Massive pleural effusion as a presentation of extramedullary hematopoiesis among patients with non transfusion dependent beta-thalassemia
Keywords:
Thalassemia, Extramedullary hematopoiesis, Pleural effusionAbstract
Thalassemia is one of the most common hemoglobinopathies. Beta-thalassemia results from a decreased or absent production of the beta-globin chain of hemoglobin. Extramedullary hematopoiesis (EMH) is one of the complications of beta-thalassemia, primarily seen among non transfusion dependent patients. EMH predominately occurs in the spleen, liver and lymph nodes but has been reported in almost all organs. Despite this, EMH is rare regarding presented with serous effusions. We present a case report of EMH occurring in the pleura with pleural effusion in a patient with beta thalassemia together with discussion of the diagnostic approaches and treatment implications.
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Subahi EA, Ata F, Choudry H, Iqbal P, AlHiyari MA, Soliman AT, et al. Extramedullary haematopoiesis in patients with transfusion dependent β-thalassaemia (TDT): a systematic review. Ann Med. 2022;54:764-74.
Taher AT, Musallam KM, Karimi M, El-Beshlawy A, Belhoul K, Daar S, et al. Overview on practices in thalassemia intermedia management aiming for lowering complication rates across a region of endemicity: the OPTIMAL CARE study. Blood. 2010;115:1886-92.
Winichakoon P, Tantiworawit A, Rattanathammethee T, Hantrakool S, Chai-Adisaksopha C, Rattarittamrong E, et al. Prevalence and risk factors for complications in patients with nontransfusion dependent alpha- and beta-thalassemia. Anemia. 2015;793025:1-7.
Haidar R, Mhaidli H, Taher AT. Paraspinal extramedullary hematopoiesis in patients with thalassemia intermedia. Eur Spine J. 2010;19:871–8.
Bartlett RP, Greipp PR, Tefferi A, Cupps RE, Mullan BP, Trastek VF. Extramedullary hematopoiesis manifesting as a symptomatic pleural effusion. Mayo Clin Proc.1995;70:1161-4.
Koch M, Kurian EM. Pleural fluid extramedullary hematopoiesis case report with review of the literature. Diagn Cytopathol. 2016; 44:41-4.
Aessopos A, Tassiopoulos S, Farmakis D, Moyssakis I, Kati M, Polonifi K, et al. Extramedullary hematopoiesis-related pleural effusion: The case of β-thalassemia. Ann Thorac Surg. 2006;81:2037–43.
Gits HC, Lester SC, McGrath LA, Martenson JA. Complete response of pleural effusions caused by extramedullary hematopoiesis to low-dose, single fraction palliative radiation therapy: Case report and literature review. Adv Radiat Oncol. 2018;3:463-6.
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