Blood transfusion services for patients with sickle cell disease in Nigeria.

BACKGROUND: Safe, timely red blood cell transfusion saves lives and chronic transfusion therapy (CTT) prevents or limits morbidities such as stroke, therefore improving quality of life of patients with sickle cell disease (SCD). METHODS: This questionnaire-based study assessed the ability of sickle cell centers in Nigeria to provide safe blood to patients with SCD between March and August 2014. RESULTS: Out of the 73 hospitals contacted, responses were obtained from 31. Twenty four (78%) hospitals were unable to transfuse patients regularly due to blood scarcity. Packed red blood cells were available in 14 (45%), while only one provided leukocyte-depletion. Most centers assessed donor risk and screened for HIV in 30 (97%), hepatitis B in 31(100%) and hepatitis C in 27 (87%) hospitals. Extended phenotyping and alloantibody screening were not available in any center. A quarter of the hospitals could monitor iron overload, but only using serum ferritin. Access to iron chelators was limited and expensive. Seventeen (55%) tertiary hospitals offered CTT by top-up or manual exchange transfusion; previous stroke was the most common indication. CONCLUSION: Current efforts of Nigerian public hospitals to provide safe blood and CTT fall short of best practice. Provision of apheresis machines, improvement of voluntary non-remunerated donor drive, screening for red cell antigens and antibodies, and availability of iron chelators would significantly improve SCD care in Nigeria.

Coexistence of JAK2 and BCR-ABL mutation in patient with myeloproliferative neoplasm.

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.

Management of AIDS-associated Kaposi's sarcoma in Nigerian children: a case series and review of literature.

INTRODUCTION: There is a paucity of published studies on the management and outcome of AIDS-associated Kaposi's sarcoma (AAKS) in African children. In this study, we reviewed the management and literature of AAKS in Nigerian children. PATIENTS AND METHODS: A prospective review of children aged 1 to 14 years and adolescents aged 15 to 18 years who presented with AAKS. Following clinical evaluation and resuscitation, patients were treated with highly active antiretroviral therapy (HAART). Stable patients were further treated with chemotherapy consisting of vincristine, doxorubicin, and bleomycin. Patients were monitored until death or loss to follow-up. RESULTS: There were 9 patients: 6 children and 3 adolescents. Three children had vertical transmission of HIV infection. Kaposi's sarcoma was the AIDS-defining disease in 5 patients. One patient was on HAART at the time of diagnosis. There were multiple skin lesions in all patients, and cervical lymph nodes and oropharynx were frequently affected. The CD4 counts at the time of AAKS diagnosis ranged 78 to 601 cells/ uL, mean of 317. Five patients had best palliative care. Three had anticancer chemotherapy, of which 2 were alive 4 years after diagnosis. Three patients died at the initial hospitalization 2 to 6 weeks after diagnosis. CONCLUSION: Children and adolescents with AAKS presented with generalized skin lesions and lymphadenopathy, which facilitated the diagnosis. The majority of the patients presented with advanced disease that was rapidly fatal. However, patients with good immunity may have a prolonged control of symptoms if treated with HAART and appropriate anticancer chemotherapy.