The author reviewed all compiled reports from computerized searches. Searches were limited to English-language sources and human subjects. Literature citations were generally restricted to published manuscripts appearing in journals listed in Index Medicus and reflected literature published up to July 1, 2013. The aim of this updated paper is to provide comprehensive and timely evidence-based guidelines and recommendations on the treatments of damaged joint among hemophilia patient. Further recommendations included the control of risk factors, interventional approaches for treatment of hemophilia, the use of bypassing factors for the inhibitors, and preventing recurrent bleeding especially in high-risk populations are provided. Prophylaxis by replacement of the missing factor in patients with hemophilia is the optimal way to prevent the occurrence of haemarthrosis and thereby the onset of arthropathy, provided that it is started early in life. Dosing should be individualized and increased in case of bleeding. Prevention of bleeding episodes through early treatment will prevent accumulation of blood in joint and the subsequent inflammation and potential hemophilic arthropathy. Treatment must be maintained until bleeding remission and patients have recovered as much of their ROM and muscular strength as possible. Clinical evaluation of the joints, gait, motion, muscle tone, functional level of disability, pain, and swelling, as well as imaging techniques, must be performed to assist in the diagnosis of chronic synovitis and to guide treatment decisions. The first step to treating synovitis, refractory to medical treatment, is the use of synovectomies, non-surgical or surgical interventions. In many cases, joint deformities have to be treated by open orthopaedic surgery. State-of-the-art treatment of patients with hemophilia requires a multidisciplinary team. Level of evidence: Level III (Systematic Literature review).

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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.

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