The tailor-made treatment in a particular case of pulmonary hypertension in thalassaemia intermedia: a case report

Background Pulmonary hypertension (PH) is a haemodynamic condition, secondary to different causes. Thalassaemia may lead to PH of different origin and needs a comprehensive analysis to be correctly characterized and possibly treated. Case summary We present a case study of a patient with a non-transfusion-dependent thalassaemia and a previous diagnosis of group 5 PH. A complete diagnostic assessment led to a specific diagnosis of chronic thromboembolic PH. Thus, we were able to start a specific therapy with riociguat that provided an improvement in terms of haemodynamic, imaging, and functional status. Discussion A correct characterization and treatment of PH are essential in order to change the patient’s prognosis. Chronic thromboembolic PH is a treatable cause of PH in thalassemic patients and should be investigated.

Pseudothrombocytosis in a Case of Beta-thalassaemia Intermedia Masquerading as Myeloproliferative Neoplasm

: Finding of spurious thrombocytopenia is a common occurrence in clinical practice whereas pseudothrombocytosis is very uncommon event. Despite several technical advancements in automated haematology analysers, a careful peripheral smear examination remains standard examination in cases of discrepancy of platelet counts. We are presenting a case of beta thalassaemia intermedia who had pseudothrombocytosis, which was falsely labelled as myeloproliferative neoplasm before the patient visited our haematology clinic.

Demographic and Treatment Status of Thalassaemia Patients in a Tertiary Hospital in Bangladesh

Introduction: Thalassaemia is now effectively treated with adequate blood transfusion and iron chelation. The disease process itself and iron overload from blood transfusions together produce multiple complications. Objective: Due to increased life expectancy of thalassaemia patients it is important to point out demographic profiles and clinical history related factors that may vary due to numerous causes. Therefore, objective of this study may lead to know the obstacles to access treatment and to find solutions to prevent complications in our socioeconomic background. Methodology: Three clinical types of thalassaemia were assessed in Bangabandhu Sheikh Mujib Medical University, Dhaka, Bangladesh. Total 109 subjects with thalassaemia were included in the study according to the inclusion criteria. They were distributed as thalassaemia major (33 subjects), thalassaemia intermedia (34 subjects) and thalassaemia minor (42 subjects). The patients were asked questions regarding demographic status and some clinical histories through a questionnaire. After compilation of the data statistical analysis was done accordingly. Results: The mean age of the subjects in years were 22.7 in thalassaemia major, 22.09 in thalassaemia intermedia and 22.5 in thalassaemia minor with nearly equal gender distributions. Students constituted 27.27% among thalassaemia major, 32.35% among thalassaemia intermedia and 30.95% among thalassaemia minor. The participants were educated up to primary level by 51.52% in thalassaemia major, 47.06 % in thalassaemia intermedia and 57.14 % in thalassaemia minor. Most of the subjects were Muslims and over 75% of the subjects came from outside the Dhaka the city. The mean number of life time transfusions were 105.33 in the patients of thalassaemia major, 33.85 in the intermedia and 0.76 in the minor. Iron chelation were done in 48.48% of thalassaemia major and 17.64% of thalassaemia intermedia at some point of their treatment. Splenectomy was done in 5 (15.15%) of thalassaemia major and 2 (5.8%) in thalassaemia intermedia patients. Before enrolment in the study 9.09% thalassaemia major, 50% intermedia and 83% minor subjects never visited haematologists or haematology OPD. Conclusion: Most of the subjects were at their working age and students and had to take treatment from inter-district facilities. Majority of the patients with thalassaemia intermedia and minor never consulted haematologists. Transfusion frequency was high with inadequate iron chelation.