Long COVID Syndrome Presenting as Hyperferritenemia Associated Toxic Encephalopathy in a Deferoxamine Compliant Patient of Beta Thalassemia

Ferritin overload is a frequent problem encountered in patients with beta thalassemia amounting to various complications. One of rare but important complication of ferritin overload is ferritin overload induced toxic encephalopathy. Serum ferritin has been recognised as a important marker of inflammation and cytokine storm in COVID19.We present a case of Long COVID syndrome presenting as toxic encephalopathy in a case of beta thalassemia in spite of the patient being compliant to desferroxamine therapy.This report highlights the synergistic effect of Severe Acute Respiratory Syndrome Coronavirus. 2(SARS CoV 2) mediated neuroinflammation through direct viral invasion and the release of inflammatory cytokines including ferritin resulting in toxic encephalopathy in a beta thalassemia major patient who was already prone to develop hyperferritenemia.Ferritin functions as a pro-inflammatory cytokine and it’s level increases in both thalassemia and COVID-19 and the index case presented here had both the conditions predisoposing him to develop toxic encephalopathy even after being compliant to deferoxamine therapy. It is important to recognise and treat this condition in order to prevent mortality and morbidity in patients of beta thalassemia who contract COVID19.

Beta Thalassemia Major with Diabetes Mellitus: A Case Report

Thalassemia results from defects in normal hemoglobin production, and represents the most common inherited anemia worldwide. Diabetes is a complication of b-thalassemia major. We report a case of Diabetes mellitus in a known case of beta thalassemia major. Patient had undergone Splenectomy 1 year back. Patient is taking chelating agent Defasirox 1000mg orally once a day in the morning. Family history reveals, born through third degree consanguineous marri age. The patient was then subjected for laboratory examination reveals BSL was high, urine ketone 2+,urine sugar 3+, ABG was normal, HbA1c was 13 & 3 month old report of serum ferritin 1200 ng/dl. Multidisciplinary management was instituted. Blood sugar level got controlled over subcutaneous insulin. Patient may have landed in Diabetic ketoacidosis but was promptly diagnosed & treated. This case is presented for its rarity. As the life expectancy of patients with thalassaemia increases, this will also expose our patients potentially to many more years of hyperglycaemia and diabetes. Sustaining metabolic control and controlling cardiovascular risk factors will be critical in the future for preventing complications due to diabetes.

Earlier detection of glomerular dysfunction in β-thalassemia major patients

Chronic transfusions program in β-thalassemia patients will inevitably lead to iron overload with a significant morbidity and mortality. Glomerular filtration rate (GFR) is progressively declined in relation to iron overload as well as chronic anemia. Objective is to define levels of Cystatin C in transfusion dependent β-thalassemia major patients as a sensitive marker for detection of earlier glomerular dysfunction in addition to understand the effect of iron overload, chelating therapy and hepatitis infection. A cross sectional study conducted at Al-Basrah Hemoglobinopathy Centre for the period from September 2017 to January 2018 to enroll 75 β-thalassemia major patients. Data collected included duration of the disease, total transfusion requirement, details of chelation therapy and its therapeutic index. In addition to blood urea, serum creatinine and Cystatin C with estimated GFR (eGFR). The mean Cystatin C was 1.075 mg/L where 66.6% of patients had abnormal renal function which is higher proportion than those with renal (42.6%) detected according to serum creatinine level Cystatin C was significantly higher in patients who received desferrioxamine as compared to those received deferasirox (P=0.007), in accordance with GFR which is significantly higher in patients receiving the latter chelation therapy (P=0.009). A significant inverse relationship between Cystatin C, and GFR, while positive relationship between ferritin and Cystatin C (P=0.0001, 0.001 respectively). Cyctatin C is better for detection and monitoring of glomerular dysfunction in B thalassemia major patient which is already not uncommon complications for the disease and iron chelation therapy.