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

2018 ◽  
Vol 10 (3) ◽  
Author(s):  
Anthony L. Nguyen ◽  
Muhammad Kamal ◽  
Ravi Raghavan ◽  
Gayathri Nagaraj
Keyword(s):  
Factor Viia ◽  
Aminocaproic Acid ◽  
Fresh Frozen Plasma ◽  
Factor Vii ◽  
Al Amyloidosis ◽  
Severe Bleeding ◽  
Factor Vii Deficiency ◽  
Fresh Frozen ◽  
Hepatic Amyloidosis ◽  
Mucosal Bleeding

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.

The Risk of Thrombotic Events in Neonates Treated with Recombinant Factor VIIa.

Blood ◽  
2007 ◽  
Vol 110 (11) ◽  
pp. 3193-3193
Author(s):  
John Puetz ◽  
Ginger Darling ◽  
Petr Brabec ◽  
Jan Blatny ◽  
Prasad Mathew
Keyword(s):  
Recombinant Factor Viia ◽  
Factor Viia ◽  
Accurate Determination ◽  
Thrombotic Event ◽  
Fresh Frozen Plasma ◽  
Factor Vii ◽  
Control Group ◽  
Fresh Frozen ◽  
Surgical Bleeding ◽  
A Prospective Study

Abstract Background: In recent years, recombinant factor VIIa (rFVIIa) has been used in non-hemophilia bleeding situations (factor VII deficiency, trauma, liver disease, uremia, surgical bleeding, platelet disorders, and intracranial hemorrhage) for achievement of hemostasis. Although, the risk of thrombosis in hemophilia patients with inhibitors receiving rFVIIa is quite low, its use in other clinical situations has been complicated by some reports of thrombotic events. Recently, rFVIIa has been used to treat coagulopathic and/or bleeding neonates with good success. However, the prevalence of thrombotic events in these neonates is completely unknown. This study was initiated to determine the risk of thrombotic events associated with rFVIIa use in neonates. Methods: We reviewed all published literature in neonates receiving rFVIIa. In addition, we reviewed all data submitted to the SeveN Bleep Registry, a database developed by the scientific standardization subcommittee on pediatric and neonatal hemostasis of the International Society on Thrombosis and Haemostasis (ISTH) to record all uses of rFVIIa in pediatric non-hemophilic patients. As the baseline prevalence of thrombosis for bleeding and/or coagulopathic neonates is also unknown, we also reviewed the records of 100 consecutive neonates from a single institution who received fresh frozen plasma (FFP) alone to treat their coagulopathy and/or bleeding. Results: A total of 98 non-hemophilic neonates received rFVIIa. The majority of these neonates received rFVIIa only after failing to achieve hemostasis with standard care (FFP, cryoprecipitate, platelet transfusions). Of those receiving rFVIIa, 7 had a thrombotic event reported. In the control group that received FFP alone, 7 neonates also suffered a thrombotic event. Although the risk of thrombosis in these two groups is similar, neonates receiving rFVIIa tended to have indwelling line related thrombosis, while those receiving FFP tended to have strokes or myocardial insults. Overall the prevalence of thrombotic events in bleeding and/or coagulopathic neonates appears to be 7%, whether or not they received rFVIIa. Conclusions: In this study, the overall prevalence of thrombotic events was similar in the rFVIIa and FFP group. As data for this study was collected in a retrospective manor, and thereby subject to publication and submission bias, a more accurate determination of the prevalence of thrombosis in neonates will require a prospective study.

scholarly journals Factor VII Deficiency and Pregnancy: A Case Report and Review of Literature

2021 ◽  
Author(s):  
Minakshi Rohilla ◽  
Rakhi Rai ◽  
Jasmina Ahluwalia ◽  
Pankaj Malhotra ◽  
Vanita Jain
Keyword(s):  
Autosomal Recessive ◽  
English Literature ◽  
Fresh Frozen Plasma ◽  
Factor Vii ◽  
Coagulation Disorder ◽  
Review Of Literature ◽  
Factor Vii Deficiency ◽  
First Case ◽  
Fresh Frozen ◽  
Frozen Plasma

