Abstract 4204
A 58 year old male with a known clinical history of Passovoy defect was evaluated for a craniotomy for an olfactory meningioma. His family history revealed that this defect was present in multiple family members (siblings, parent and child). In the past, he had had several successful and several complicated surgeries. According to his past history, bleeding did not occur intra-operatively but typically 24-72 hours post-operatively. There was no evidence of mucosal bleeding, petechiae, rashes or ecchymosis on examination. There was no past history of spontaneous hemorrhage, epistaxis, hemoptysis, hematemesis, melena, petechiae or ecchymosis. Past surgical history revealed that when bleeding occurred and FFP was transfused post-operatively, the bleeding subsided. When Fresh Frozen Plasma (FFP) was transfused pre-operatively, intra-operatively and post-operatively there was no significant bleeding. Laboratory tests showed normal ProthrombinTime (PT), activated Partial Thromboplastin Time (aPTT), ThrombinTime (TT), Fibrinogen, Factor VIII and Factor XIII, vWFAntigen and vWF multimers. Platelet Aggregation studies were normal except for a decreased response to epinephrine. Thromboelastogram studies revealed a mild coagulation factor deficiency. Our workup did not show any of the common coagulation defects. There was no evidence of a prolonged Partial Thromboplastin Time (aPTT) as reported in the literature. Due to normal aPTT results (average 27.7 secs, range 25.5– 38.1 secs), mixing studies were not performed. Due to his significant past clinical history, the patient was transfused with Fresh Frozen Plasma (FFP) pre-operatively, intra-operatively and post- operatively for 72 hrs. The PT/aPTT and platelet counts remained within normal range throughout the patient's entire hospital stay with no evidence of bleeding post-operatively.
Passovoy Defect is a rare autosomal disorder and is a coagulation abnormality affecting the intrinsic coagulation system characterized by a prolonged aPTT. The defect is associated with a clinical bleeding tendency characterized by easy bruising and undue blood loss following trauma and/or surgery. Fresh Frozen Plasma appears to provide effective prophylaxis during surgery. The bleeding phenotype is variable. There may be no bleeding after major surgeries. Major bleeding after minor surgeries is not uncommon.
Our case represents a rare type of a defect in the intrinsic system that may not be encountered in the daily setting with a normal active Partial Thromboplastic Time (aPTT). The Passovoy Defect in our patient appears to be transmitted as an autosomal dominant trait. Despite a very significant clinical and family history, the aPTT was within normal range throughout his hospitaization. No diagnostic laboratory findings were noted. It has been reported that the prolongation of aPTT in some of the patients may be relatively mild. However this was not the case in our patient. We do not know whether our patient has a deficiency of an unknown coagulation factor or that our aPTT reagent (HemosIL SynthASIL Catalogue # 20006800, DiaPharma Group Inc, West Chester, OH, USA) was not sensitive to the Passovoy Defect.
No relevant conflicts of interest to declare.
Asterisk with author names denotes non-ASH members.
