Incidence of factor FVIIIC deficiency in live male infants undergoing circumcision in South West, Nigeria.

A prominent evidence of inherited bleeding disorder in newborn males is excessive post-circumcision bleeding. Male circumcision in Nigeria is the rule rather than the exception. Male siblings of some of the Nigerian haemophiliacs consequently died from severe post-circumcision bleeding. The aim was to determine the incidence of inherited factor VIIIc (FVIIIc) deficiencies in live male infants undergoing circumcision in South West, Nigeria. The study population was 244 male infants drawn from University College Hospital and Our Lady of Apostles Catholic Hospital, Oluyoro, Ibadan. Pre-circumcision prothrombin time, activated partial thromboplastin time and FVIIIc levels were determined. Clinical features of inherited bleeding disorder particularly family history of bleeding diathesis, history of cephalhaematoma and bleeding from the umbilical stump in neonatal life were determined with the aid of a questionnaire. Only one of the mothers (0.4%) gave a family history of bleeding disorder. A history of excessive bleeding from the umbilical stump post delivery was obtained in three (2%) of the patients. Five (2%) other subjects had cephalhaematoma post delivery. Two of the subjects (0.8%) had prolonged activated partial thromboplastin time. The factor VIIIc level was between 31% and 49% in 16.1%, while 1.6% of the neonates had levels between 20% and 26%. This study detected four of the 244 (1.64%) neonates with FVIIIc deficiency, suggestive of either mild haemophilia or von Willebrand's disease. A larger study (including family studies) will be required, so as to arrive at the exact incidence of both haemophilia A and vWD in live male infants in Nigeria.

Myelomatosis: a case report of an unusual presentation.

BACKGROUND: Multiple myeloma (MM) is a clonalexpansion of plasma cell characterized by skeletal dissemination of malignant plasma cells resulting in the production of homogenous monoclonal immunoglobulin termed the monoclonal (M) protein. MM is the most prevalent cancer after non-Hodgkin's lymphoma and is responsible for 2% of all cancer deaths. Several unusual presentation of MM have been described in the literature. Many serial reports have documented progression of Plasmacytoma to MM, or relapsed into MM after radiation therapy. STUDY DESIGN: This is a case report of a 36 yr old man with a huge fungating right clavicular mass, with a histological of plasmacytoma (anaplastic variant). RESULT: The patient's tumour was resistant to radiotherapy, terminating as multiple myeloma, with rapid progression to death within 19 months post diagnosis. CONCLUSION: This report helps to validate the rapid progression of the anaplastic plasmacytoma variant to disseminated myeloma, and indicates the poor response to radiation therapy. Our patient showed the important poor prognostic features in some plasmacytomas that are characterized by rapid progression to MM, namely tumour size > 5 cm, young age and spinal cord compression.

Case report: extra pulmonary tuberculosis in sickle cell disease.

In apparently healthy individuals, tuberculosis (TB) affects mainly the lungs however, worsening immune status tend to predispose to an increased tendency for extra-pulmonary tuberculosis (EPTB). We report the case of a 22 year old known sickle cell anaemia (HBS) female student with three-month history of bilateral hip pain, weight loss and swelling of the left hip with multiple discharging sinuses, paraplegia and recurrent fever. There was no preceding history of trauma. Full Blood Count (FBC) revealed leukocytosis with neutrophilia, monocytosis, thrombocytosis and Packed Cell Volume (PCV) of 23%. Erythrocyte sedimentation rate (ESR) was 120mm/Hr (Western Green) and retroviral screening was negative. Bacteriologic culture of the discharging sinuses grew Escherichia coli and Staphylococcus aureus, both sensitive to sparfloxacin. Smear microscopy for acid-fast-bacilli (AFB) was negative. Chest X-Ray was reported normal but X-Ray of the pelvis showed loss of L4/L5 disc space and appearances suggestive of avascular necrosis of the femoral heads. Clinical and haematological profile of the patient started to improve by the second month on therapeutic trial of anti-TB regimen. She had nine-month course of therapy and later discharged to physiotherapy clinic. Management of EPTB requires a high index of clinical suspicion and well-equipped laboratory to support the diagnosis. Therefore, this case report highlights the need to upgrade TB-diagnostic facilities in this environment.