INHIBITOR TO FIBRINOGEN WITHOUT PROPHYLACTIC THERAPY IN A PATIENT WITH CONGENITAL AFIBRINOGENEMIA. A CASE REPORT
(Abstract release date: 05/19/16)
EHA Library. Patıroglu T. 06/09/16; 134651; PB1751
Prof. Dr. Turkan Patıroglu
Contributions
Contributions
Abstract
Abstract: PB1751
Type: Publication Only
Background
Afibrinogenemia is a rare condition and has an estimated prevalance of 1:1000000. The more common manifestations of afibrinogenemia are umblical stump bleeding, and bleeding from mucosal surfaces, particularly menorrhagia, epistaxis, gastrointestinal bleeding and intracranial hemorrhage.
Aims
We report a case with congenital afibrinogenemia which had pulmonary and intracranial hemorrhage. Although the patient had not prophylactic therapy with fibrinogen concentrate nor cryoprespitate before admission to the hospital, laboratory investigation revealed mixed test positivity
Methods
A case of an 18 years-old girl with congenital afibrinogenemia admitted to our department because of dyspnea and tachipnea. Her chest X-ray and CT showed pulmonary hemorrhage. Therefore, she was administered with fibrinogen conscentrate in doses of 50 mg per kg and prednisolon in doses 1 mg per kg. Furthermore, she underwent a generalized convulsion on second day and neuroimaging tests revealed intracranial hemorrhage. Although she was treated with fibrinogen concentrate and cryoprespitate every day her laboratory investigation revealed prolonged PT, aPTT and TT tests and fibrinogen level in plasma lower than 0.5g /L. Hence, we performed mixed test and the result was positive. On follow up, she was given IVIG in doses of 2 g per kg once and plasma exchange was planned every day. Furthermore, she was treated with fibrinogen concentrate and cryoprespitate every day to raise fibrinogen level in plasma to 1 g/L. Unfortunately, she died at intensive care unite because of intracranial hemorrhage 17 days after admission to the hospital.
Results
Replacement therapy is generally effective in treating of bleeding episodes in congenital afibrinogenemia. Options for replacement include plasma-derived fibrinogen concentrate, cryoprespitate, and fresh frozen plasma; Antifibrinolytic agents may be used especially for dental procedures. Prophylactic therapy have been employed after life-threatening bleedings such as intrcranial hemorrhage. Acquired inhibitors have been rarely reported after replacement and prophylactic therapy.
Conclusion
We surprisingly revealed inhibitor to fibrinogen (mixed test positivity) although the patient did not have prophylactic therapy with fibrinogen concentrate nor cryoprespitate before admission to the hospital.
Session topic: E-poster
Keyword(s): Fibrinogen, Hemorrhage, Inhibitor, Treatment
Type: Publication Only
Background
Afibrinogenemia is a rare condition and has an estimated prevalance of 1:1000000. The more common manifestations of afibrinogenemia are umblical stump bleeding, and bleeding from mucosal surfaces, particularly menorrhagia, epistaxis, gastrointestinal bleeding and intracranial hemorrhage.
Aims
We report a case with congenital afibrinogenemia which had pulmonary and intracranial hemorrhage. Although the patient had not prophylactic therapy with fibrinogen concentrate nor cryoprespitate before admission to the hospital, laboratory investigation revealed mixed test positivity
Methods
A case of an 18 years-old girl with congenital afibrinogenemia admitted to our department because of dyspnea and tachipnea. Her chest X-ray and CT showed pulmonary hemorrhage. Therefore, she was administered with fibrinogen conscentrate in doses of 50 mg per kg and prednisolon in doses 1 mg per kg. Furthermore, she underwent a generalized convulsion on second day and neuroimaging tests revealed intracranial hemorrhage. Although she was treated with fibrinogen concentrate and cryoprespitate every day her laboratory investigation revealed prolonged PT, aPTT and TT tests and fibrinogen level in plasma lower than 0.5g /L. Hence, we performed mixed test and the result was positive. On follow up, she was given IVIG in doses of 2 g per kg once and plasma exchange was planned every day. Furthermore, she was treated with fibrinogen concentrate and cryoprespitate every day to raise fibrinogen level in plasma to 1 g/L. Unfortunately, she died at intensive care unite because of intracranial hemorrhage 17 days after admission to the hospital.
Results
Replacement therapy is generally effective in treating of bleeding episodes in congenital afibrinogenemia. Options for replacement include plasma-derived fibrinogen concentrate, cryoprespitate, and fresh frozen plasma; Antifibrinolytic agents may be used especially for dental procedures. Prophylactic therapy have been employed after life-threatening bleedings such as intrcranial hemorrhage. Acquired inhibitors have been rarely reported after replacement and prophylactic therapy.
Conclusion
We surprisingly revealed inhibitor to fibrinogen (mixed test positivity) although the patient did not have prophylactic therapy with fibrinogen concentrate nor cryoprespitate before admission to the hospital.
