Multiple myeloma in Nigeria: a multi-centre epidemiological and biomedical study

Introduction: Myelomatosis is a malignant proliferation of plasma cells in the bone marrow, with  relatively high prevalence in African populations. Variation in genetic mutations has been observed in individual patients and may be responsible for differences in disease pattern and treatment outcomes. This study described the presentations and treatment outcomes of multiple myeloma in nigerian. Methods: The data was obtained retrospectively from the case notes of 135 patients diagnosed with multiple myeloma from eight tertiary health institutions across the six geopolitical zones of Nigeria from 2005 to 2014. Data analysis was carried out using SPSS 17.0.Results: The predominant presentations were bone pain in 97 (74%), nephropathy in 47 (35.9%) and pathological fractures in 58 (44.3%). Sixty-seven percent (67%) of the patients were less than 60 years, and 35% had Bence Jones proteinuria. The overall survival beyond 6 months was 91.3%, mean duration of survival rate was 7.4 months. Majority (66.2%) were on Melphalan alone or on melphalan-containing combinations. A higher packed cell volume (PCV) and total serum protein levels at presentation were associated with increased survival, p=0.033 and 0.036, respectively.Conclusion: This study portrayed the importance of detail investigation on the causes of bone pain and anaemia in person's aged 40 years and above. There is a high prevalence of nephropathy in this cohort of patients which needs to be further investigated. Majority of the patients, though < 65 years of age were placed on melphalan-containing combinations, which foreclosed chances of future autologous bone marrow transplantation.Key words: Multiple myeloma, Nigeria, presentation, treatment modalitie

Avascular necrosis significantly impairs quality of life in sickle cell disease

Introduction: Quality of life (QoL) assessment has become an integral component of the assessment of the holistic care of patients with chronic diseases, including sickle cell disease (SCD). Objective: To evaluate the quality of life in patients with SCD managed in our centre. Patients and Methods: Eighty consecutive patients with confirmed hemoglobin SS or SC were recruited. Age and sex-matched volunteers served as controls. Ethical approval was obtained from the Institutional Review Board and all participants gave informed consent. Information on socio-demographic, quality of life and clinical variables, including the presence of complications were recorded in a modified version of the WHO Quality of Life Brief version (WHOQOL-BREF) questionnaire. Data was analyzed using Microsoft Excel and SPSS 17 computer softwares. Descriptive statistics were used to represent socio-demographic variables while the Student t-test was used to explore relationship between the variables and the quality of life domains. Results: Significantly fewer participants with SCD are married compared to their age- and sex-matched controls (P = 0.01). Similarly, participants with SCD scored significantly lower in the physical and psychological domains as well as in overall QoL and general health domains compared to controls (P = 0.001). Avascular necrosis of the femur significantly affected the overall QoL and general health of participants with SCD, respectively while the means of the QoL assessment domains were not significantly different in participants with SCD with and without complications, except in the general health domain (P < 0.001). Conclusion: Avascular necrosis of the femoral head significantly affects overall QoL in participants with SCD

Thromboprophylaxis Reduced Venous Thromboembolism in Sickle Cell Patients with Central Venous Access Devices: A Retrospective Cohort Study

Sickle cell disease (SCD) induces a chronic prothrombotic state. Central venous access devices (CVADs) are commonly used for chronic transfusions and iron chelation in this population. CVADs are an additional venous thromboembolism (VTE) risk factor. The role of thromboprophylaxis in this setting is uncertain. The objectives are: (1) to determine whether thromboprophylaxis reduces VTE risk in SCD patients with CVAD and (2) to explore characteristics associated with VTE risk. We identified adults with SCD and CVAD intended for chronic use (≥3 months) at two comprehensive SCD centers. Thromboprophylaxis presence; type; intensity; and patient-, catheter-, and treatment-related VTE risk factors were recorded. Among 949 patients, 49 had a CVAD (25 without and 24 with VTE prophylaxis). Thromboprophylaxis type and intensity varied widely. Patients without thromboprophylaxis had higher VTE rates (rate ratio (RR) = 4.0 (95% confidence interval: 1.2–12.6), p = 0.02). Hydroxyurea was associated with lower VTE rates (RR = 20.5 (6.4–65.3), p p = 0.02, and RR = 58.2 (15.0–225.0), p < 0.001, respectively). Thromboprophylaxis, hydroxyurea, and CVAD subtype were independently associated with VTE. The potentially protective role of thromboprophylaxis and hydroxyurea for VTE prevention in patients with SCD and CVAD merits further exploration