Cancer reports (Hoboken, N.J.) · 2026
Case Report: Co-Occurrence of Lung Adenocarcinoma and Congenital Dysfibrinogenemia-Diagnostic and Perioperative Management Challenges.
Клинический случайСтатьяПоддержка исследования: вне правительства СШАНазвание и аннотация: Перевод пока не подготовлен
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Zheng He, Wang Qingsong, Wang Mingpu, Luo Shu, Ma Yuzhen, Wan Zhengqiang
Аннотация
BACKGROUND: Congenital dysfibrinogenemia (CD), a rare autosomal dominant coagulation disorder, poses significant perioperative challenges in oncologic surgery due to hypofibrinogenemia and variable bleeding-thrombosis risks.
CASE: A 67-year-old woman presented with a 2.9 × 1.4 cm spiculated mass in the right middle lobe (RML) and persistent hypofibrinogenemia (0.56-0.58 g/L). Despite conventional fresh frozen plasma and cryoprecipitate transfusions, fibrinogen levels remained critically low. Preoperative optimization with human fibrinogen concentrates (total 4.0 g) normalized levels to 1.76 g/L within 24 h, enabling video-assisted thoracoscopic (VATS) RML lobectomy with systematic lymphadenectomy (pT1cN0M0, Stage IA3). Postoperatively, fibrinogen gradually declined to 0.68 g/L at 1-month follow-up, necessitating extended surveillance. Genetic testing identified a heterozygous FGA c.103C>T (Arg35Cys) mutation, confirming CD diagnosis and thrombotic risk.
CONCLUSION: This case establishes three paradigms: (1) Mandatory comprehensive coagulation profiling in pulmonary resection candidates to identify CD; (2) superiority of fibrinogen concentrate over plasma-derived products for rapid, sustained hemostasis in CD; (3) individualized thromboprophylaxis balancing hemorrhagic-thrombotic risks through genetic risk stratification.
CASE: A 67-year-old woman presented with a 2.9 × 1.4 cm spiculated mass in the right middle lobe (RML) and persistent hypofibrinogenemia (0.56-0.58 g/L). Despite conventional fresh frozen plasma and cryoprecipitate transfusions, fibrinogen levels remained critically low. Preoperative optimization with human fibrinogen concentrates (total 4.0 g) normalized levels to 1.76 g/L within 24 h, enabling video-assisted thoracoscopic (VATS) RML lobectomy with systematic lymphadenectomy (pT1cN0M0, Stage IA3). Postoperatively, fibrinogen gradually declined to 0.68 g/L at 1-month follow-up, necessitating extended surveillance. Genetic testing identified a heterozygous FGA c.103C>T (Arg35Cys) mutation, confirming CD diagnosis and thrombotic risk.
CONCLUSION: This case establishes three paradigms: (1) Mandatory comprehensive coagulation profiling in pulmonary resection candidates to identify CD; (2) superiority of fibrinogen concentrate over plasma-derived products for rapid, sustained hemostasis in CD; (3) individualized thromboprophylaxis balancing hemorrhagic-thrombotic risks through genetic risk stratification.