Abstract Inherited factor VII deficiency is an autosomal recessive coagulation disorder with broad range of bleeding manifestations. The association between bleeding and absolute factor VII level is poor. Usually, the bleeding is associated with FVII levels of less than 1% of the normal value. Factor VII deficiency is associated with prolongation of prothrombin time only with normal activated partial thromboplastin time. Approximately 66 pregnant women have been reported with factor VII deficiency so far in English literature. We hereby, report 2 cases along with the review of literature of Factor VII deficiency during pregnancy. Our patients were diagnosed to have factor VII deficiency after deranged coagulogram with factor VII level of < 1% and 17.1% respectively, however could be managed by fresh frozen plasma only in first case and fresh frozen plasma & factor VII concentrate in second case successfully. Coagulogram is a simple, easily available, affordable and lifesaving investigation to detect this deficiency in pregnancy.

Associated Risk of Recombinant Activated Factor VIIa Application

2013 ◽  
Vol 16 (3) ◽  
pp. 132
Author(s):  
Arndt-Holger Kiessling ◽  
Janine Nitsch ◽  
Ulrich Strouhal ◽  
Angela Kornberger ◽  
Andreas Zierer ◽  
...  
Keyword(s):  
Factor Viia ◽  
Therapeutic Option ◽  
Treatment Algorithm ◽  
Fresh Frozen Plasma ◽  
Severe Bleeding ◽  
Acute Bleeding ◽  
Packed Red Blood Cells ◽  
Fresh Frozen ◽  
Thrombotic Complications ◽  
Ischemic Attack

<p><b>Background:</b> The recombinant human coagulation FVIIa was approved for the treatment of bleeding in hemophilia patients. The reports of a good hemostatic effect were followed by studies and applications without a regulatory extension of the therapeutic indication (off-label use). The aim of this retrospective study is the evaluation of thromboembolic adverse events and side effects in a large cohort of patients with FVIIa therapy.</p><p><b>Methods:</b> In the period from January 2009 to March 2011, a total of 143/2453 (5.8%) cardiac surgical patients (69% male; age 67 � 11 years; 39% thoracic aorta) were treated with different doses (mean, 6.1 mg; range, 1 to 27.2 mg) of factor VIIa. The administration of FVIIa was seen as a last therapeutic option and administered at the end of the treatment algorithm for severe bleeding.</p><p><b>Results:</b> Due to an acute bleeding situation in 143 patients, 7.9 � 5.8 units of packed red blood cells, 9.5 � 6.1 units of fresh frozen plasma, 1740 � 1860 IU PPSB (Prothrombin-Proconvertin-Stuart Factor-Antihemophilic Factor B), 5.6 � 4 g fibrinogen, and 7.9 � 7.6 units of platelets were administered. A re-thoracotomy was necessary, despite maximal procoagulant therapy, in 55% of patients. The in-hospital mortality was 36% (51/2453 = 2%). Thrombotic complications occurred with a frequency of 16% (mesenteric infarction, n = 9; stroke/transient ischemic attack, n = 3; myocardial infarction, n = 3; other, n = 8).</p><p><b>Conclusion:</b> The proof of direct causality of the events in relation to the administration of FVIIa is difficult because the temporal and therapeutic relationships with concomitant vasoconstrictive and procoagulant therapies were not obvious. However, there remains a suspicion that a higher rate of mesenteric infarctions may be provoked by the administration of FVIIa.</p>

scholarly journals A case of open surgical treatment of staghorn calculus in a patient with factor VII deficiency

2019 ◽  
Vol 100 (5) ◽  
pp. 828-832
Author(s):  
S B Imamverdiev ◽  
T A Talybov ◽  
E A Gadimova ◽  
M Z Talybova
Keyword(s):  
Renal Artery ◽  
Fresh Frozen Plasma ◽  
Factor Vii ◽  
Coagulation System ◽  
Clotting Factors ◽  
Factor Vii Deficiency ◽  
Before And After ◽  
Prothrombin Index ◽  
Fresh Frozen ◽  
Staghorn Calculus

