Bone marrow necrosis in sickle cell disease : a clinical & pathological study

الملخص EN

Bone marrow necrosis (BMN) is a rare clinical-pathological entity.

It is mostly associated with post-mortem changes.

Ante-mortem existence of BMN is quite rare and usually indicates a poor prognosis.

However, its association with non-hematological malignancies seems not to be as poor prognostic feature as in post-mortem changes.

One of the most commonly disorders associated with BMN is sickle cell disease (SCD), which is common among people in Basrah province.

The current study probably can put light on the incidence of such pathology among those with SCD, its most clinical presenting features and its relation with specific sickle disease genotypes.

The incidence of BMN in this study was 4.9 %.

The mostly encountered features with wide spread necrosis were bone pains, fever, and pallor.

Peripheral blood showed a florid leucoerythroblastic picture with reticulocytosis and leucopenia in another case with focal necrosis.

On comparison with those patients without BMN, patients with BMN showed a significantly lower Hb concentration, higher Hb S concentration, lower Hb F concentration, smaller splenic size, higher number of irreversibly-sickled cells and more frequent painful crises during their life.

Those results were compatible with some observations and contradict with other.

However, there is no previous study conducted in Iraq to compare with.