血液疾患と輸血ジャーナル

概要

Disease Severity Scores and Haemogram Parameters in Nigerian Sickle Cell Disease Patients

Emmanuel Okocha, Emmanuel Onwubuya, Charles Osuji, Gladys Ahaneku, Uche Okonkwo, Nancy Ibeh, John Aneke , Ebele Nwachukwu and Christian Onah

Background: Sickle cell disease (SCD) is associated with high mortality in Nigeria and the rest of sub-Saharan Africa; there is need to find easily available parameters that could predict disease severity and influence therapy.

Objective: To evaluate the haemogram of a population of SCD patients and correlate these with objective scores of disease severity.

Methods: Sixty (60) asymptomatic steady state (ASS) SCD patients in our clinic were randomly selected and interviewed with a questionnaire. Their haemogram was done using a 17 parameter, 3-part white cell differential, auto-analyser (KX 21N, Sysmex corporation, Chuo ku, Kobe, Japan) and objective severity scores calculated using a modification of the method proposed by Anyeagbu et al. Statistical analysis of data was done using Statistical Package for Social Sciences software, version 20 (SPSS Inc., IL, Chicago, USA), with significance assigned to p values less than 0.05.

Result: Of the 60 subjects assessed, severity scores were calculated for 49: 11 (22.4%), 31 (63.3%) and 7 (14.3%) met the criteria for mild, moderate and severe disease respectively. The haemogram parameters that were significantly correlated with disease severity were mean corpuscular haemoglobin concentration (MCHC), and white blood cell count (WBC), p=0.014, and 0.001 respectively. Haemoglobin concentration (Hb) and packed cell volume (PCV) were negatively correlated with disease severity (p=0.001).

Conclusion: In addition to already known haemogram parameters that affect SCD severity (such as WBC, Hb concentration, and PCV) MCHC also does same and can be manipulated by drugs and other kinds of therapy to ameliorate severity in patients.