The paper presents a clinical case of treatment of staghorn nephrolithiasis complicated in the postoperative period by bleeding caused by a latent deficiency of factor VII of the blood coagulation system. Often the disease is latent and is detected in serious injuries or during the surgeries. Sometimes the diagnosis is made in profuse bleeding. Under endotracheal anesthesia, the left-sided nephrolithotomy and intrarenal stenting with clamping of the renal artery was performed. The time of clamping of the renal artery was 12 minutes. Before clamping the renal artery, 3 mg of furosemide 0.2 mg of verapamil and 1 mg of methylethylpiridinol were introduced intravenously per kilogram of patient' weight. A stone sized 3.02.5 cm was removed from the kidney, and a number of small stones were washed out from the lower group of calyces. At the time of diagnosis, prothrombin time and prothrombin index were checked. The patient's hemostasis system, including clotting factors, were examined. The results showed that the patient's blood clotting time by LeeWhite method increased to 15 minutes (reference, 713 minutes), and the time of activated partial thromboplastin time (aPTT) to 41.6 seconds (reference, 2838 seconds), while the activity of factor VII decreased to 40% (reference, 70120%). After that, the patient was diagnosed with factor VII deficiency or hypoproconvertinemia. During treatment, the patient was transfused twice a day with fresh frozen plasma and only once during the entire period, 1000 IU of antiinhibitoty coagulant complex (FEIBA) was administered intravenously. From the first day of treatment hematuria decreased, there was a reverse development of hemorrhagic syndrome. Obvious and latent comorbidities before and after the surgery should not be left out without attention. Only in this case it is possible to successfully complete the treatment and achieve the correct results.

Homozygous 2bp Deletion in the Human Factor VII Gene: A Non-Lethal Mutation that Is Associated with a Complete Absence of Circulating Factor VII

2000 ◽  
Vol 84 (10) ◽  
pp. 635-637 ◽  
Author(s):  
Pier Mannucci ◽  
P. Jenkins ◽  
Anselm Lee ◽  
Raffaella Coppola ◽  
David Perry ◽  
...  
Keyword(s):  
Human Factor ◽  
Fresh Frozen Plasma ◽  
Factor Vii ◽  
Causative Mutation ◽  
Direct Sequence ◽  
Factor Vii Deficiency ◽  
Plasma Factor ◽  
Fresh Frozen ◽  
Direct Sequence Analysis ◽  
Frozen Plasma

SummaryWe report the case of a 5-year-old boy with severe factor VII deficiency. The affected child presented at the age of 8 months and again at 18 months with bleeding from the gastrointestinal tract but the diagnosis of factor VII deficiency was not made until the age of 3 years. He was treated with fresh frozen plasma and subsequently factor VII concentrates and to date remains well. To identify the causative mutation, the factor VII gene was screened by SSCP and direct sequence analysis. A single homozygous 2bp deletion (-CT) mutation was identified in exon 1a removing nucleotides 27/28 (codons 52/53). Both parents, who were first cousins, were heterozygous for the mutation. The mutation located in the prepropeptide of factor VII, results in a complete absence of factor VII in plasma. This case indicates that a complete absence of plasma factor VII is not necessarily a lethal condition.

scholarly journals Factor V11 deficiency: A rare cause of nasal bleeding

2021 ◽  
Vol 2 (3) ◽  
Author(s):  
Georgina G Balyorugulu ◽  
◽  
Richard F Kiritta ◽  
Emmanuela Ambrose ◽  
Erius Tebuka ◽  
...  
Keyword(s):  
Blood Transfusion ◽  
Prothrombin Time ◽  
Fresh Frozen Plasma ◽  
Activated Partial Thromboplastin Time ◽  
Factor Vii ◽  
Factor Vii Deficiency ◽  
Fresh Frozen ◽  
Nasal Bleeding ◽  
Frozen Plasma ◽  
Functional Factor

