J A Olaniyi1, U M Abjah. 1. Department of Haematology, University College Hospital, PMB 5116, Ibadan, Nigeria. johnniyi2001@yahoo.com
Abstract
BACKGROUND: Hepatobiliary and splenic complications (amongst others) are common in sickle cell disease (SCD) as a consequence of the progressive injury resulting from repeated sickling of HbS red blood cells. OBJECTIVE: To determine the degree and the frequency of persistent hepatomegaly and splenomegaly in relation to determinants of clinical severity in patients with SCD. METHODS: Two hundred and twenty SCD patients in clinical steady state were assessed for the presence and the size of palpably enlarged spleen and liver. Patients with hepatomegaly or splenomegaly were re-assessed after three months. Average size was recorded as well as the electrophoresis pattern, age at asessment, sex and the haematocrit. These were compiled over one year RESULTS: The mean age of the SCD patients was 24.7 (8.7) years. HbS and female subjects predominated, constituting 89% and 57% respectively. Females had statistically significantly higher mean age of years than males, (p < 0.001). Splenomegaly was present in 46 (21%) of SCD patients, of which 39 (84.8%) were HbSS, with a mean splenic size of 8.7 (7) cm. Out of the 220 SCD patients, hepatomegaly was present in 59%, with a mean size of 7.6 (6.5) cm. However, higher frequency and a significantly bigger size of hepatomegaly were found in patients with HbS than in those with HbSC. The mean age of 27.2 (10.9) years and the mean of PCV of 24.1 (4.9) in the 82 (37%) patients without hepatomegaly and splenomegaly were significantly higher (p = 0.001) than the general population of SCD patients studied. CONCLUSION: Hepatomegaly of varying sizes occurs commonly in patients with HbS and probably connotes a severe clinical course. The aversions that there is predominance of HbS, survival advantage of females and the fact that HbSC fair better than HbS remain true.
BACKGROUND:Hepatobiliary and splenic complications (amongst others) are common in sickle cell disease (SCD) as a consequence of the progressive injury resulting from repeated sickling of HbS red blood cells. OBJECTIVE: To determine the degree and the frequency of persistent hepatomegaly and splenomegaly in relation to determinants of clinical severity in patients with SCD. METHODS: Two hundred and twenty SCDpatients in clinical steady state were assessed for the presence and the size of palpably enlarged spleen and liver. Patients with hepatomegaly or splenomegaly were re-assessed after three months. Average size was recorded as well as the electrophoresis pattern, age at asessment, sex and the haematocrit. These were compiled over one year RESULTS: The mean age of the SCDpatients was 24.7 (8.7) years. HbS and female subjects predominated, constituting 89% and 57% respectively. Females had statistically significantly higher mean age of years than males, (p < 0.001). Splenomegaly was present in 46 (21%) of SCDpatients, of which 39 (84.8%) were HbSS, with a mean splenic size of 8.7 (7) cm. Out of the 220 SCDpatients, hepatomegaly was present in 59%, with a mean size of 7.6 (6.5) cm. However, higher frequency and a significantly bigger size of hepatomegaly were found in patients with HbS than in those with HbSC. The mean age of 27.2 (10.9) years and the mean of PCV of 24.1 (4.9) in the 82 (37%) patients without hepatomegaly and splenomegaly were significantly higher (p = 0.001) than the general population of SCDpatients studied. CONCLUSION:Hepatomegaly of varying sizes occurs commonly in patients with HbS and probably connotes a severe clinical course. The aversions that there is predominance of HbS, survival advantage of females and the fact that HbSC fair better than HbS remain true.