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Defects in Coagulation Factors Leading to Recurrent Pregnancy Loss
Published in Howard J.A. Carp, Recurrent Pregnancy Loss, 2020
The three overlapping hereditary abnormalities of fibrinogen—afibrinogenemia, dysfibrinogenemia, and hypofibrinogenemia—have been associated with recurrent pregnancy loss. Afibrinogenemia—a defect in hepatic fibrinogen secretion or release—is inherited as an autosomal recessive trait and is associated with bleeding diathesis, impaired wound repair, and recurrent pregnancy loss. A related form of this disorder is hypofibrinogenemia. Hereditary dysfibrinogenemias are characterized by the biosynthesis of structurally and functionally abnormal fibrinogen.
Concepts of Replacement Therapy: Blood Components, Blood Derivatives, and Medications
Published in Harold R. Schumacher, William A. Rock, Sanford A. Stass, Handbook of Hematologic Pathology, 2019
The most common indication for the use of cryoprecipitate in the United States today is hypofi-brinogenemia. Other derivatives that contain fibrinogen in a concentrated form are not approved for use by the Food and Drug Administration. Most cases of hypofibrinogenemia are associated with patients with a consumptive coagulopathy, who should receive other blood components. Cryoprecipitate can provide an appropriate concentration of fibrinogen in the smallest volume possible. Fibrinogen levels above 100 mg/dL are usually adequate for hemostasis. Cryoprecipitate can also be used for patients with von Willebrand’s disease, but this product should be reserved for those cases that are unable to receive or unresponsive to DDAVP, or when a factor VIII product that contains vWF is not available. The same rule should apply to patients with hemophilia A when factor VIII concentrates are not available. A growing use of cryoprecipitate is as the source of fibrinogen and fibrin-stabilizing factor (factor XIII), which is mixed with bovine thrombin to produce a surgical adhesive, commonly called fibrin glue (13).
Primary Hyperfibrino(Geno)Lytic Syndromes
Published in Genesio Murano, Rodger L. Bick, Basic Concepts of Hemostasis and Thrombosis, 2019
Rodger L. Bick, Genesio Murano
The majority of patients with chronic liver disease experience a hyperfibrino (geno)lytic syndrome20, 21 due to increased quantities of circulating plasmin. Primary hyperfibrino(geno)lysis (circulating plasmin) causes numerous hemostatic defects. Hypofibrinogenemia and “pseudodysfibrinogenemia” occurs in the presence of fibrino(geno)lysis. Pseudodysfibrinogenemia is manifest in several forms; one is the creation of high solubility (1-8, 1-9) fractions,22 low thrombin clottability fibrinogen subspecies. This represents fibrinogen that has undergone minimal cleavage by plasmin (Chapter 3). Because of fibrino(geno)lysis, patients with chronic liver disease usually demonstrate elevated FDP. The early degradation products also create a pseudodysfibrinogenemia by complexing with native fibrinogen and thus interfering with fibrin monomer polymerization. In addition, the latter degradation products coat the surfaces of platelets, rendering them dysfunctional.23 Circulating plasmin can also cause the proteolysis of Factor V, Factor VIII, Factor IX, and Factor XI. Thus these factors may be decreased. These additional fibrino(geno)lytic insults to hemostasis must always be borne in mind when clinical care is being rendered to the patient with chronic liver disease.
