Hemorrhagic parapneumonic effusion in a 64 year-old patient as the first symptom of hemophilia B

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 p...

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Bibliographic Details
Published in:	Pneumonologia i alergologia polska Vol. 77; no. 3; pp. 320 - 326
Main Authors:	Grabczak, Elzbieta M, Krenke, Rafał, Jeleńska, Magdalena, Komarow, Iwan, Ambroziak, Urszula, Fangrat, Adam, Chazan, Ryszarda
Format:	Journal Article
Language:	Polish
Published:	Poland 2009
Subjects:	Age of Onset Hemophilia B - complications Hemophilia B - diagnosis Hemophilia B - diagnostic imaging Hemophilia B - surgery Hemothorax - diagnosis Hemothorax - diagnostic imaging Hemothorax - etiology Hemothorax - surgery Humans Male Middle Aged Pleural Effusion - diagnosis Pleural Effusion - diagnostic imaging Pleural Effusion - etiology Pleural Effusion - surgery Radiography Thoracic Surgery, Video-Assisted - methods Treatment Outcome
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Summary:	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.
Bibliography:	ObjectType-Case Study-2 SourceType-Scholarly Journals-1 ObjectType-Feature-4 content type line 23 ObjectType-Report-1 ObjectType-Article-3
ISSN:	0867-7077