title: [Hemorrhagic parapneumonic effusion in a 64 year-old patient as the first symptom of hemophilia B].
abstract: Hemophilia B is an inherited, X chromosome-linked disease. It is usually diagnosed in childhood, sometimes in adolescence. The commonest symptoms include spontaneous or post-traumatic bleeding into the joints and/or muscles, as well as mucosal bleeding. Respiratory symptoms are rarely reported. We present the case of a 64 year-old man in whom bloody parapneumonic effusion (hemothorax) was the first symptom of hemophilia B. The reason for prolonged activated partial thromboplastin time (APTT) found on admission has not been elucidated. Since antibiotic therapy and pleural tube thoracostomy with intrapleural streptokinase were found to be ineffective, video-assisted thoracic surgery was performed with the right lung decortication. Post-operative treatment was complicated by massive pleural bleeding requiring two subsequent thoracotomies. Additional blood tests revealed factor IX deficiency and resulted in hemophilia B being diagnosed. The presented case proves that hereditary bleeding disorders may be diagnosed even in late adulthood. Intrapleural bleeding related to pneumonia and pleural inflammation might be the first presenting symptom. Hemophilia should be considered as a potential cause of APTT prolongation, even in an elderly patient with atypical presentation. Explaining the reason for APTT prolongation before the surgical procedure could have allowed to avoid severe bleeding in the described patient.
authors: Katedra i Klinika Chorób Wewnetrznych, Pneumonologii i Alergologii Warszawskiego Uniwersytetu Medycznego. mgrabczak@yahoo.com
authors: Elzbieta M
authors: EM
authors: Grabczak
authors: Rafał
authors: R
authors: Krenke
authors: Magdalena
authors: M
authors: Jeleńska
authors: Iwan
authors: I
authors: Komarow
authors: Urszula
authors: U
authors: Ambroziak
authors: Adam
authors: A
authors: Fangrat
authors: Ryszarda
authors: R
authors: Chazan
mesh_terms: D017668:Age of Onset; D002836:Hemophilia B / Q000150:complications / Q000175:diagnosis* / Q000000981:diagnostic imaging / Q000601:surgery*; D006491:Hemothorax / Q000175:diagnosis* / Q000000981:diagnostic imaging / Q000209:etiology / Q000601:surgery*; D006801:Humans; D008297:Male; D008875:Middle Aged; D010996:Pleural Effusion / Q000175:diagnosis* / Q000000981:diagnostic imaging / Q000209:etiology / Q000601:surgery*; D011859:Radiography; D020775:Thoracic Surgery, Video-Assisted / Q000379:methods; D016896:Treatment Outcome