Rare inherited coagulation disorders in young children in Oman
Published in Pediatric Hematology and Oncology, 2022
Surekha Tony, Roshan Mevada, Abdulhakim Al Rawas, Yasser Wali, Mohamed Elshinawy
Fibrinogen disorders were identified in 26 patients [hypofibrinogenemia (18/26), afibrinogenemia (6/26), and dysfibrinogemia (2/26)], accounting for 32.9% of all patients with rare coagulation disorders. Initial presentations included spontaneous mucocutaneous, musculoskeletal, umbilical stump, postcircumcision, intracranial, and posttraumatic musculoskeletal bleeds (Table 1). Atypical severe clinical presentations of hypofibrinogenemia are displayed in Table 2. Two out of six patients with afibrinogenemia (33%) presented with ICH. Others presented with spontaneous mucocutaneous/musculoskeletal bleeds. In total, 12/26 patients (46%) with severe bleeding phenotype (6 patients with afibrinogenemia and 6 patients with hypofibrinogenemia) received regular prophylactic replacement therapy. The main indications for prophylaxis included recurrent bone cysts, splenic rupture, hemorrhagic ovarian cyst, ICH, and massive orbital hematoma. Two adolescent girls required hormonal therapy as an adjuvant treatment for uncontrollable menorrhagia.
Hemostatic defects in massive transfusion: an update and treatment recommendations
Published in Expert Review of Hematology, 2021
It is important to perform early assessment for hypofibrinogenemia in bleeding patients with viscoelastic testing in addition to measurement of plasma fibrinogen concentrations. In an important study of ATC in adult trauma patients (n = 623) with ISS > 15, plasma fibrinogen measurement and TEG were performed within 15 minutes of each other and within 1 hour of admission. The estimated optimal cutoffs for diagnosing hypofibrinogenemia (plasma fibrinogen <200 mg/dL) were 1.5 minutes for K-time, 70.0° for α-angle, and 60.9 mm for MA. The estimated optimal cutoffs for diagnosing severe hypofibrinogenemia (plasma fibrinogen <100 mg/dL) were 2.4 minutes for K-time, 60.6° for α-angle, and 51.2 mm for MA. Currently recommended K-time and α-angle cutoffs from the American College of Surgeons Massive Transfusion Guideline had low sensitivity for diagnosing hypofibrinogenemia (3%-29%), but sensitivity improved to 74% when using optimal cutoffs defined in this study. This study confirmed that Kaolin-TEG parameters can accurately diagnose hypofibrinogenemia/severe hypofibrinogenemia in trauma patients. Many patients are likely to be undertreated for hypofibrinogenemia using current national guidelines. [72]
Prevalence of hematologic toxicity from copperhead envenomation: an observational study
Published in Clinical Toxicology, 2020
Brandon K. Wills, Michael Billet, S. Rutherfoord Rose, Kirk L. Cumpston, Francis Counselman, Kendall J. Shaw, Kellen G. Cresswell, Nathan Charlton
In total, there were 388 patients considered for analysis, of which 244 fulfilled inclusion and exclusion criteria. 130 of the 144 excluded patients were from the Eastern region and were omitted due to the inability to verify copperhead species. Liver disease, underlying coagulopathy, and baseline anticoagulation account for the remaining 14 excluded patients. Of included patients, 143 were male (59%) and 101 were female (41%). 186 (76%) of patients were treated with antivenom, 54 (22%) did not receive antivenom and 4 (2%) did not have information regarding antivenom administration. The secondary characteristics of the patient population are summarized in Table 1. Hematologic toxicity was found in 14% (95% CI 10–18%) of patients. Among bite victims, the prevalence of thrombocytopenia was 1.2% (95% CI 0.4–3.6%), while the prevalence of hypofibrinogenemia was 0.7% (95% CI 0.0–3.8%). Additionally, elevated PT was identified in 10.0% (95% CI 6.8–14.5%) of patients and elevated aPTT in 3.9% (95% CI 2.1–7.2%) of patients. Table 2 contains summary statistics for all abnormal laboratory values. The single patient with hypofibrinogenemia had a fibrinogen level of 93 mg/dL. There was no statistical difference between the study sites (Table 3). Table 4 summarizes the occurrence of hematologic toxicities, separated by antivenom administration. We found no statistically significant association between antivenom administration and the occurrence of any of the hematologic toxicities. For this analysis, the four patients without data on antivenom treatment were excluded. There was no clinically significant bleeding reported in any case.