UNASSIGNED: Inherited mild @entity19434 belongs to one of the most underdiagnosed @entity548 disorders so far. This is, because most @entity1 do not develop @entity548 complications in daily life. @entity1 , methods: A @entity1 (age: 64 years) without a history of @entity548 presented with @entity4048 , @entity353 , @entity417 and @entity548 from the right tonsil necessitating tonsillectomy. Histologic and immunohistochemical evaluation revealed @entity5426 . @entity1003 status showed significantly elevated D-dimer and decreased FXIII levels (FXIII-activity 35%, FXIIIA-Ag 16-26%). Plasma mixing studies excluded neutralizing antibodies against FXIII. RESULTS: A novel heterozygous @entity21939 gene nonsense mutation ( @entity69915 , @entity69916 ) was found confirming heterozygous FXIII-A deficiency. The same mutation was detected in two further asymptomatic relatives. For further clinical management the @entity1 was transfused with FXIII-concentrate and showed an adequate increase of FXIII ruling out @entity942 to be induced by increased turnover. Despite this haemostatic management and antifibrinolytic treatment the @entity1 had to undergo several revisions due to delayed, Hb relevant @entity548 after cervical lymph nodes extirpation and resection of tonsil. Two chemotherapy cycles with @entity1311 and palliative radiotherapy of the neck area were performed, but the @entity1 died unfortunately two months after diagnosis. CONCLUSIONS: It is a unique case showing the combination of a highly aggressive @entity5426 and presence of @entity74 . It is also a rare example demonstrating the benefit of FXIII genotyping besides the expected acquired @entity942 possibly due to @entity5 induced increased consumption by elevated crosslinking of fibrin fibers.

XXXX -induced @entity548 in inherited, heterozygous FXIII-A  deficiency.

@entity1 :: ('9606', 'Species') :: ['patients', 'patient', 'Patient', 'man'] @entity353 :: ('MESH:D015431', 'Disease') :: ['weight loss'] @entity548 :: ('MESH:D006470', 'Disease') :: ['episodic bleeding', 'bleeding'] @entity417 :: ('MESH:D000740', 'Disease') :: ['anemia'] @entity5 :: ('MESH:D009369', 'Disease') :: ['neoplasm'] @entity5426 :: ('MESH:D006394', 'Disease') :: ['cytokeratin-positive epitheloid angiosarcoma', 'angiosarcoma'] @entity1311 :: ('MESH:D017239', 'Chemical') :: ['paclitaxel'] @entity1003 :: ('MESH:D001778', 'Disease') :: ['Blood coagulation'] @entity69916 :: ('c|SUB|G|307|T', 'DNAMutation') :: ['c.307G>T'] @entity4048 :: ('MESH:D019547', 'Disease') :: ['painful swelling of neck'] @entity74 :: ('MESH:D030342', 'Disease') :: ['inherited FXIII deficiency'] @entity69915 :: ('p|SUB|E|103|X', 'ProteinMutation') :: ['p.Glu103Ter'] @entity21939 :: ('2162', 'Gene') :: ['F13A1'] @entity19434 :: ('MESH:D005177', 'Disease') :: ['factor XIII deficiency'] @entity942 :: ('MESH:D003677', 'Disease') :: ['FXIII deficiency']
@entity5 :: (MESH:D009369,Disease) :: ['Neoplasm']
