Isolated acquired factor VII deficiency: review of the literature

Mulliez, Sylvie; Devreese, Katrien

doi:10.1179/2295333715y.0000000073

Cited by 20 publications

(31 citation statements)

References 21 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…It is well known that PT is prolonged by a series of conditions, including direct acting anticoagulants (among them argatroban, dabigatran, rivaroxaban, apixaban, edoxaban)[29] and intake of vitamin K antagonists[30], vitamin K deficiency[31], coagulation factor deficiency, lupus anticoagulans[32], polycitemia vera[33]. Similarly, it was shown that factor VII deficiency is frequent in patients with infections, neoplasia, trauma, nephrotic syndrome, left heart failure, penicillin intake and clearly in patients under vitamin K dependent anticoagulation[34, 35]. However, ATIII should only be altered due to mutations[36], consumption[37] or hepatocellular dysfunction[38, 39].…”

Section: Discussionmentioning

confidence: 99%

Early prediction of postoperative liver dysfunction and clinical outcome using antithrombin III-activity

et al. 2017

View full text Add to dashboard Cite

Background and aimsAntithrombin III (ATIII) has been reported to be associated with liver pathologies and was shown to predict outcome in patients undergoing liver resection for hepatocellular carcinoma. We now aimed to assess whether perioperative ATIII-activity could predict postoperative outcome in patients without underlying liver disease, as well as in a routine clinical setting of patients undergoing hepatic resection.MethodsATIII-activity was evaluated preoperatively and on the first (POD1) and fifth day after liver resection in a retrospective evaluation cohort of 228 colorectal cancer patients with liver metastasis (mCRC). We further aimed to prospectively validate our results in a set of 177 consecutive patients undergoing hepatic resection.ResultsPatients developing postoperative liver dysfunction (LD) had a more pronounced postoperative decrease in ATIII-activity (P<0.001). ATIII-activity on POD1 significantly predicted postoperative LD (P<0.001, AUC = 84.4%) and remained independent upon multivariable analysis. A cut-off value of 61.5% ATIII-activity was determined using ROC analysis. This cut-off was vital to identify high-risk patients for postoperative LD, morbidity, severe morbidity and mortality (P<0.001, respectively) with a highly accurate negative predictive value of 97%, which could be confirmed for LD (P<0.001) and mortality (P = 0.014) in our independent validation cohort. Further, mCRC patients below our cut-off suffered from a significantly decreased overall survival (OS) at 1 and 3 years after surgery (P = 0.011, P = 0.025).ConclusionsThe routine laboratory parameter ATIII-activity on POD1 independently predicted postoperative LD and was associated with clinical outcome. Patients with a postoperative ATIII-activity <61.5% might benefit from close monitoring and timely initiation of supportive therapy.Trial registrationClinicalTrials.gov NCT01700231

show abstract

Section: Discussionmentioning

confidence: 99%

Early prediction of postoperative liver dysfunction and clinical outcome using antithrombin III-activity

et al. 2017

View full text Add to dashboard Cite

show abstract

“… 8 , 9 Other rare etiologies of acquired factor VII deficiency include cases secondary to administration of penicillins, cephalosporins and diseases such as myeloma, underlying malignancy and usage of anti-thymocyte globulin and IL- 2. 10 , 11 The levels of factor VII in previous cases presenting with severe hemorrhage were between <1% to 38% of normal factor VII levels. 11 Additionally, factor VII levels have been measured in the setting of decompensated cirrhosis with mean level of 40%, which is significantly lower than than levels in patients with compensated cirrhosis, obstructive jaundice, and noncirrhotics with alcoholic liver disease.…”

