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).

Objective: The aim was to evaluate the efficacy and safety profile of dapsone as a cheap second-line treatment for chronic immune thrombocytopenia (ITP) in developing countries. Materials and Methods: A prospective study on 100 chronic ITP patients. These patients were put on dapsone after ruling out glucose 6 phosphate dehydrogenase deficiency and secondary causes of ITP. Results: The basic work up for secondary causes of ITP was negative. All these patients had been treated with steroids in the past. Anti-D had been given acutely in 20 patients, and intravenous immunoglobulin G had been given in 10 patients. Vincristine had been given to 20 of these patients. Dapsone was started in these patients, and 44% patients showed a response to treatment. The mean time to onset of response was 21 days. Out of these 44 patients, 21 (47.7%) went into remission and had platelet count >100,000/μl at 2 years post tapering of the treatment. Remaining 23 patients were kept on low dose dapsone and maintained their platelet counts. Adverse drug reactions included mild skin eruptions in 5% of patients, pruritus in 10% of patients, dose-related hemolysis in 1% of patients, methemoglobinemia in 1% of patients and Stevens Johnson syndrome in 1% of patients. 56 patients were nonresponders to the available, affordable conventional medical treatment and were referred to the surgical department for splenectomy, with a cure rate of 86% postsurgery. Conclusions: Dapsone is a safe, cheap and effective treatment option for patients with chronic ITP, who cannot afford the usual costlier second-line drugs.

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.

Background: The aim is to detect the prevalence and specificity of red cell allo-antibodies underlying pan reactive auto-antibodies in Egyptian AIHA patients Patients and Methods: Sixty multi-transfused Egyptian AIHA patients received 715 units of blood were studied during the period from February 2013 to November 2013. Initial antibody screening and identification (using pre-warming technique in cases of cold auto-antibodies), direct antiglobulin, auto-control and immediate spin, allogenic adsorption for patients` plasma then detection of possible underlying allo-antibodies were done. Results: Five patients with cold auto-antibodies were identified with absence of underlying allo-antibodies. From the 55 patients with warm auto-antibodies, 23 (41.8%) had allo-antibodies with a total number of 40 underlying allo-antibodies indicating that Egyptians carry the highest percentage of allo-antibodies compared to other studies. Conclusion: Red cell allo-immunization has a very high frequency among multi-transfused Egyptian AIHA patients.

Introduction: Standard guidelines assert that any vacutainer, which is 10% less under-filled should be rejected as it may affect the results. These guidelines refer to the studies using liquid K ₃ -ethylene diamine tetra acetic acid (EDTA). Although the new guidelines recommend K ₂ -EDTA instead of earlier K ₃ -EDTA but has not clearly commented upon whether the same holds true for the new anticoagulant K ₂ -EDTA. Materials and Methods: Blood samples from 100 healthy blood donors were included. We took 7-8 ml of blood and was then transferred to three 3 ml capacity vacutainers-up to 3 ml, 2.5 ml, 1 ml (marked a, b, c respectively) containing spray-dried K ₂ -EDTA as anti-coagulant. Thus, b and c vacutainers were under-filled. Hematological parameters in all the samples were analyzed immediately using automatic analyzer (Cell Dyn). Statistical analysis was done with the help of SPSS v. 17.0 (Chicago, IL, USA) using one-way ANOVA. Results: The results showed that three groups were comparable with respects to hemoglobin, hematocrit, total leukocyte count, differential leukocyte counts, total platelets, mean corpuscular volume, mean hemoglobin concentration, mean cellular hemoglobin concentration along with red cell distribution width. Conclusion: Our study suggested that under-filling till 67% (1 ml in 3 ml standard capacity) of the recommended volume in a spray-dried K ₂ -EDTA vacutainers did not affect hematological parameters in healthy people. In view of these results, there is need to further investigate the same in diseased. If further studies done on under filling of the pathological samples also give similar results as in our study then only the present guidelines related to under filling could be revised when K ₂ -EDTA is used as an anticoagulant.

Cryoglobulinemic vasculitis is one of the rare manifestations of plasma cell dyscrasias. A 54-year-old diabetic housewife presented with pain and blackish discoloration of her right foot and medial four fingers of the right hand. She underwent transmetatarsal amputation of her right and left feet due to digital gangrene 1 year and 9 months back respectively. On diagnostic evaluation, she was found to have multiple myeloma with cryoglobulinemic vasculitis. This case report emphasizes the importance of considering the possibility of cryoglobulinemic vasculitis and underlying plasma cell dyscrasias in patients presenting with gangrene of extremity.

Plasma cell leukemia (PCL) is a rare presentation of multiple myeloma characterized by the presence of >20% plasma cells in peripheral blood, and an absolute plasma cell count >2 × 109 K/L. It is classified as primary and secondary with the latter representing a terminal event in 12% of patients. A case of a 78-year-old man who presented a secondary form of PCL during post-chemotherapy remission phase is discussed accompanied with a brief review.

Previously, researchers were reported P value as a basis for conclusion, but in this era of evidence-based medicine we are interested now about the precision of the results obtained that allow us to make right decisions in health care practice. The confidence interval (CI) of a parameter goes beyond the P value by also reflecting the precision of the degree to which one can estimate the true population difference. For a particular size of the difference in the population, the larger sample size, the smaller the P value and the narrower will be the CI.