The World Health Organisation (WHO) classifies myeloproliferative neoplasm (MPN) into BCR-ABL positive chronic myeloid leukaemia (CML Ph ⁺ ) and Ph⁻ MPN. The JAK2 V617F mutation is specific for Ph⁻ MPN and occurs in approximately 50% of primary myelofibrosis. Earlier reports suggest that the occurrence of JAK2 and BCR-ABL mutations are mutually exclusive. However, recent reports have documented the coexistence of BCR-ABL and JAK2 mutation in the same patient mostly following treatment with tyrosine kinase inhibitors (TKIs). We thus report a 60-year-old male with atypical clinical and laboratory features of MPN and the presence of both BCR-ABL and JAK2 Mutations.

Zidovudine induced pure red cell aplasia: A case report

Introduction: Microalbuminuria (MA) an early marker of glomerular dysfunction is also associated with increased risk of cardiovascular disease (CVD) in the general population, and is frequently seen among patients with human immunodeficiency virus (HIV) infection. Measurement of MA is not routinely done in many HIV/acquired immunodeficiency syndrome (AIDS) patients in our environment, especially those on antiretroviral therapy (ART). This study was to determine the prevalence of MA among HIV/AIDS patients in Zaria. Subjects and Methods: Screening for MA was carried out in this cross-sectional study involving 101 HIV/AIDS patients on ART. Patients with hypertension, diabetes, renal disease, pregnancy, and with features suggestive of malignancy were excluded from the study. Urinary albumin and creatinine, serum creatinine, glucose, cholesterol, and CD4 count were assayed. The data obtained were analyzed using the statistical software package for the social sciences version 16.0 (SPSS 16.0). Result: The mean values for urine albumin, urine creatinine, and albumin creatinine ratio (ACR) were 9.35 mg/L ± 1.32 mg/L, 6.50 mmol/L ± 0.35 mmol/L and 1.77 mg/mmol ± 0.34 mg/mmol, respectively. Twenty-one (20.8%) patients were found to have MA (ACR 3-30 mg/mmol) with remaining 80 (79.2%) having normoalbuminuria (ACR <3 mg/mmol). Among patients with MA, 6 (5.9%) had estimated creatinine clearance of <60 ml/min. There was a statistically significant association (P < 0.001) between ACR and age in microalbuminuric patients. Conclusion: Prevalence of MA is high among HIV/AIDS patients on ART in Zaria. Routine measurement of microalbumin in urine is suggested for early identification of renal disease and CVD and possibly reduces morbidity and mortality among patients with HIV/AIDS infection.

Background: Immune thrombocytopenic purpura (ITP) is a rare bleeding disorder that may remit spontaneously. Life-threatening bleeding may require transfusion support, steroids, and other immunosuppressive therapy or splenectomy. Objective: To review the clinical presentation and laboratory features of ITP at Ahmadu Bello University Teaching Hospital (ABUTH), Zaria, Nigeria. Subjects and Methods: A retrospective analytic study of case notes and bone marrow (BM) records of patients diagnosed with ITP at Haematology Department, ABUTH, Zaria, from January 1, 2004, to December 31, 2012. Results: There were nine cases (six females, three males), aged 6–20 (mean 11.11) years. The presentations were epistaxis 8 (88.9%), purpura 4 (44.4%), gum bleeding 4 (44.4%), menorrhagia 2 (22.2%), and intracranial hemorrhage (ICH) 1 (11.1%). Only 1 (11.1%) had clinical splenomegaly. Platelet count of <20 × 10⁹/L was found in 4 (44.4%) while 6 (66.7%) had packed cell volume of <25%. All the nine cases had BM megakaryocytic hyperplasia. Six patients had blood transfusion support while 7 (77.8%) patients received oral prednisolone therapy with time to cessation of bleeding of 12–16 (mean of 8) weeks. One case had spontaneous remission while another had anti-D due to relapse after steroid therapy; this resulted in transient rise in platelet counts. None had other immunosuppressive therapy or splenectomy. Six (66.7%) cases were lost to follow-up after achieving remission and one died of ICH. Conclusion: ITP is not common in our center though its clinical presentations are varied. However, prednisolone and blood transfusion therapy are central to the management of these patients with favorable outcome.

Background: Sickle cell disease (SCD) is an autosomal recessive haemoglobin disorder, affecting about 7.74 million individuals worldwide, but it is more prevalent among Africans and Asians. SCD is characterised by many complications, and it is a major health issue in Nigeria, the country with the largest burden of the disease globally. This work aims to present the design and implementation of electronic registries (ER) for SCD in a tertiary hospital in Nigeria. Methods: Registry design was initiated during a staff exchange programme within the ARISE initiative (EU grant agreement no. 824021). Ethical approval was obtained, and paper records were retrieved and transferred into one adult and one paediatric database, developed with Microsoft Access. Results: Data from 2659 SCD patients were entered in the ERs, including 698 (26.3%) adults and 1961 (73.7%) children. There were 287 (41%) male adults, 404 (58%) female and 7 (1%) patients whose gender was missing. There were 1041 (53.1%) male children, 906 (46.2%) female and 14 (0.7%) whose gender was missing. Information on phenotype was available for 2385 subjects, and most of them (2082, 87.3%) were SS. The most prevalent SCD-related complication was painful events (26.6% in adults and 68.7% in children, considering valid cases). Conclusions: About 60% of SCD patients in the centre were included in the ERs providing useful, hands-on recommendations for future ER design in SCD. These ERs might be an appropriate tool for collecting and analysing SCD patients’ data.