Extramedullary haematopoiesis in patients with transfusion dependent ß-thalassaemia (TDT): a systematic review.

INTRODUCTION: Around 5% of the world's population is expected to have some degree and type of thalassaemia. Beta thalassaemia (BT) occurs due to a deficient production of the beta-globin chain of haemoglobin. Extramedullary haematopoiesis (EMH) is one of the complications of BT, mainly observed in minor/intermedia subtypes. EMH is the production of blood cells outside the marrow as a compensatory response to longstanding hypoxia. Due to chronic transfusions, it is not expected in patients with beta-thalassaemia major (BTM). However, there are increasingly reported cases of EMH in BTM. The incidence of EMH in BTM is thought to be <1%. We aim to pool the available data and provide cumulative evidence on the occurrence of EMH in BTM patients. METHODS: This is a systematic review of case reports, series, and retrospective studies that presented data on the occurrence of EMH in BTM patients. Data were recorded and analyzed in Microsoft Excel 2016 and SPSS 26. The protocol has been registered in PROSPERO: CRD42021242943. RESULTS: Data from 253 cases of EMH in BTM patients were extracted with a mean age of 35.3 years. Mean haemoglobin at presentation with EMH was 8.2 mg/dL. Lower limb weakness was the most common presenting feature (N = 23) (paraspinal EMH). Magnetic resonance imaging (MRI) was the most widely used diagnostic modality (226). Overall, blood transfusion was the commonest reported treatment (30), followed by radiotherapy (20), surgery (15), hydroxyurea (12), steroids (6), and exchange transfusion (2). An outcome was reported in 20% of patients, all recovered, except one who died as a result of nosocomial infection. CONCLUSION: EMH is rare in BTM and can occur in any organ system with varied clinical features. MRI can effectively diagnose EMH, and conservative management has similar results compared to invasive treatments. Larger studies, focussing on outcomes may enhance guidelines on preventive and therapeutic strategies for managing EMH in BTM.KEY MESSAGESExtramedullary haematopoiesis is a rare complication in beta thalassaemia. Although it is more common in non-transfusion dependent thalassaemia, increasingly reported cases suggest a higher prevalence of EMH in TDT than what is known before.There are no clear guidelines on the management of EMH in TDT, with reported patients showing similar outcomes with conservative invasive treatment modalities.More extensive and preferably prospectively designed studies are required focussing on the management of EMH and its outcomes in patients with TDT to formulate evidence-based guidelines.

Breast Cancer Screening Practices in a Tertiary Care Center in the State of Qatar: A Cross-Sectional Survey.

INTRODUCTION: Breast cancer is the most common cancer in females. In Qatar, mortality related to breast cancer came in third after lung cancer and leukemia. In this study, we aim to comprehensively evaluate the rate of internal medicine residents and faculty compliance with breast cancer screening in Hamad Medical Corporation (Doha, Qatar), as well as to identify barriers and facilitators that could potentially augment changes to enhance physician-led cancer screening. METHODS: A cross-sectional web-based survey was distributed among internal medicine physicians between December 2018 and March 2019 at a tertiary medical centre. It focused on the knowledge, attitude, and practice of physicians regarding breast cancer screening guidelines and explored potential barriers and proposed solutions. Chi-square and t-test statistics were used to draw conclusions where appropriate. RESULTS: A total of 158 physicians responded to the survey, with a response rate of 61%. 75.9% were postgraduate trainees. Around three-quarters of the physicians mentioned that they would recommend breast cancer screening for their age-appropriate average-risk patients. There was a statistically significant difference between the trainees, consultants, and specialists regarding the modality of choice, where the majority of the trainees opted mammogram every 2 or 3 years while 44.4% of the consultants indicated yearly self-breast exam (p<0.001). The percentage of survey participants who rarely to never offer breast cancer screening in the outpatient settings was 37.8%. Unclear pathway (40%) and lack of time in clinic/ward rounds (26.5%) were the major reported barriers for cancer screening. CONCLUSION: In the current era of personalized medicine, physicians should be more oriented to local guidelines to provide optimal care to their patients. While the attitude towards breast cancer screening is positive, the overall compliance with the national recommendations is sub-optimal. Further initiatives and intervention programs are required to promote the breast cancer screening in Qatar.

Mycoplasma pneumoniae associated with severe autoimmune hemolytic anemia: case report and literature review.

We report a case of severe hemolytic anemia following Mycoplasma pneumoniae infection in a 29-year-old male patient who was treated with azithromycin. Direct Coombs' test was strongly positive and the cold agglutinin titer was high, with anti-I specificity. Antimycoplasma antibody titer by complement fixation was high 1:10,240. The patient was discharged after 12 days of hospitalization in good health. He remains clinically well with no recurrence of jaundice.