Leishmania donovani bodies in bone marrow

Key clinical message: We report a case of a 5-year-old female, resident of Afghanistan, who presented with fever and massive splenomegaly. Bone marrow revealed Leishmania donovani bodies (LD bodies) in macrophages characterized by a kinetoplast and characteristic double dot appearance. She was diagnosed as visceral leishmaniasis which is transmitted by sandflies (Phlebotomus)

Compliance of hand written transfusion requisition form and improvement after online request - A clinical audit

Objective: To assess the compliance of healthcare personnel with regard to sending completely filled transfusion requisition forms.Methods: The audit was conducted at Aga Khan University Hospital, Karachi, and comprised requisition slips received at the hospital blood bank from September 2014 to February 2015. The British Committee for Standards in Haematology guidelines was used as the standard. Percentage of each variable on the proforma was analsyed. Rating \u3c50% for each form was defined as needs improvement , 51-99% as good compliance and 100% as excellent compliance . After implementing strategies to increase awareness and the launching of an online transfusion requisition form, a re-audit of physician compliance was done from February to April 2016 and the results were compared with the initial audit.. Data was analysed using SPSS 21.Results: The audit and the re-audit both comprised 1000 transfusion requisition forms each. In the audit, The sum of total scores of all the transfusion requisition forms was 4911, indicating a compliance rate of 46.9%, while the corresponding numbers in the re-audit were 10000 and 100%.Conclusions: The implementation of online blood transfusion requisition system had a positive impact on compliance rate

Anemia in a middle aged female with aortitis: a case report.

Abstract BACKGROUND: Idiopathic aortitis is among the most common causes of non-infectious aortitis, which rarely presents with anemia. CASE PRESENTATION: Here we report a case of a 49-year-old muhajir female who presented with shortness of breath and easy fatigability for the past 6 months. Physical examination revealed pallor and a diastolic murmur in the aortic region. Echocardiography showed thickened and calcified aortic and mitral valves, severe aortic regurgitation and dilatation of ascending aorta. She was advised aortic valve replacement and was referred to a haematologist due to concomitant anemia. Complete blood counts revealed haemoglobin: 7.7 gm/dl, mean corpuscular volume (MCV): 78 fl, mean corpuscular haemoglobin (MCH):23 pg, total white cell count: 9.0 × 10(9)/L and platelet count: 227 × 10(9)/L. Erythrocyte sedimentation rate (ESR) was 100 mm/hr. There was suspicion of myelodysplastic syndrome, but could not be confirmed as the patient refused bone marrow and cytogenetic studies. She was given erythropoietin, folic acid and ferrous sulphate. Following relatively prolonged therapy, her haemoglobin level increased to approximately 9.0 gm/dL. She was transfused with packed red cells and underwent aortic valve and ascending aorta replacement. The ascending aorta was dilated and aortic wall markedly thick and irregular. Histopathology of the resected aorta revealed granulomatous aortitis. She was prescribed prednisolone, which resulted in further incremental rise of haemoglobin to 13.1 gm/dL. One month later, she developed complaints of blurred vision in the right eye and was diagnosed with central retinal vein occlusion. She was treated with antiplatelet agents and her vision improved. After 3 months, she was asymptomatic and her haemoglobin level rose to 11.2 gm/dL without hematinic therapy or blood transfusion. She was begun on anticoagulant therapy and remains clinically stable. CONCLUSION: We report a case of idiopathic aortitis with presumed diagnosis of anemia of chronic disease exhibiting a transient response towards steroid therapy post-valvuloplasty

Inherited bleeding disorders-experience of a not-for-profit organization in Pakistan

Patient registry is a powerful tool for planning health care and setting groundwork for research. This survey reports a detailed registry of inherited bleeding disorders (IBD) and their management at a not-for-profit organization in a developing country to form the basis for planning development and research. We reviewed medical records of patients with IBD from 8 hemophilia treatment centers of Fatimid Foundation located in various cities. Information collected included sociodemographic data, diagnostic tests, severity of hemophilia A and B, number of bleeding episodes per year, site and frequency of hemarthrosis, and seropositivity for viral diseases. We analyzed 1497 patients from November 1, 2015, to April 30, 2016. There were 1296 (87%) males and 201 (13%) females with a mean age of 24.5 (11) years (range, 6 months to 65 years). Hemophilia A constituted the bulk of IBD (848, 57%) followed by von Willebrand disease (172, 11%), hemophilia B (144, 10%), platelet function defect (106, 7%), and rare bleeding disorders (70, 5%). Mucocutaneous bleeding (1144, 76%) and hemarthrosis (1035 patients, 69%) were the main complications. There were 1026 (69%) patients who received only blood components for treatment of any bleeding episode while the remaining 464 (31%) were on combination therapy (blood components and factor concentrate). Seroreactivity for hepatitis C was frequent (28%), while hepatitis B (1%) and human immunodeficiency virus (0.01%) were less commonly seen. This study was an important step toward a patient registry in a hemophilia treatment center in Pakistan. Hemophilia A is the most common bleeding disorder and hepatitis C is the most frequent treatment-related complicatio

