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Paravertebral extramedullary hematopoiesis associated with improvement of anemia in congenital dyserythropoietic anemia type II

✍ Scribed by Gilles Lugassy; Joseph Michaeli; Noam Harats; Eugene Libson; Eliezer A. Rachmilewitz

Publisher
John Wiley and Sons
Year
1986
Tongue
English
Weight
329 KB
Volume
22
Category
Article
ISSN
0361-8609

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✦ Synopsis

Thoracic masses resulting from extramedullary hematopoiesis developed in two sisters of Moroccan origin with congenital dyserythropoietic anemia type I1 (HEMPAS). In one patient, the diagnosis of extramedullary hematopoiesis was confirmed histologically. The appearance of extramedullary foci of hematopoiesis mimicking mediastinal tumors has not been previously described in HEMPAS. These masses result from persistent erythropoietic stimulation associated with chronic hemolytic anemia. In both patients, detection of the asymptomatic masses was preceded by normalization of hemoglobin levels. Thus unexpected correction of a chronic refractory anemia associated with the appearance of mediastinal masses might be the heralding manifestation of an effective extramedullary hemopoiesis.

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