Severe Bleeding Diathesis From Acquired Factor XIII Inhibitor Secondary to SLE

CHEST Journal ◽  
2013 ◽  
Vol 144 (4) ◽  
pp. 924A
Author(s):  
Ian Lee ◽  
Jo Persoon-Gundy ◽  
Ching-Fei Chang
Keyword(s):  
Factor Xiii ◽  
Severe Bleeding ◽  
Bleeding Diathesis

scholarly journals Factor XIII-A: An Indispensable “Factor” in Haemostasis and Wound Healing

2021 ◽  
Vol 22 (6) ◽  
pp. 3055
Author(s):  
Fahad S. M. Alshehri ◽  
Claire S. Whyte ◽  
Nicola J. Mutch
Keyword(s):  
Bone Marrow ◽  
Wound Healing ◽  
Crucial Role ◽  
Factor Xiii ◽  
Severe Bleeding ◽  
Bleeding Diathesis ◽  
Unknown Mechanism ◽  
Protein Substrates ◽  
Physiological Processes ◽  
Isopeptide Bonds

Factor XIII (FXIII) is a transglutaminase enzyme that catalyses the formation of ε-(γ-glutamyl)lysyl isopeptide bonds into protein substrates. The plasma form, FXIIIA2B2, has an established function in haemostasis, with fibrin being its principal substrate. A deficiency in FXIII manifests as a severe bleeding diathesis emphasising its crucial role in this pathway. The FXIII-A gene (F13A1) is expressed in cells of bone marrow and mesenchymal lineage. The cellular form, a homodimer of the A subunits denoted FXIII-A, was perceived to remain intracellular, due to the lack of a classical signal peptide for its release. It is now apparent that FXIII-A can be externalised from cells, by an as yet unknown mechanism. Thus, three pools of FXIII-A exist within the circulation: plasma where it circulates in complex with the inhibitory FXIII-B subunits, and the cellular form encased within platelets and monocytes/macrophages. The abundance of this transglutaminase in different forms and locations in the vasculature reflect the complex and crucial roles of this enzyme in physiological processes. Herein, we examine the significance of these pools of FXIII-A in different settings and the evidence to date to support their function in haemostasis and wound healing.

scholarly journals Neutralizing autoantibody against factor XIII A subunit resulted in severe bleeding diathesis with a fatal outcome - characterization of the antibody

2016 ◽  
Vol 14 (8) ◽  
pp. 1517-1520 ◽  
Author(s):  
K. Pénzes ◽  
K. Rázsó ◽  
É. Katona ◽  
A. Kerényi ◽  
M. Kun ◽  
...  
Keyword(s):  
Factor Xiii ◽  
Fatal Outcome ◽  
Severe Bleeding ◽  
Bleeding Diathesis ◽  
A Subunit

scholarly journals Novel treatment for congenital FXIII deficiency

Blood ◽  
2012 ◽  
Vol 119 (22) ◽  
pp. 5060-5061 ◽  
Author(s):  
Hans P. Kohler
Keyword(s):  
Factor Xiii ◽  
Autosomal Recessive ◽  
Rare Disorder ◽  
Severe Bleeding ◽  
Bleeding Diathesis ◽  
Inherited Disease ◽  
Novel Treatment ◽  
Fxiii Deficiency ◽  
Congenital Factor

Congenital Factor XIII (FXIII) deficiency is a rare autosomal recessive inherited disease leading to severe bleeding diathesis. In this issue of Blood, Inbal and colleagues report on a safe and novel treatment of this rare disorder with recombinant FXIII (rFXIII).1

Kardiochirurgische Eingriffe bei schweren Blutungsdiathesen

2012 ◽  
Vol 32 (S 01) ◽  
pp. S83-S86 ◽  
Author(s):  
R. Schobess ◽  
A. Siegemund ◽  
C. J. Correia ◽  
J. Oppermann ◽  
J. Banusch ◽  
...  
Keyword(s):  
Case Report ◽  
Cardiovascular Diseases ◽  
Bleeding Risk ◽  
Coagulation Factors ◽  
Developed Countries ◽  
Severe Bleeding ◽  
Bleeding Diathesis ◽  
Concomitant Therapy ◽  
Kardiochirurgische Eingriffe ◽  
The Developed Countries

SummaryCardiovascular diseases are the most common disorder in the developed countries. Invasive cardiological and cardiosurgical techniques are known therapies.Yet, patients with severe hereditary haemorrhagical diseases (haemophilia, rare deficiencies of coagulation factors) have an increased bleeding risk by the use of anticoagulants. Therefore, the treatment of these patients requires a concomitant therapy.This article shows eight patients with a severe bleeding diathesis and cardiosurgical interventions in the years 2006 to 2011. This case report shall demonstrate that an adequate therapy can be accomplished with the help of a good cooperation between haemostaseologists and colleagues of the cardioinvasive/ cardiosurgical disciplines.