Quality indicators for critical INR results in a haemostatic laboratory

Background: The aim of this study was to evaluate the performance indicators for critical INR results. Methods: This is a clinical audit conducted at The Aga Khan University Hospital, Karachi from February to September 2010. Samples were received in the laboratory in 3.2% Na citrate tubes and PT/INR was performed (Sysmex ®1500, Innovin with ISI of 1.02). Critical value of INR was defined as more than 5.0. All critical INRs were rechecked and if the value remained unchanged, the patient was contacted and an in house questionnaire was filled. All patients irrespective of age and gender with critical INR were included. Exclusion criterion was defined as those patients who could not be contacted. Performance indicators were also identified. Results: Total INR values that were reported are 36861. Out of these, 287(0.77%) had INR above 5.0 (or 7/1000 tests). There were 211 evaluable patients out of which 81 (38.4%) were males and 130 (61.6%) were females. Majority of the patients were between the ages of 60 to 80 years. Warfarin intake accounted for critical INR in 94.3% patients and valvuloplasty was the commonest indication (48%). Only 7% had bleeding on Warfarin therapy while majority (84%) had no adverse effects. Conclusions: Critical INR should be communicated at priority basis. Performance indicators should be monitored quarterly. Females, older age group and patients with valvuloplasty are at higher risk of developing critical INR. Key words: Critical INR, Performance indicator, Clinical audi

Response and Adverse Effects of Nilotinib in Imatinib-resistant Chronic Myeloid Leukemia Patients: Data From a Developing Country

PURPOSE: The purpose of this study was to determine the frequency of major cytogenetic response (MCyR) and adverse events with nilotinib in adults with imatinib-resistant Philadelphia chromosome-positive chronic myeloid leukemia (CML).Methods: This is a descriptive cross-sectional study conducted at The Aga Khan University Hospital in Karachi from October 17, 2010, to October 17, 2011. A cytogenetic assessment using fluorescent in situ hybridization was performed on peripheral blood before initiation of treatment and 6 months after treatment with nilotinib. The frequency of adverse effects was assessed at 6 months, and the patient overall survival was calculated after 3 years.FINDINGS: This study enrolled 82 imatinib-resistant patients. The mean (SD) patient age was 38.9 (12.2) years. There were 62 patients (75.6%) in chronic phase, 15 patients (18.3%) in accelerated phase, and 5 patients (6%) in blast crisis phase. At 6 months cytogenetic studies were available for 40 (52%) of 77 patients, and MCyR was observed in 31 (77.5%) of 77 patients. The patients in chronic phase had the highest frequency of MCyR (n = 27 [87%] of 31). We observed 6 deaths (7.3%), and the overall survival at 3 years was 92.7%. There was isolated thrombocytopenia in 12 patients (15.6%). The most frequent nonhematologic adverse events were myalgia and headache.IMPLICATIONS: The nilotinib response rates were higher in chronic phase patients, and the most common adverse events were thrombocytopenia, myalgia, and headache

Quality indicators for critical international normalized ratio measurements in a hemostatic laboratory

Objective: This study aimed at reviewing the quality indicators for reporting critical international normalized ratio (INR) in a coagulation laboratory.DESIGN AND Methods: This is a retrospective study conducted at Aga Khan University Hospital, hemostatic laboratory from February 2010 till January 2011. Critical INR was defined as ≥5.0. All critical INRs were rechecked and results were communicated to the doctor or patient. Two quality indicators monitored were % of results communicated to the patient/doctor and % of results that remained critical after re-testing.Results: During the study period, a total of 59,980 INRs were reported. Of these 376 or 0.6% were critical. Successful communication of critical results to the doctor or patient was achieved in 275/376 (73.1%). Overall 353 or 94% (343 initial and 10 re-draw) samples had critical INR on repetition. Twenty five patients of the 240 warfarinized patients with critical INR had mild bleeding. No life threatening bleeding was observed in any patient.CONCLUSION: We observed poor communication for notifying critical INR results during the study period. Routine repeat analysis of critical INR did not alter results in majority of samples. The study assisted in improving communication in subsequent years. Further work is needed to establish evidence based upper and lower cutoff of critical INR. Effect of replicate analysis on turnaround time and accuracy of results needs evaluation

Monitoring and reporting transfusion reactions as a quality indicator – a clinical audit

Background and Objective: This audit was conducted as a part of a quality assurance activity to assess the frequency of receiving completely filled out blood transfusion reaction forms which were accompanied by the required samples. Once this information is known, we will elevate the bar each year to achieve 100% compliance. The sub-aim was to evaluate the frequency of the reported transfusion reactions. Materials and Methods: The study was conducted from 1st April 2010 to 30th April 2011. The information was evaluated and the frequency of receiving completely filled blood transfusion reaction forms was assessed. The variables identified were the type of transfusion reaction, the blood component transfused, the health care personnel filling the form, and whether there was legible handwriting and a completely filled form. Transfusion reactions were reported as a percentage of the total number of units transfused. Results: During the study period, 17,880 packed red cells, 13,200 platelets, 13,620 fresh frozen plasma and 2256 cryoprecipitate were transfused and 106 transfusion reactions (0.23%) were reported. Of these, febrile non hemolytic transfusion reaction was the most common (47%), the majority caused by packed red cells. Conclusion: Eighty-four percent of the transfusion reaction forms were completely filled as per our criteria. Febrile non hemolytic transfusion reactions were the most common reactions reported

Bone marrow aplasia in haemophilia A with hepatitis C and human immunodeficiency virus co-infection — a case report

Haemophilia A is an inherited bleeding disorder caused due to the deficiency of factor VIII. This case report of a 17-year-old HA boy co-infected by hepatitis C (HCV) and human immunodeficiency virus (HIV) followed by bone marrow aplasia seeks to address the key clinical question of the causality and management of bone marrow aplasia in a resource-constrained setting. Our patient developed pancytopenia which prompted the diagnosis and management of HCV and HIV. Bone marrow biopsy revealed severe aplasia. He was treated with highly active antiretroviral therapy (HAART). Two years later, he developed septic arthritis and haemarthrosis of the elbow and knee joints. He underwent arthrotomy of the knee joint. The patient expired, postoperatively, due to septic shock. This case underlines the need for truly universal access to virally inactivated replacement therapy to prevent complications secondary to infections transmitted by transfusion