Factor VII deficiency is a rare inherited disorder. Clinically the patient presents with bleeding tendencies. Diagnosis is made by prolonged prothrombin time, normal activated partial thromboplastin time and low functions factor VII assay or factor VII antigen. Therapy involves factor VII concentrates, recombinant factor VII, fresh frozen plasma and fibrinolytic inhibitors. We present a 6 years old boy with nose bleed for six months of whom prothrombin time was prolonged with functional factor VII assay of less than 1% confirming factor VII deficiency. He was managed with fresh frozen plasma, blood transfusion, tranexamic acid. Factor VII deficiency even though rare should be sought out in children presenting with bleeding. Keywords: Factor VII deficiency; coagulation; hemorrhage.

scholarly journals MENORRHAGIA;

2013 ◽  
Vol 20 (05) ◽  
pp. 846-848
Author(s):  
FARZANA ZIA ◽  
SHAHZEEM BHAYANI ◽  
RUBINA HUSSAIN
Keyword(s):  
Autosomal Recessive ◽  
Past History ◽  
Fresh Frozen Plasma ◽  
Factor Vii ◽  
Severe Anemia ◽  
Factor Vii Deficiency ◽  
Fresh Frozen ◽  
Life Threatening ◽  
Frozen Plasma

Factor VII deficiency is a rare, autosomal recessive coagulopathy that becomes symptomatic in the form of a hemorrhagicsyndrome characterized by severe life threatening bleeding. This may present in young women as severe anemia due to bleeding pervaginum. We report one such case of a 12 year old girl who presented at the gynecology outpatient department with complaints of severemenorrhagia at menarche. Her past history was consistent with episodes of profuse epistaxis and bleeding from gums. Her completeblood count showed severe anemia. Upon further investigation her prothrombin time was prolonged but her APTT was normal which wasindicative of Factor VII deficiency and was confirmed by serum assays of Factor VII. It is important to diagnose this disorder earlier in orderto avoid long term complications especially in women who may suffer from severe life threatening hemorrhage during menses orrecurrent miscarriages during pregnancy. Therefore, our patient was transfused with packed cells and fresh frozen plasma immediatelyand started on low dose oestrogen and progesterone pill along with tranexemic acid to control her symptoms.

Direct (new) oral anticoagulants (DOACs): Drawbacks, bleeding and reversal

2021 ◽  
Vol 19 ◽  
Author(s):  
Ozgur Karcioglu ◽  
Sehmus Zengin ◽  
Bilgen Ozkaya ◽  
Eylem Ersan ◽  
Sarper Yilmaz ◽  
...  
Keyword(s):  
Oral Anticoagulants ◽  
Fresh Frozen Plasma ◽  
New Oral Anticoagulants ◽  
Coagulation Cascade ◽  
Thrombin Inhibitor ◽  
Severe Bleeding ◽  
Prothrombin Complex Concentrates ◽  
Clinical Scenarios ◽  
Fresh Frozen ◽  
Frozen Plasma

