Hematological and Clinical Findings Predictive of Mortality in Italian Patients with Sickle-Cell Disease: A Case-Control Study.

Background: the number of cases with Sickle-Cell Disease (SCD) registered in Italy at 2000 was 928. Six-hundred-twenty-two (67%) of these were with Sickle-Cell/βthalassemia. Moreover, while the main causes of death in Afro-American patients with SCD is well known, we do not have many informations in Italian subjects. For this reason, we carried ahead a case-control study in this group of patients.

Aim of the study: the evaluation of the main hematological and clinical parameters predictive of mortality.

Materials and Methods: clinical and hematological findings of 14 SCD died patients were compared with a SCD alive control population homogeneous for age, sex, phenotype and number of painful crises per year. Two alive control subjects were studied for each died patient.

Results: the main causes of death were: Acute Chest Syndromes (ACS) (n°6), Cardiovascular Diseases (n°3),Hepatic Failure (n°1),Hodgkin Lymphoma (n°1), Stroke (n°1), Lung Cancer (n°1), Surgery Complication (n°1). The main correlated hematological findings with mortality were Hb (gr/dl) levels (p=<.05), WBC count (10³ / ml) (p= <.05), ferritin levels (ng/ml) (p=<.01). The main correlated clinical features were: the number of crises >3/year (Odd Ratio= 1.64; p= <.01), previous stroke (Odd Ratio= 1.53; p= <.01), HU treatment (Odd Ratio=<.01; p=<.01), leg ulcers (Odd Ratio=1.46;p=<.01).

Conclusions: these data suggest that the main causes of death in Italian patients with SCD are similar to those reported for Afro-American subjects (M.H. Steinberg, JAMA, April 2, Vol. 289, N°13, 1645–1651). Particularly, the lack of HU treatment, anemia, high ferritin levels, number of crises per year and previous stroke are the most important predictive factors of mortality.