Bulky extramedullary hematopoiesis is not a rare complication of congenital dyserythropoietic anemia

Hermann Heimpel (1) , Ulrich Dührsen (2) , P. Hofbauer (3) , V. Rigamonti-Wermlinger (4) , Ernst-Dietrich Kreuser (5) , Klaus Schwarz (6) , Max Solenthaler (4) , S. Pauls (7)

1 Medizinische Klinik III
2 Klinik für Hämatologie
3 Fachpraxis für Lungenkrankheiten
4 Abteilung für Hämatologie and Hämatologisches Zentralaboratoratorium
5 Klinik für Onkologie
6 Institut für klinische Transfusionsmedizin und Immungenetik und Institut für Transfusionsmedizin
7 Klinik für Diagnostische und Interventionelle Radiologie

Bulky extramedullary hematopoiesis, usually detected in the thorax by imaging techniques, is a well-known complication in many types of congenital anemias. Here, we describe 12 cases of congenital dyserythropoietic anemia with extramedullary hematopoiesis which was always located in the paravertebral space of the thoracic spine and in other paraspinal regions in a few cases. All bulks were originally detected in chest radiographs and confirmed by imaging techniques such as computed tomography and/or magnetic resonance imaging. In some cases, thoracotomy was performed for suspected malignancy. Although the true prevalence is not known, paravertebral masses in patients with CDA of any type are not uncommon and should be the first differential diagnosis considered when masses adjacent to the spine are detected in this disorder.

Mots clés: Congenital dyserythropoietic anemia; Extramedullary hematopoiesis; Imaging techniques

Hermann Heimpel, Ulrich Dührsen, P. Hofbauer, V. Rigamonti-Wermlinger, Ernst-Dietrich Kreuser, et al.. Bulky extramedullary hematopoiesis is not a rare complication of congenital dyserythropoietic anemia. Annals of Hematology, 2009, 88 (10), pp.937-941. ⟨10.1007/s00277-009-0735-5⟩. ⟨hal-00535048⟩