Background: The clinical prospects of hydroxyurea therapy in the management of sickle cell disease (SCD) require evaluation in the Nigerian setting to develop indigenous guidelines. This survey examines the pattern of hydroxyurea therapy, its clinico-haematologic benefits and safety profile in Nigerian SCD subjects. Methods: A cross sectional pilot survey was carried out among 60 adult SCD subjects over 3 months. Data on clinical phenotypes, relevant haematological parameters and details of hydroxyurea therapy were obtained using a structured questionnaire through an interview process and case file review. Results: The median age was 30 years. Thirty-four (56.7%) of the subjects are aware of hydroxyurea therapy in SCD. Twenty-four (40%) SCD patients had previously used hydroxyurea. Only 4 subjects were fully compliant. Reasons for non-compliance included poor knowledge and lack of funds. In particular, hydroxyurea reduced leucocyte count and increased mean red cell volume (MCV) in compliant subjects. Conclusion: Hydroxyurea use is low among Nigerian SCD subjects despite its proven efficacy/clinical prospects in the developed nations. Large scale multicenter studies and clinical trials are needed to form a basis for developing standard local treatment protocol for its use.

Blood transfusion is associated with the development of alloantibodies. Routine blood grouping for multi-transfused patients with SCA should be extended to include other blood group antigens in addition to Rhesus D and ABO antigens.

Sixty patients were prospectively studied with the aim of analyzing the clinical and laboratory features and outcome of patients diagnosed with chronic lymphocytic leukaemia (CLL) in a major referral center in Niger Delta region of Nigeria for 10 years (1995-2005). The peripheral blood, bone marrow cytology, clinical features and stage at diagnosis were studied. Treatment modalities, response to treatment and survival outcome of the patients were analysed. Sixty patients (15 men and 45 women) were seen, with female preponderance (M : F ratio,1 : 3). The CLL incidence was 36.4% of total leukaemias. The median age was 56 years with peak age group at 51-60 years while 15% were below 40 years. Major clinical findings include lymphadenopathy (91.7%), anaemia (58.3%), abdominal swelling (58.3%), and splenomegaly (50%) with 53 patients (88.3%) presenting as International (Binet) stage B and C while only seven patients (11.7%) were seen in stage A. The least clinical presentation includes Richter's syndrome in 3.3% of cases and herpes Zoster skin manifestations in two patients (3.3%). There was a strong association between the blood counts at diagnosis and outcome of therapy. The 2-year survival for young (<55 years) and older (>55 years) CLL patients was 27.2% and 28.9%, respectively, which is still very poor because of a number of strong limiting factors. CLL is not rare in Southern Nigeria and its presentations are similar to cases seen worldwide. Contrary to existing literature a female predominance was observed in this study with majority of patients seeking medical intervention late. It is therefore recommended that future research into the genetic make up/HLA typing of patients of African descent is needed to clarify some of the differences observed.

Banked blood is a limited resource in Nigeria. We sought to evaluate factors that may further limit the effective utilisation of donor blood in a tertiary hospital in Benin City. The records of the blood transfusion unit of the hospital were studied to identify the methods of blood procurement and utilisation from January 1, 2000 to December 31, 2002. A total of 11,021 units of blood were received in the blood transfusion unit within this period out of which 1491 (13.5%) donor blood samples were found unfit for transfusion and, hence, discarded. Commercial blood donation accounted for 95.3%, compared to 4.7% from replacement and volunteer donors. Commercial blood donation was a major risk factor for likely disposal of donor blood (x 2 = 74.3, p < 0.0001, OR = 21.1, 95% CI = 7.8-56.7). Expired units of blood with low PCV were discarded for lack of infrastructure to fractionate and store them. Over N 0.8million (US$6000.00) is wasted annually on discarded units of donor blood mainly from commercial donors. A policy on blood procurement to include subgroup selection of donors and improved funding of blood banking services may enhance efficient and effective utilisation of donor blood.

