Extramedullary hematopoiesis in β-thalassemia major patient: a case report and review of the literature

Keikhaei, Bijan; Purrahman, Daryush; Marashi, Batool; Moezzi, Meisam; Mahmoudian-Sani, Mohammad Reza

doi:10.1007/s12308-022-00506-7

Cited by 2 publications

(2 citation statements)

References 20 publications

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“…Hematopoiesis is the production and maturation of elements of blood, which typically takes place in the vertebrae, ribs, and marrow of long bones in adults, while in the fetus, the principal sites of hemopoiesis are the liver, spleen, and yolk sac [1]. Few hematopoietic stem cells (HSCs) circulate in the spleen and peripheral blood normally [4]. When these primary sites in the adult fail like in a few blood disorders and the patients do not receive indicated blood transfusions, to overcome the resultant anemia, the bone marrow gets ramped up leading to osteoporosis and deformities in bone [1][4].…”

Section: Discussionmentioning

confidence: 99%

Radiological Features of Extramedullary Hematopoiesis in a Young Male With Beta-Thalassemia: A Case Report

Suryadevara,

Mishra,

Shetty

et al. 2024

Cureus

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The formation of the blood elements and their maturation is called hematopoiesis. In adults, this typically takes place in the bone marrow of vertebrae, ribs, and long bones. In contrast, during fetal development, the primary sites of hematopoiesis are the spleen, liver, and the yolk sac. This process of hematopoiesis, when it occurs in sites other than the bone marrow, is called the extramedullary hematopoiesis (EMH). Extramedullary hematopoiesis usually happens in patients with blood disorders like sickle cell disease and thalassemia, where there is failure of hematopoiesis in the primary sites. Here, we present a young male with beta-thalassemia who presented with shortness of breath and palpitations for one month. This manuscript discusses the imaging findings of the EMH in our patient.

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Section: Discussionmentioning

confidence: 99%

Radiological Features of Extramedullary Hematopoiesis in a Young Male With Beta-Thalassemia: A Case Report

Suryadevara,

Mishra,

Shetty

et al. 2024

Cureus

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“…Meanwhile, a recent systematic review estimated it to be evident in less than 1% of patients with transfusion‐dependent thalassemia (TDT) 2 . This dichotomy is likely related to the early aggressive management of TDT due to it being symptomatic, while there will be a delay in detecting patients with NTDT 3 . The management of EMH was always dependent on the severity of the symptoms, the clinical condition of the patient, and the EMH location, with options including transfusion, chemotherapy with hydroxyurea (HU), surgery, and radiation therapy as the EMH is considered radiosensitive 4 …”

Section: Introductionmentioning

confidence: 99%

Luspatercept's use in a patient with transfusion‐dependent beta‐thalassemia and intrathoracic extramedullary hematopoiesis (EMH)

Seijari,

Alshurafa,

Yassin

2024

Clinical Case Reports

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Key Clinical MessageThis case report and literature review examine the use of a relatively novel agent in a transfusion‐dependent beta‐thalassemia patient with extramedullary hematopoiesis (EMH). It examines the benefits and risks associated with its use and reviews the available literature while highlighting the drug's results in our patient with a higher risk profile.AbstractBeta thalassemia can be complicated by EMH, which causes different symptoms based on location and size. Luspatercept is a new agent approved for transfusion‐dependent thalassemia and Non‐transfusion‐dependent thalassemia (NTDT). Still, its use in patients with EMH was not well studied, and literature showed an increased risk of EMH expansion or development of new masses after its use. We discuss, in this case, the results of luspatercept treatment in a patient with transfusion‐dependent thalassemia who is considered high risk for its use due to the patient's specific characteristics (history of symptomatic intrathoracic EMH, previous splenectomy, refusal to use antithrombotic medications). While also highlighting the benefits of using luspatercept regarding decreasing the iron overload and improving hemoglobin levels and examining how it was used safely to manage a transfusion‐dependent thalassemia patient with an extramedullary hematopoiesis mass with no adverse events of note.

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Extramedullary hematopoiesis in β-thalassemia major patient: a case report and review of the literature

Cited by 2 publications

References 20 publications

Radiological Features of Extramedullary Hematopoiesis in a Young Male With Beta-Thalassemia: A Case Report

Radiological Features of Extramedullary Hematopoiesis in a Young Male With Beta-Thalassemia: A Case Report

Luspatercept's use in a patient with transfusion‐dependent beta‐thalassemia and intrathoracic extramedullary hematopoiesis (EMH)

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