Session topic: E-poster
Keyword(s): Fibrinogen, Hemorrhage, Inhibitor, Treatment
Abstract: PB1751
Type: Publication Only
Background
Afibrinogenemia is a rare condition and has an estimated prevalance of 1:1000000. The more common manifestations of afibrinogenemia are umblical stump bleeding, and bleeding from mucosal surfaces, particularly menorrhagia, epistaxis, gastrointestinal bleeding and intracranial hemorrhage.
Aims
We report a case with congenital afibrinogenemia which had pulmonary and intracranial hemorrhage. Although the patient had not prophylactic therapy with fibrinogen concentrate nor cryoprespitate before admission to the hospital, laboratory investigation revealed mixed test positivity
Methods
A case of an 18 years-old girl with congenital afibrinogenemia admitted to our department because of dyspnea and tachipnea. Her chest X-ray and CT showed pulmonary hemorrhage. Therefore, she was administered with fibrinogen conscentrate in doses of 50 mg per kg and prednisolon in doses 1 mg per kg. Furthermore, she underwent a generalized convulsion on second day and neuroimaging tests revealed intracranial hemorrhage. Although she was treated with fibrinogen concentrate and cryoprespitate every day her laboratory investigation revealed prolonged PT, aPTT and TT tests and fibrinogen level in plasma lower than 0.5g /L. Hence, we performed mixed test and the result was positive. On follow up, she was given IVIG in doses of 2 g per kg once and plasma exchange was planned every day. Furthermore, she was treated with fibrinogen concentrate and cryoprespitate every day to raise fibrinogen level in plasma to 1 g/L. Unfortunately, she died at intensive care unite because of intracranial hemorrhage 17 days after admission to the hospital.
Results
Replacement therapy is generally effective in treating of bleeding episodes in congenital afibrinogenemia. Options for replacement include plasma-derived fibrinogen concentrate, cryoprespitate, and fresh frozen plasma; Antifibrinolytic agents may be used especially for dental procedures. Prophylactic therapy have been employed after life-threatening bleedings such as intrcranial hemorrhage. Acquired inhibitors have been rarely reported after replacement and prophylactic therapy.
Conclusion
We surprisingly revealed inhibitor to fibrinogen (mixed test positivity) although the patient did not have prophylactic therapy with fibrinogen concentrate nor cryoprespitate before admission to the hospital.
Session topic: E-poster
Keyword(s): Fibrinogen, Hemorrhage, Inhibitor, Treatment
Type: Publication Only
Background
Afibrinogenemia is a rare condition and has an estimated prevalance of 1:1000000. The more common manifestations of afibrinogenemia are umblical stump bleeding, and bleeding from mucosal surfaces, particularly menorrhagia, epistaxis, gastrointestinal bleeding and intracranial hemorrhage.
Aims
We report a case with congenital afibrinogenemia which had pulmonary and intracranial hemorrhage. Although the patient had not prophylactic therapy with fibrinogen concentrate nor cryoprespitate before admission to the hospital, laboratory investigation revealed mixed test positivity
Methods
A case of an 18 years-old girl with congenital afibrinogenemia admitted to our department because of dyspnea and tachipnea. Her chest X-ray and CT showed pulmonary hemorrhage. Therefore, she was administered with fibrinogen conscentrate in doses of 50 mg per kg and prednisolon in doses 1 mg per kg. Furthermore, she underwent a generalized convulsion on second day and neuroimaging tests revealed intracranial hemorrhage. Although she was treated with fibrinogen concentrate and cryoprespitate every day her laboratory investigation revealed prolonged PT, aPTT and TT tests and fibrinogen level in plasma lower than 0.5g /L. Hence, we performed mixed test and the result was positive. On follow up, she was given IVIG in doses of 2 g per kg once and plasma exchange was planned every day. Furthermore, she was treated with fibrinogen concentrate and cryoprespitate every day to raise fibrinogen level in plasma to 1 g/L. Unfortunately, she died at intensive care unite because of intracranial hemorrhage 17 days after admission to the hospital.
Results
Replacement therapy is generally effective in treating of bleeding episodes in congenital afibrinogenemia. Options for replacement include plasma-derived fibrinogen concentrate, cryoprespitate, and fresh frozen plasma; Antifibrinolytic agents may be used especially for dental procedures. Prophylactic therapy have been employed after life-threatening bleedings such as intrcranial hemorrhage. Acquired inhibitors have been rarely reported after replacement and prophylactic therapy.
Conclusion
We surprisingly revealed inhibitor to fibrinogen (mixed test positivity) although the patient did not have prophylactic therapy with fibrinogen concentrate nor cryoprespitate before admission to the hospital.
Session topic: E-poster
Keyword(s): Fibrinogen, Hemorrhage, Inhibitor, Treatment
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