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Inflammation
Published in George Feuer, Felix A. de la Iglesia, Molecular Biochemistry of Human Disease, 2020
George Feuer, Felix A. de la Iglesia
Factor X deficiency disease has also been described, and individuals with this deficiency have a bleeding tendency similar to that of classic hemophilia.99,143 The congenital defect of Factor II (thrombin) is very rare. Most of the individuals with this disorder have 2 to 10% prothrombin activity, and the susceptibility to bleeding shows some parallelism to the level of prothrombin.238,251,415 Factor XI and Factor XII deficiencies have also been observed. Deficiency of Factor XI is a relatively uncommon disorder and is manifested as a hemorrhagic disorder. The hemorrhagic tendency is, however, much less severe than in hemophilia.129 Congenital deficiency of Factor V is also rare, and the associated bleeding tendency is only moderately severe.354
The Non-Hodgkin’s Lymphomas and Plasma Cell Dyscrasias
Published in Harold R. Schumacher, William A. Rock, Sanford A. Stass, Handbook of Hematologic Pathology, 2019
Lynne V. Abruzzo, L. Jeffrey Medeiros
The clinical manifestations of amyloidosis result from the widespread deposition of amyloid protein that leads to organ dysunction. Renal involvement leads to nephrotic syndrome in approximately one-third of patients. Amyloid deposition in the heart results in congestive heart failure and arrhythmias. Patients may develop malbsorption when the gastrointestinal tract is involved. Involvement of the nervous system is characterized by peripheral neuropathy. Coagulation abnormalities result from acquired factor X deficiency, increased fibrinolysis, and decreased fibrinogen. Amyloid deposition in the liver and spleen leads to hepatomegaly and splenomegaly, but lymphadenopathy is unusual.
Hereditary Plasma Protein Disorders
Published in Genesio Murano, Rodger L. Bick, Basic Concepts of Hemostasis and Thrombosis, 2019
These two disorders40 are extremely rare, with only 60 to 70 cases of congenital Factor VII deficiency having been reported. One in 100,000 has Factor X deficiency. Both are inherited as autosomal recessive traits and both manifest a similar clinical bleeding diathesis. The patients suffer intra-articular bleeding and hemarthroses, but, more commonly, demonstrate only mild mucosal bleeding, manifest by epistaxis, genitourinary and gastrointestinal bleeding. Significant bleeding can occur with surgery or trauma. In several instances, no bleeding manifestations whatsoever have been present. In both congenital Factor VII and congenital Factor X deficiency two variants exist. Some patients with congenital Factor VII deficiency have been reported to have a true deficiency (CRM”), while others have a dysfunctional Factor VII molecule (CRM+). The same situation appears to exist for congenital Factor X deficiency. In both of these disorders the prothrombin time is prolonged. The differential diagnosis is made by the utilization of the Stypven® time (prothrombin time performed with Russell’s viper venom). The Stypven® time will be normal in congenital Factor VII deficiency and will be prolonged in Factor X deficiency. Bleeding in both of these disorders, if it reaches significant or life-threatening proportions, can be controlled with prothrombin complex concentrates.
Overcoming the challenges of treating hemophilia in resource-limited nations: a focus on medication access and adherence
Published in Expert Review of Hematology, 2021
Kanjaksha Ghosh, Kinjalka Ghosh
Improving blood transfusion services in any country not only improves care of PWH and other bleeding disorders it also improves overall health service wherever blood or blood products are required. Once the various services of blood banking is slowly developed in the country initial products i.e. FFP, Cryoprecipitate, Cryodepleted plasma can be used for management of some of the cases, they could even be made safer by proper donor control, holding the product till the regular donor comes back for next transfusion and serologically test negative, instituting NAT testing facility, initially may be done in small batches and then individual donors may be tested. In many resource constrained countries, particularly in Asia, rare inherited bleeding disorders are often not so rare because of consanguinity [30]and they need different concentrates that are not readily available but can be provided by FFP or cryoprecipitate. For example severe factor XIII deficiency can easily be treated with once a month FFP infusion as factor XIII has a long half-life of 10–13 days, low levels of this factor can adequately prevent bleeding. Similar argument may be made for factor X deficiency. These FFPs can be freeze dried or can be made virologically safe by solvent detergent [31] and wet heat treatment in a cost effective manner.
Guidelines for non-transplant chemotherapy for treatment of systemic AL amyloidosis: EHA-ISA working group
Published in Amyloid, 2023
Ashutosh D. Wechalekar, M. Teresa Cibeira, Simon D. Gibbs, Arnaud Jaccard, Shaji Kumar, Giampaolo Merlini, Giovanni Palladini, Vaishali Sanchorawala, Stefan Schönland, Christopher Venner, Mario Boccadoro, Efstathios Kastritis
Standard therapy should be used for these patients with careful monitoring to avoid thrombocytopenia, which could increase the risk of major, clinically significant bleeding. Replacement with clotting factor concentrates, as indicated, may be considered. In patients with factor X deficiency and life-threatening bleeding, activated factor VIIa may have role. IMiDs need careful assesement in patients at high bleeding risk due to complexity of balancing risks of bleeding vs. clotting and challenge in use of thromboprophylaxis. The anti-fibrinolytic agent, tranexamic acid, may be used to help decrease the rate of bleeding if there is no prothrombotic risk factors (like severe nephrotic syndrome or history of ischaemic heart disease or stroke).
Spontaneous periocular ecchymosis: a major review
Published in Orbit, 2023
Matthew J. Hartley, Pav Gounder, Huw Oliphant
Amyloid-related vessel fragility (due to direct fibril cytotoxicity to vessel walls), platelet dysfunction, association with myeloma, and increased risk of factor X deficiency all contribute to bruising diathesis in delicate tissues such as the periocular region.21 Acquired factor X deficiency is presumed to be caused by the adsorptive removal of factor X to circulating amyloid fibrils, and its reported incidence is between 8.7% and 14% in amyloidosis patients.22,23