Concomittant Acquired Von Willebrand Disease and Acquired Factor V Deficiency Causing a Severe Bleeding Diathesis Due To Multiple Myeloma With Amyloidosis

Blood ◽  
2013 ◽  
Vol 122 (21) ◽  
pp. 4788-4788
Author(s):  
Nataliya Melnyk ◽  
Jonathan Harrison
Keyword(s):  
Blood Count ◽  
Case Reports ◽  
Von Willebrand Disease ◽  
Severe Bleeding ◽  
Factor V ◽  
Factor X ◽  
Bleeding Diathesis ◽  
Acquired Von Willebrand Disease ◽  
Factor V Deficiency ◽  
Von Willebrand

Background Acquired coagulopathies are a common problem in Hematology, and are most often due either to medication effect, liver disease, or consumption. Among the uncommon causes of acquired coagulopathy, inhibitory auto-antibodies may develop, either in the setting of autoimmune diseases, in the setting of lymphoproliferative disorders, or as isolated inhibitory immunoglobulins. Uncommonly, the adsorption of coagulation factors from the circulation into the tissues by extracellular deposition of pathologic amyloid results in an acquired factor deficiency, due to clearance of factor from the circulation that exceeds the body's ability to produce factor. When amyoidosis does cause a coagulapathy, it is most often the result of the adsorption of Factor X by the amyloid protein, resulting in an acquired Factor X deficiency. However, there are rare reports of amyloidosis being associated with other factor deficiencies. We report a case of amyloidosis that was associated with a severe bleeding diathesis, with the etiology of the bleeding disorder being due to both acquired Factor V deficiency and concomitant acquired von Willebrand Disease. Case Report A previously healthy 51-year-old gentleman presented to an outside medical center for evaluation and management of recurrent bleeding episodes. The patient had a prior medical history significant only for right ankle trauma in the year 2005, following which he underwent a total of 4 surgical procedures; there was no excessive bleeding complicating the patient's surgeries. He was then in his usual state of health until September, 2012 when he developed onset of severe abdominal pain and was admitted to the outside facility. Following hospitalization for several months at the outside facility, he was admitted to our institution. Physical examination was remarkable for extensive ecchymoses, and for splenomegaly to 18 cm. span by exam, confirmed by imaging. CT scan showed multiple peri-caval and periaortic nodes present up to 1.7 cm in size, with shotty inguinal lymph nodes. A complete blood count showed White blood count 21,600, hemoglobin 8.0 g/dL, hematocrit 24%, platelet count 370,000, Hepatic function studies and renal function studies, as well as electrolytes, were normal on admission. Coagulation studies revealed Prothrombin Time prolonged at 16.8 seconds (normal < 12.7), aPTT prolonged at 44. Mixing patient plasma with equal volume normal plasma corrected both the PT and aPTT. Detailed factor assays showed markedly decreased Factor V activity of 27%; Ristocetin Cofactor activity was markedly decreased at 49%, but von Willebrand antigen was elevated at 213%. Multimer analysis was consistent with Type II vWD (see figure 1). The patient received fresh frozen plasma and Humate P, with transient correction of the bleeding diathesis. This permitted inguinal lymph node biopsy, which documented AL amyloidosis. Extraction of the protein from the lymph node documented AL lambda light chain amyloid (see figure 2). Marrow biopsy documented IgG lambda multiple myeloma. The patient was treated using Bortezumib plus Dexamethasone, and achieved a complete remission, with normalization of the coagulation parameters and factor levels over the following several months. His bleeding diathesis has fully resolved, and Karnofsky performance status improved to 100%. Conclusion Although there are several case reports of acquired von Willebrand disease on the basis of amyloidosis, and several case reports of acquired Factor V deficiency on the basis of amyloidosis, this appears to be the first reported case of both acquired vWD and acquired Factor V deficiency on the basis of amyloidosis. Disclosures: No relevant conflicts of interest to declare.