Background and Objective: Direct (new) Oral Anticoagulants (DOACs) have emerged as a contemporary and promising option in the treatment of thromboses and VTE, while protecting the coagulation cascade against untoward bleeding events. They are used in the management and prophylaxis of Venous Thromboembolism (VTE) and other thrombotic diseases. The most prominent complication of these agents is bleeding. These agents have similar or lower rates of major intracranial hemorrhages, while they had a higher risk of major gastrointestinal bleeding when compared to warfarin. This manuscript is aimed to revise and update the literature findings to outline the side effects of DOACs in various clinical scenarios. Methods: A narrative review of currently published studies was performed. Online database searches were performed for clinical trials published before July 2021, on the efficacy and adverse effects attributed to the anticoagulant treatment, especially DOACs. A literature search via electronic databases was carried out, beginning with the usage of the agents in the Western Languages papers. The search terms initially included direct (new) oral anticoagulants, dabigatran, rivaroxaban, apixaban, edoxaban, idarucizumab, andexanet, prothrombin complex concentrates, and fresh frozen plasma. Papers were examined for methodological soundness before being included. Results: Severe bleeding episodes require aggressive interventions for successful management. Therefore, bleeding should be evaluated in special regard to the location and rate of hemorrhage, and total volume of blood loss. Patient's age, weight and organ dysfunctions (e.g., kidney/liver failure or chronic respiratory diseases) directly affect the clinical course of overdose. Conclusion: Management recommendations for hemorrhage associated with DOAC use vary, depending on the class of the culprit agent (direct thrombin inhibitor vs. FXa inhibitor), the clinical status of the patient (mild/ moderate vs. severe/life-threatening), and capabilities of the institution. Specific reversal agents (i.e., idarucizumab and andexanet alfa) can be used if available, while prothrombin complex concentrates, fresh frozen plasma and/ or tranexamic acid can also be employed as nonspecific replacement agents in the management of DOAC-related bleeding diathesis.

scholarly journals Efficacy and safety of recombinant factor VIIa preparation (NovoSeven) for patients with congenital factor VII deficiency

2006 ◽  
Vol 17 (6) ◽  
pp. 695-705 ◽  
Author(s):  
Hideji HANABUSA ◽  
Kazushige OYAMA ◽  
Satoshi WATANABE ◽  
Yuzuru SAKAKIBARA ◽  
Yuji HIRAMATSU ◽  
...  
Keyword(s):  
Recombinant Factor Viia ◽  
Factor Viia ◽  
Factor Vii ◽  
Efficacy And Safety ◽  
Factor Vii Deficiency ◽  
Congenital Factor

scholarly journals Congenital Deficiency in Factor VII Revealed by Menorrhagia: Case Report

2020 ◽  
pp. 1-5
Author(s):  
Nadia Mebrouk ◽  
Abdelilah Radi ◽  
Mohamed Selouti ◽  
Amal Hassani ◽  
Abdelhakim Ourrai ◽  
...  
Keyword(s):  
Treatment Options ◽  
Specific Treatment ◽  
Fresh Frozen Plasma ◽  
Factor Vii ◽  
Activity Measurement ◽  
Recombinant Activated Factor Vii ◽  
Congenital Deficiency ◽  
Activated Factor Vii ◽  
Fresh Frozen ◽  
Fvii Deficiency

Factor VII (FVII) deficiency is the most common among rare inherited autosomal recessive bleeding disorders. It is a multifaceted disease because of the lack of a direct correlation between plasma levels of coagulation FVII and bleeding manifestations. Clinical phenotypes range from asymptomatic condition—even in homozygous subjects—to severe, life-threatening bleedings (e.g., central nervous system and gastrointestinal bleeding). Menorrhagia is a frequent type of bleeding in FVII deficiency, with a prevalence rate of two in three women aged 10 to 50 years and with a peak prevalence in teenagers. When menorrhagia is observed and once the gynecological causes are excluded, it is important to carry out a hemostasis assessment because, if an anomaly is found, specific treatment can be administered and preventive measures taken. Basic diagnostic work-up includes routine assays, prothrombin level, activated partial thromboplastin time and platelet count, followed by FVII coagulant activity measurement for isolated decreased prothrombin level. To confirm the diagnosis, FVII assay should be repeated at least once. Several treatment options are currently available for FVII deficiency: Recombinant activated Factor VII (rFVIIa), plasma-derived Factor VII, fresh frozen plasma and prothrombin complex concentrates. rFVIIa is the most used replacement therapy. Other medical therapies of menorrhagia includes hemostatic agents and hormonal treatments (combined oral contraceptives, levonorgestrel intrauterine devices), in combination or not with rFVIIa. We report the case of a fourteen-and-a-half-year-old girl who presented menorrhagia of great abundance at the age of thirteen, the exploration of which revealed a congenital deficit in FVII.

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