Radiotherapy combined with erythropoietin for the treatment of extramedullary hematopoiesis in an alloimmunized patient with thalassemia intermedia

Abstract

We report a patient with thalassemia intermedia who developed a mediastinal syndrome due to the growth of paravertebral hematopoietic masses in the posterior mediastinum. Because the patient did not receive blood transfusions due to alloimmunization, she was first treated with human recombinant erythropoietin (escalating low-moderate doses) to recover hemoglobin levels, then in association with radiotherapy to prevent a worsening of her anemia. The mean Hb level dramatically increased and peaked at week 11, to 83 g/l, and remained unchanged before and after radiotherapy (81 versus 78 g/l). Immediately after radiotherapy extramedullary hematopoiesis volume decreased by 16.4%.

Study of red blood cell alloimmunization risk factors in multiply transfused thalassemia patients: role in improving thalassemia transfusion practice in Fayoum, Egypt

“…Results from a number of studies have demonstrated various frequencies of alloantibodies formation in multiply transfused patients ranging from 5% to 30 % . Development of RBC autoantibodies can result in clinical hemolysis and in cross‐matching problems …”

Platelet alloimmunization after long‐term red cell transfusion in transfusion‐dependent thalassemia patients

“…RBC autoimmunization appears less frequently, but it can result in clinical hemolysis and cause difficulty in cross‐matching blood. Patients with autoantibodies may have a higher transfusion rate and often require immunosuppressive drugs, a splenectomy, or alternative treatments 4‐6 …”

Painful Spinal Cord Compression as a Complication of Extramedullary Hematopoiesis Associated with β-Thalassemia Intermedia

“…Management options include hypertransfusion, radiotherapy, surgery or a combination of these modalities. Recently hydroxyurea or erythropoietin in combination with radiotherapy was described [4,7].…”