Anemia Heart Disease in a Patient with Suspected Non-Transfusion Dependent Thalassemia, a Case Report

Authors

  • Agustinus Alfred Gunawan General Practitioner, Waikabubak General Hospital, West Sumba, Indonesia
  • Rambu Farah Effendy Internal Medicine Doctor, Waikabubak General Hospital, West Sumba, Indonesia

DOI:

https://doi.org/10.59188/eduvest.v4i6.1215

Keywords:

Thalassemia, Cardiac Complication, Limited Access

Abstract

Case Report. A female, 22 years old, came to the emergency room at Waikabubak General Hospital with complaints of weakness and pallor that had worsened since 1 week ago. Patients feel shortness of breath after heavy work, which improves after resting, nausea, and heart pounding. The patient has some previous medical history and familial history, Physical examination showed facies cooley appearance, short stature with anemic conjunctiva, icteric sclera, and hepatosplenomegaly. Laboratories showed microcytic hypochromic anemia with elevated liver enzymes. Peripheral blood smear showed microcytic anemia with anisocytosis and poikilocytosis with target cells. CXR showed cardiomegaly and USG showed hepatosplenomegaly. Echocardiography showed LV eccentric hypertrophy, LA dilatation, mild AR, mild MR, mild TR with a low probability of pulmonary hypertension, normal systolic function, grade I LV diastolic dysfunction, and ejection fraction 55,2%. This patient was treated with omeprazole injection, blood transfusion, oxygen supplementation, oral lisinopril, and bisoprolol. Over two weeks showed improvement in symptoms and laboratory. Discussion. The diagnosis is made from anamnesis, physical examination, and limited supporting examination. Determining the exact diagnosis, and management of the underlying disease and complication based on patient symptoms and simple laboratory results. Conclusion. Cardiac involvement in thalassemia is a major complication. Management of complications is difficult in limited resources facilities. Education and pre-marital screening are required to reduce the incidence of underlying disease.

References

Aessopos, A., Kati, M., & Farmakis, D. (2007). Heart disease in thalassemia intermedia: a review of the underlying pathophysiology. Haematologica, 92(5), 658–665.

Akiki, N., Hodroj, M. H., Bou-Fakhredin, R., Matli, K., & Taher, A. T. (2023). Cardiovascular Complications in β-Thalassemia: Getting to the Heart of It. Thalassemia Reports, 13(1), 38–50.

Aliyeva, G., Asadov, C., Mammadova, T., Gafarova, S., & Abdulalimov, E. (2018). Thalassemia in the laboratory: pearls, pitfalls, and promises. Clinical Chemistry and Laboratory Medicine (CCLM), 57(2), 165–174.

Angastiniotis, M., & Lobitz, S. (2019). Thalassemias: an overview. International Journal of Neonatal Screening, 5(1), 16.

Bajwa, H., & Basit, H. (2022). Thalassemia.[Updated 2021 Nov 5]. StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing.

Cappellini, M.-D., Cohen, A., Eleftheriou, A., Piga, A., Porter, J., & Taher, A. (2013). Guidelines for the clinical management of thalassaemia.

Khan, A., & Rehman, A. U. (2022). Laboratory Evaluation of Beta Thalassemia.

Kremastinos, D. T., Farmakis, D., Aessopos, A., Hahalis, G., Hamodraka, E., Tsiapras, D., & Keren, A. (2010). β-thalassemia cardiomyopathy: history, present considerations, and future perspectives. Circulation: Heart Failure, 3(3), 451–458.

Lasocki, S., Pène, F., Ait-Oufella, H., Aubron, C., Ausset, S., Buffet, P., Huet, O., Launey, Y., Legrand, M., & Lescot, T. (2020). Management and prevention of anemia (acute bleeding excluded) in adult critical care patients. Annals of Intensive Care, 10, 1–12.

Sarkar, K., Pramanik, N., Goswami, R. P., Mandal, P. K., Chowdhury, P., Jana, K., & Das, S. (2020). Correlation of Liver Function Test with Different Age and Sex Group and with Ferritin Level in Thalassemia. Indian Journal of Public Health Research & Development, 11(6), 452–457.

Shah, R., & Agarwal, A. K. (2013). Anemia associated with chronic heart failure: current concepts. Clinical Interventions in Aging, 111–122.

Wahidiyat, P. A., Sari, T. T., Rahmartani, L. D., Iskandar, S. D., Pratanata, A. M., Yapiy, I., Setianingsih, I., Atmakusuma, T. D., & Lubis, A. M. (2022). Thalassemia in Indonesia. Hemoglobin, 46(1), 39–44.

Wood, J. C. (2009). Cardiac complications in thalassemia major. Hemoglobin, 33(sup1), S81–S86.

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Published

2024-06-20