Background:The prevalence of anemia in HIV/ AIDS patients is high, with a multitude of possible etiologies; autoimmune hemolytic anemia (AIHA) in HIV/AIDS patients has been associated with a poor prognosis when treated with red cell transfusion. Our aim was to demonstrate the frequency of AIHA in a cohort of adult Nigerian HIV/AIDS patients and to see if the presence or not of AIHA is related to the severity of the disease with regards to the CD4 counts and the presence or absence of opportunistic infections. Method: Ninety-eight adult patients with HIV infection were screened for the presence of AIHA using the packed cell volume (PCV), direct antiglobulin test (DAT) and reticulocyte count (RC). Results: The frequency of AIHA was 3.06%, 36.74% of our study population were anemic; 11.22% had a positive DAT. Mean RC was 2.22 +/ 0.90 for all the patients. There was no statistically significant difference in the PCV of patients that had positive and negative DAT. There was no correlation between the presence of AIHA, use of ART, presence of opportunistic infections or CD4 counts. Conclusion:We conclude that in spite of the low frequency of AIHA in HIV/AIDS patients, the fact that most patients will respond to standard treatment makes it imperative to screen HIV/AIDS patients with anemia for the presence of AIHA. Again since HIV/AIDS patients with AIHA may have a fatal reaction to red cell transfusion, we suggest that anemic patients with HIV/AIDS in non-emergency situations be screened for the presence of AIHA before receiving red cell transfusions when indicated. Key words: Autoimmune, anemia, hemolysis, HIV/AIDS RésuméCulture générale: Il y a trop de fréquence d'anémie parmi les malades sero-positifs ou les malades atteints de sida, souvent accompagne d'un grand nombres d'aetiologies: anémie haemolylique autoimmuno (AHAI) parmi les malades sero-positifs est directement lie a une mauvaise pronostic, surtout quand on fait le traitement avec une transformation du globule rouge. Notre objectif est de démontrer la fréquence de (AHAI) parmi un nombre d'adultes nigérians sero-positifs ou qui sont atteints du sida. C'est aussi de vérifier si la présence ou l'absence de (AHAI) est lie directement lie a la gravite de la maladie, de la maladie surtout quand il s'agit de la numération CD4 et de la présence ou absence des infections opportunistic. Modalité: À l'aide de l'analyse directe antiglobulin (ADA) et la numération réticulocyte (NR) et le packed cell volume (PCV) ; quatre-vingt-dix-huit malades sero-positifs, ont passé un test de dépistage pour vérifier la présence de (AHAI) Résultat: La fréquence de (AHAI) était 3.06%, 36.74% de notre population était anémique ; 11.22% était positif de l'analyse antiglobulin.Pour la numération reticulocy, la moyenne était 2.22+\-0.90 pour tous les malades, on n'a trouvé aucune différence qui est statistiquement significatif parmi les malades Autoimmune haemolytic anemia in HIV patients. Olayemi E. et al.Page | 73 positif de (ADA) et négatif de (ADA). Il n y avait aucun rappor...

This study was aimed at evaluating the iron status of multiple blood transfused sickle cell anaemia (SCA) patients in Benin City, Nigeria. A total of 86 subjects participated in the study, comprising of 30 multiple transfused SCA subjects, 30 of rarely transfused SCA subjects and 26 of age and sex matched healthy control subjects. Serum ferritin was determined by enzyme linked immuno sorbent assay technique. Serum iron and Total iron binding capacity was determined by spectrophometric method. The mean serum ferritin concentration was elevated in the sickle cell anaemia patients whose multiple transfusions (MT) are more than those who were rarely transfused (RT) as compared with the control groups (p < 0.001). There was a positive correlation between the serum ferritin and the number of units of blood transfused (r = 0.719, p = 0.000). This study revealed that a high level of serum ferritin, percentage transferrin saturation and a reduction in total iron binding capacity were observed in sickle cell anaemia patients who received ≥3 units of packed cells in one year.

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In a prospective study using standard methods, haemorheological parameters were assessed in 10 sickle cell anaemia patients in pain crises. Patients were investigated for possible changes in determinants of rheology in the course of painful episodes: blood samples were taken for plasma fibrinogen concentration (PFC); plasma viscosity (PV); haematocrit (Hct), whole blood viscosity (WBV) and the erythrocyte sedimentation rate (ESR) using standard methods. Samples were collected on presentation to the emergency unit and daily for 4 consecutive days. Whole blood viscosity and plasma fibrinogen concentration were significantly higher at onset of crises when compared with baseline values (p < 0.01 and p < 0.0001), respectively. Plasma fibrinogen and blood viscosity peaked within 24 h of onset of crisis and started declining 48-72 h later. These parameters approached baseline values by the 4th day of painful crisis.There was no significant change in the haematocrit and the erythrocyte sedimentation rate during the period of painful crisis despite change in whole blood viscosity and fibrinogen. In conclusion, this study showed elevated haemorheological parameters in sickle cell anaemia patients in VOC, it also demonstrated an acute rise in these parameters at onset of crisis and a return to almost baseline levels within 96 h of onset of painful episodes.