A novel syndrome complicating sickle cell disease : generalized bone marrow necrosis

Abstract EN

Three adults with sickle cell disease (SCD) presented in the span of less than two years with pancytopenia, signs of Disseminated Intravascular Coagulation (DIC) and no localizing pain to suggest vaso-occlusive crisis (VOC).

Bone marrow examination showed almost complete necrosis of the whole series of marrow cells, thus explaining the pancytopenia.

Some necrotic leukocytes were noted also in the peripheral blood.

The condition was self-limiting with marrow regeneration occurring in all three patients after transfusions of red cells and platelets.