Arthritis as a presentation of acute leukemia

A 14 year old boy presented with 3 months history of low back pain as well as pain and swelling of both knee joints. The pain was inflammatory in nature. He was thoroughly evaluated but no underlying cause was detected. However, the boy was treated with NSAIDs and sulphasalazine. A few weeks later he developed anemia and took the blood transfusion. Then he developed fever and the joint pain re-appeared. On examination, the patient was found moderately anemic, positive bony tenderness and splenomegaly. Musculoskeletal examination revealed right knee joint effusion. The investigation revealed pancytopenia. Bone marrow morphology and immunophenotyping revealed acute lymphoblastic leukemia. We treated the patient according to modified UKALL-2003 protocol and patient condition improved and arthritis disappeared

Haemoglobin E/Beta Thalassaemia- A Study in BSMMU

Background: Thalassaemias and haemoglobinopathies have been found sporadically in every ethnic group and geographic region, they occur with particularly high frequency from the shores of the Mediterranean and Africa through the Middle East, the Indian subcontinent, Burma and Southeast Asia. Objective: The study was designed to find out the incidence of HbE/beta thalassaemia in BSMMU. Method: A total of 700 patients suspected to have been suffering from haemolytic anaemia were included in the study. Patients having evidence of haemolysis in peripheral blood film were selected for reticulocyte count and haemoglobin electrophoresis in cellulose acetate membrane at pH 8.6. Result: The study group of 700 patients underwent Hb-electrophoresis of which only 52 (7.4%) cases were diagnosed as HbE/beta thalassaemia. Out of 52 cases, 34 (65.4%) patients were found symptomatic and the remaining 18 (34.6%) patients were asymptomatic. Out of 34 symptomatic cases of HbE/beta thalassaemia, only 14 cases needed blood transfusion. Among the 14 patients, only 8 patients needed more than 10 units of transfusion and 6 patients needed frequent transfusion that is two units of blood in every month. Conclusion: It is clearly evident from the present and other studied so far carried out in this Indian subcontinent and South-East Asia that hereditary haemolytic anaemia due to globin chain defects are quite common in this region, especially in Bangladesh and are responsible for considerable morbidity and mortality. Key words: Thalassaemia; Haemoglobinopathies; HbE/beta thalassemia. DOI: 10.3329/bsmmuj.v2i2.4762 BSMMU J 2009; 2(2): 78-8

Frequency of consanguineous marriage among the thalassaemia major patients in Bangabandhu Sheikh Mujib Medical University

Thalassaemia is a commonly occurring hereditary disorder. There is a high prevalence of thalassaemia disease in South-East Asia as well as Bangladesh. It is an autosomal recessive disorder, so consanguineous marriage is a very important factor for this disease. Mutated beta globin gene of haemoglobin from both parents is responsible for this disease to occur. But when the number of thalassaemia carriers is miserably high, then only avoidance of consanguineous marriage can’t prevent the birth of children with thalassaemia major. So, this cross sectional study was conducted among 120 diagnosed (by haemoglobin electrophoresis or high performance liquid chromatography) thalassaemia major patients in the Department of Haematology, Bangabandhu Sheikh Mujib Medical University, Dhaka from July 2019 to May 2020. History from each patient was taken and blood samples were collected from their parents to confirm carrier state by haemoglobin electrophoresis. Blood samples were also collected from patients and their parents toperform complete blood count and peripheral Blood Film. This study showed the mean age of the participants was 15±9.34 and 73 (60.8%) patients were male and 47 (39.2%) were female. About, 71% of the study population lived in urban area, 81 (67%) patients were transfusion dependent thalassaemia patients. 15% thalas- saemia major patients had the history of consanguinity of their parents. Among the parents of the thalassaemia major patients, 62.5% were Beta thalassaemia trait (heterozygous state), 25.83% parents were Hb E trait ( heterozygous state), 7.08% parents were suffering from Beta Thalassaemia Major(homozygous state) and 11 (4.58%) parents were suffering from Hb E/Beta Thalassaemia which is a compound heterozygous state. Though consanguinity is a very influential factor, but the most important causative factor is the presence of high number of thalassaemia carrier in the population. So, it’s a burning issue for the nation to perform a routine carrier screening for all rather than focusing on consanguinity only, to lessen the burden of thalassaemia disease in Bangladesh. BSMMU J 2022; 15(1): 25-2

Arthritis as a presentation of acute leukemia

A 14 year old boy presented with 3 months history of low back pain as well as pain and swelling of both knee joints. The pain was inflammatory in nature. He was thoroughly evaluated but no underlying cause was detected. However, the boy was treated with NSAIDs and sulphasalazine. A few weeks later he developed anemia and took the blood transfusion. Then he developed fever and the joint pain re-appeared. On examination, the patient was found moderately anemic, positive bony tenderness and splenomegaly. Musculoskeletal examination revealed right knee joint effusion. The investigation revealed pancytopenia. Bone marrow morphology and immunophenotyping revealed acute lymphoblastic leukemia. We treated the patient according to modified UKALL-2003 protocol and patient condition improved and arthritis disappeared

Effectiveness, safety and economic viability of daycare versus usual hospital care management of severe pneumonia with or without malnutrition in children using the existing health system of Bangladesh: a cluster randomised controlled trialResearch in context

Summary: Background: We aimed to define clinical and cost-effectiveness of a Day Care Approach (DCA) alternative to Usual Care (UC, comparison group) within the Bangladesh health system to manage severe childhood pneumonia. Methods: This was a cluster randomised controlled trial in urban Dhaka and rural Bangladesh between November 1, 2015 and March 23, 2019. Children aged 2–59 months with severe pneumonia with or without malnutrition received DCA or UC. The DCA treatment settings comprised of urban primary health care clinics run by NGO under Dhaka South City Corporation and in rural Union health and family welfare centres under the Ministry of Health and Family welfare Services. The UC treatment settings were hospitals in these respective areas. Primary outcome was treatment failure (persistence of pneumonia symptoms, referral or death). We performed both intention-to-treat and per-protocol analysis for treatment failure. Registered at www.ClinicalTrials.gov, NCT02669654. Findings: In total 3211 children were enrolled, 1739 in DCA and 1472 in UC; primary outcome data were available in 1682 and 1357 in DCA and UC, respectively. Treatment failure rate was 9.6% among children in DCA (167 of 1739) and 13.5% in the UC (198 of 1472) (group difference, −3.9 percentage point; 95% confidence interval (CI), −4.8 to −1.5, p = 0.165). Treatment success within the health care systems [DCA plus referral vs. UC plus referral, 1587/1739 (91.3%) vs. 1283/1472 (87.2%), group difference 4.1 percentage point, 95% CI, 3.7 to 4.1, p = 0.160)] was better in DCA. One child each in UC of both urban and rural sites died within day 6 after admission. Average cost of treatment per child was US$94.2 (95$184.8 (95% CI, 178.6 to 190.9) for DCA and UC, respectively. Interpretation: In our population of children with severe pneumonia with or without malnutrition, >90% were successfully treated at Day care Clinics at 50% lower cost. A modest investment to upgrade Day care facilities may provide a cost-effective, accessible alternative to hospital management. Funding: UNICEF, Botnar Foundation, UBS Optimus Foundation, and EAGLE Foundation, Switzerland