Clinical and laboratory factors associated with splenic dysfunction among patients with sickle cell disease in a malaria endemic region.

BACKGROUND: Although loss of splenic function is the expected natural course for individuals with sickle cell disease (SCD), factors such as high HbF and coexistence of alpha thalassemia may ameliorate this process. We evaluated factors associated with two surrogate markers of spleen dysfunction, namely Howell-Jolly bodies (HJBs) and argyrophilic inclusion (AI) red cell counts, among patients with SCD. METHODS: Cross-sectional data of 182 patients with SCD (median age 11 y; 1-45 y) and 102 normal controls (median age 12 y; 1-32 y) were evaluated. Blood tests including full blood count, serum chemistry and high-performance liquid chromatography were performed. The HJB and AI red cell counts were performed on peripheral blood smears. RESULTS: The percentages of HJB and AI red cells rose significantly with increasing age in the SCD group. On regression analysis, the frequency of HJB red cells associated positively with mean corpuscular hemoglobin (MCH) (ß=0.289; p=0.001) and negatively with HbF (ß=-0.259; p=0.002). The AI red cell counts also associated positively with MCH (ß=0.321; P=0.001) and negatively with HbF (ß=-0.242; p=0.020). CONCLUSIONS: Data from this study indicate that the negative association of HbF with both markers of splenic dysfunction among our patients with SCD residing in a malaria endemic region is similar to findings elsewhere of its ameliorating effect on splenic dysfunction.

Evaluation of two red cell inclusion staining methods for assessing spleen function among sickle cell disease patients in North-East Nigeria.

The loss of splenic function is associated with an increased risk of infection in sickle cell disease (SCD); however, spleen function is rarely documented among SCD patients in Africa, due partly to the non-availability of sophisticated techniques such as scintigraphy. Methods of assessing splenic function which may be achievable in resource-poor settings include counting red blood cells (RBC) containing Howell Jolly Bodies (HJB) and RBC containing silver-staining (argyrophilic) inclusions (AI) using a light microscope. We evaluated the presence of HJB-and AI-containing RBC as markers of splenic dysfunction among SCD patients in Nigeria. We prospectively enrolled children and adults with SCD in steady state attending outpatient clinics at a tertiary hospital in North-East Nigeria. The percentages of HJB-and AI-containing red cells were estimated from peripheral blood smears and compared to normal controls. There were 182 SCD patients and 102 healthy controls. Both AI- and HJB-containing red cells could be easily identified in the participants blood smears. SCD patients had a significantly higher proportion of red cells containing HJB (1.5%; IQR 0.7%-3.1%) compared to controls (0.3%; IQR 0.1%-0.5%) (P <0.0001). The AI red cell counts were also higher among the SCD patients (47.4%; IQR 34.5%-66.0%) than the control group (7.1%; IQR 5.1%-8.7%) (P < 0.0001). The intra-observer reliability for assessment of HJB- (r = 0.92; r2 = 0.86) and AI- containing red cells (r = 0.90; r2 = 0.82) was high. The estimated intra-observer agreement was better with the HJB count method (95% limits of agreement, -4.5% to 4.3%; P = 0.579).We have demonstrated the utility of light microscopy in the assessment of red cells containing-HJB and AI inclusions as indices of splenic dysfunction in Nigerian SCD patients. These methods can be easily applied in the routine evaluation and care of patients with SCD to identify those at high risk of infection and initiate appropriate preventive measures.