Section: Discussionmentioning

confidence: 94%

Acquired factor VII deficiency causing severe bleeding disorder secondary to AL amyloidosis of the liver

et al. 2018

View full text Add to dashboard Cite

A 52 year-old male presented with neck pain after undergoing thyroidectomy for a goiter three weeks prior which was complicated by a neck hematoma requiring evacuation. Computed tomography (CT) scan showed a neck hematoma requiring evacuation and he received desmopressin with cessation of bleeding. Coagulation studies were normal. He returned eighteen months later with severe oral mucosal bleeding after a dental procedure and required transfusions with red blood cells, platelets, and fresh frozen plasma (FFP) in addition to desmopressin, Humate-P, aminocaproic acid, and surgical packing. A comprehensive bleeding diathesis workup was normal. He was readmitted six months later due to abdominal pain and distention and found to have massive hepatosplenomegaly on CT. A new coagulopathy workup revealed prolonged INR to 1.5, corrected prothrombin time mixing study, and a low factor VII level (29%), suggesting acquired factor VII deficiency. A transjugular liver biopsy revealed extensive involvement by ALamyloidosis- Kappa type. He then developed a large right retroperitoneal hematoma which required multiple transfusions with FFP, cryoprecipitate, aminocaproic acid, and vitamin K with slight success. Hemorrhage was subsequently stabilized with recombinant factor VIIa administered every four hours which corresponded with correction of factor VII levels and PT and eventual cessation hemorrhage. Acquired factor VII deficiency causing severe coagulopathy was attributed to hepatic amyloidosis ALkappa subtype. We started treatment with bortezomib, dexamethasone, and cyclophosphamide, however, the patient succumbed to uncontrolled hemorrhage. Acquired factor VII deficiency is extremely rare and to our knowledge, this is the only known case of factor VII deficiency secondary to amyloidosis involving the liver.

show abstract

“…If symptomatic patients usually have umbilical stump bleeding, skin and mucosal tract hemorrhage[72].FVII is a 50 Kda single chain polypeptide encoded by F7 gene located on chromosome 13 and FVII levels are influenced by age, sex and health condition such as blood cholesterol and triglyceride levels[73,74]. FVII deficiency is observed in 1 in 500,000, with variable phenotypes[74]. Some patients do not show bleeding phenotype despite very low FVII levels, whereas others with similar FVII antigen levels show severe bleeding phenotype[74].…”

mentioning

confidence: 99%

“…FVII deficiency is observed in 1 in 500,000, with variable phenotypes[74]. Some patients do not show bleeding phenotype despite very low FVII levels, whereas others with similar FVII antigen levels show severe bleeding phenotype[74]. The bleeding phenotypes of FVII deficiency include central nervous system hemorrhage, epistatic and menorrhagia[74].…”

mentioning

confidence: 99%

“…Some patients do not show bleeding phenotype despite very low FVII levels, whereas others with similar FVII antigen levels show severe bleeding phenotype[74]. The bleeding phenotypes of FVII deficiency include central nervous system hemorrhage, epistatic and menorrhagia[74]. Frozen fresh plasma, prothrombin complex concentrates, plasma derived FVII concentrate, recombinant FVIIa are typically used to treat FVII defi-…”

mentioning

confidence: 99%

See 1 more Smart Citation

Understanding the Clotting Cascade, Regulators, and Clinical Modulators of Coagulation

Pilli¹

2018

Hematology - Latest Research and Clinical Advances

View full text Add to dashboard Cite

The circulatory system plays a vital role in the survival of an organism by supplying it with essential nutrients, signaling molecules and eliminating the waste or toxic products from the body. This flow is tightly regulated by various factors, procoagulants support the formation of hemostatic plugs to prevent the leakage or blood loss and anticoagulants prevent the formation of unwanted clots. Disruption or dysregulation of procoagulants and anticoagulants lead to clinical complexities. In this chapter defects in the coagulation system, hereditary, acquired coagulation disorders, their diagnosis and recent clinical modulators of the coagulation system are discussed.

show abstract

Isolated acquired factor VII deficiency: review of the literature

Cited by 20 publications

References 21 publications

Early prediction of postoperative liver dysfunction and clinical outcome using antithrombin III-activity

Early prediction of postoperative liver dysfunction and clinical outcome using antithrombin III-activity

Acquired factor VII deficiency causing severe bleeding disorder secondary to AL amyloidosis of the liver

Understanding the Clotting Cascade, Regulators, and Clinical Modulators of Coagulation

Contact Info

Product

Resources

About