Revista de investigación de la sangre y enfermedades hematológicas

Management of neuroendocrine tumour of the appendix in a patient with moderate haemophilia B: a rare coincidence

Beatriz Oliveira Sousa*, Diogo Carrola Gomes, Alexandra Santos, Margarida Antunes and Maria Joao Diniz

Haemophilia B is a rare X linked congenital bleeding disorder characterized by a deficiency in coagulation Factor IX (FIX). Its estimated prevalence is 5 cases per 100.000 male births. In haemophilia, the bleeding events severity usually correlates with the degree of the clotting factor deficiency. Patients with moderate haemophilia (1-5%) endure prolonged bleeding with minor trauma or surgery and occasional spontaneous bleeding. We report the case of a thirty four year old male with moderate haemophilia B (no FIX inhibitors) and acute onset of mild right iliac abdominal pain, persistent for 24 hours and associated with loss of appetite. No history of recent abdominal trauma. No systematic prophylactic therapy. The patient was diagnosed with acute appendicitis and underwent laparoscopic appendectomy. Histopathology revealed an appendix neuroendocrine tumour and the patient was scheduled for robotic assisted laparoscopic right hemicolectomy. During his hospital stay, under cautious haematological controls, he progressed favourably with gradual resolution of the hemoperitoneum and abdominal wall hematoma, haemoglobin rise and decreased need for factor IX replacement therapy.