[Severe sickle cell anemia revealed in an adult by medullary necrosis].

INTRODUCTION

Drepanocytosis, even severe, may only be discovered in adults further to severe complications.

OBSERVATION: A 44 year-old woman was hospitalised for a severe vaso-occlusive crisis, revealing a drepanocyte syndrome with heterozygote S-beta zero thalassemia. Evolution was marked by severe anaemia, the aregenerative nature of which, uncommon during vaso-occlusive crises and the absence of Parvovirus B19 infection, led to the diagnosis of medullar necrosis. Evolution was rapidly improved after transfusion of erythrocyte concentrations and symptomatic treatment of the pain.

COMMENTS: Medullar necrosis is a rare entity with multiple causes. In severe drepanocyte syndromes it is concomitant to a severe vaso-occlusive syndrome, resulting from medullar ischemia due to specific microvascular damage.