Severe Bleeding Diathesis Associated with Moxalactam Administration

1984 ◽  
Vol 18 (9) ◽  
pp. 721-722 ◽  
Author(s):  
Steven Meisel
Keyword(s):  
Patient Monitoring ◽  
Mechanisms Of Action ◽  
Severe Bleeding ◽  
Bleeding Diathesis

A case is presented of a severe bleeding diathesis associated with moxalactam administration that was confirmed on rechallenge. Potential mechanisms of action and recommendations for patient monitoring are discussed.

scholarly journals Fatal intracerebral hemorrhage secondary to Lonomia obliqua caterpillar envenoming: case report

2006 ◽  
Vol 64 (4) ◽  
pp. 1030-1032 ◽  
Author(s):  
Pedro André Kowacs ◽  
Juliana Cardoso ◽  
Marlene Entres ◽  
Edison Mattos Novak ◽  
Lineu César Werneck
Keyword(s):  
Case Report ◽  
Intracerebral Hemorrhage ◽  
Clinical Features ◽  
Healthy Woman ◽  
Severe Bleeding ◽  
Bleeding Diathesis ◽  
Pertinent Literature ◽  
Intracerebral Hemorrhages ◽  
Brain Hemorrhages ◽  
Lonomia Obliqua

The case of a 70 year-old, previously healthy woman who developed a severe bleeding diathesis shortly after touching a Lonomia obliqua caterpillar and finally died from multiple intracerebral hemorrhages is described. Brain hemorrhages are the leading cause of death in patients envenomed by the Lonomia species. The pertinent literature is reviewed and the most relevant clinical features highlighted, with emphasis on diagnosis. The use of new therapeutic options such as anti-Lonomia serum is discussed.

Relevant bleeding diathesis due to acquired factor XIII deficiency

2013 ◽  
Vol 33 (S 01) ◽  
pp. S50-S54 ◽  
Author(s):  
M. Janning ◽  
K. Holstein ◽  
B. Spath ◽  
C. Schnabel ◽  
P. Bannas ◽  
...  
Keyword(s):  
Factor Xiii ◽  
False Lumen ◽  
Surgical Patients ◽  
Major Surgery ◽  
Severe Bleeding ◽  
Acute Type ◽  
Activity Levels ◽  
Bleeding Tendency ◽  
Limited Information ◽  
Fxiii Deficiency

SummaryAcquired factor XIII (FXIII) deficiency is associated with reduced clot firmness and increased bleeding in patients undergoing major surgery. In contrast, only limited information is available on the haemostatic relevance of acquired FXIII deficiency in non-surgical patients.An 81-year-old patient, who had experienced acute type-A dissection of the aorta eight years earlier, presented with a 3-year history of progressive mucocutaneous and softtissue bleeding. Diagnostic work-up was unremarkable for global coagulation tests, but FXIII and alpha2-antiplasmin were decreased to 33% and 27%, respectively, while plasma D-dimer was elevated to > 35 mg/l. A FXIII inhibitor was excluded by mixing studies. CT scanning revealed a massively elongated and progressively dilated aorta with a false lumen reaching from the left carotid artery to the iliac bifurcation. Bleeding control was achieved by single doses of FXIII at 20-30 IU/ kg body weight and tailored oral tranexamic acid.Acquired FXIII deficiency with activity levels of 30–35% may confer a severe bleeding tendency in non-surgical patients, especially in the context of increased thrombin an fibrin generation.

The Incidence and Clinical Significance of Amiodarone and Acenocoumarol Interaction

1989 ◽  
Vol 62 (03) ◽  
pp. 906-908 ◽  
Author(s):  
Yoseph Caraco ◽  
Tova Chajek-Shaul
Keyword(s):  
Control Group ◽  
Severe Bleeding ◽  
Sensitivity Index ◽  
Amiodarone Therapy ◽  
Bleeding Diathesis ◽  
Initial Reduction ◽  
International Normalised Ratio ◽  
Daily Dose ◽  
Before And After ◽  
Acenocoumarol Interaction

SummaryAcenocoumarol sensitivity index (ASI) was calculated in 36 patients receiving amiodarone and acenocoumarol concomitantly, by dividing the acenocoumarol daily dose (μg kg−1 day−1) by the prothrombin ratio expressed as International Normalised Ratio (INR). It was found to be significantly lower (4.2 ± 0.3 pg kg−1 day−1) than the ASI (11 ± 0.6 μg kg−1 day−1) determined in 35 patients in the control group, who received acenocoumarol and not amiodarone. In 15 patients, ASI was 13.2 ± 1.4 and 4.2 ± 0.6 μg kg−1 day−1 before and after initiation of amiodarone therapy, respectively. In 5 patients, ASI was 6.2 ± 0.6 μg kg−1day−1, while patients were receiving both medications, and 28.1 ± 6.1 μg kg−1 day−1 following discontinuation of amiodarone therapy. ASI determined while patients were on both medications was not affected by the presence of mild impaired renal or liver function. In 7 patients treated with both drugs concomitantly, severe bleeding diathesis developed. It is concluded that amiodarone augmented the anticoagulant effect of acenocoumarol in all patients receiving both drugs. An initial reduction of acenocoumarol daily dose by 50% and further adjustment of acenocoumarol dose according to daily prothrombin ratio is recommended. It is also suggested that acenocoumarol dose should be adjusted after cessation of amiodarone therapy.

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