Head of Service of Hematology Hospital General de Valdepenas Valdepenas, Castilla-La Mancha, Spain
Background: Factor VII deficiency, although rare, is relatively common in congenital coagulopathies consultations, generally with a milder bleeding phenotype than other rare bleeding disorders, although factor quantification often does not correspond to clinical manifestations.
Aims: The objective of this work is to document the correlation between the hemorrhagic phenotype and the quantification of factor VII in patients classified as non-severe deficit.
Methods: Retrospective, analytical and single-center study that documents the quantification of factor VII in patients labeled as mild (20-50%) or moderate (10-20%) deficit and bleeding manifestations at diagnosis or follow-up in a rural center in Spain. Clinical and analytical results were collected.
Results: Ten patients (with percentage parity by sex) with a median age of 41 years (range 7-79 years) were diagnosed with mild (70%) or moderate (30%) factor VII deficiency. 30% of the sample (2 moderate deficits and 1 mild) debuted at diagnosis with metrorrhagia as a cardinal symptom, while most of the sample were asymptomatic (analytical diagnosis due to altered prothrombin time). A moderate patient was diagnosed after postoperative bleeding due to digestive neoplasia, in need of replacement treatment with rFVIIa, with clinical improvement. None of the non-severe patients required prophylactic treatment.
Conclusion(s): Non-severe factor VII deficiency is expressed heterogeneously from the clinical point of view, so it is essential to assess the hemorrhagic phenotype on an individual basis and with special emphasis prior to aggressive surgical procedures.
