Acquired Factor X (Stuart Factor) Deficiency in a Patient with Mycoplasma Pneumonial Infection

1979 ◽  
Author(s):  
F Peuscher ◽  
W van Aken ◽  
A Swaak ◽  
L Sie ◽  
L Statius van Eps

An isolated deficiency of factor X is known to occur in a hereditary form, the Stuart-Prower disease, and in an acquired form in some patients with para-proteinaemia and sporadically in systemic amyloidosis. Transient deficiency of factor X in the presence of normal levels of factors II, VII and V appears to be rare. In the literature, only three cases have been described. We have studied a patient with a severe haemorrhagic diathesis and concomitant mycoplasma pneumonial infection. The bleeding tendency proved to be due to isolated factor X deficiency. No circulating inhibitors of factor X were present. Systemic amyloidosis could not be demonstrated. Factor X-related antigen could not be detected (this test was kindly performed by Dr.Daryl S.Fair, Scripps Clinic and Research Foundation, La Jolla, U.S.A.). Treatment with vitamin K, prothrombin complex concentrate, fresh plasma and whole blood proved not to influence factor X activity in the patient’s plasma. However, 20 days after admission⋅to hospital both factor X activity and antigen spontaneously returned to normal. These results suggest that the synthesis of factor X was transiently defective. Since other conditions known to affect factor X activity could not be demonstrated, it is postulated that the acquired factor X deficiency in this patient was related to the infection with mycoplasma pneumoniae.

1979 ◽  
Author(s):  
F.W. Peuscher ◽  
W.G. van Aken ◽  
A.J.G. Swaak ◽  
L.H. Sie ◽  
L.W. Statius van Eps

An isolated deficiency of factor X is known to occur in a hereditary form, the Stuart-Prower disease, and in an acquired form in some patients with para-proteinaemia and sporadically in systemic amyloidosis. Transient deficiency of factor X In the presence of normal levels of factors II, VII and V appears to be rare. In the literature, only three cases have been described. We have studied a patient with a severe haemorrhage diathesis and concomitant mycoplasma pneumonlal infection. The bleeding tendency proved to be due to isolated factor X deficiency. No circulating inhibitors of factor X were present. Systemic amyloidosis could not be demonstrated. Factor X-related antigen could not be detected (this test was kindly performed by Dr. Daryl S. Fair, Scripps Clinic and Research Foundation, La Jolla, U.S.A.). Treatment with vitamin K, prothrombin complex concentrate, fresi plasma and whole blood proved not to influence factor X activity in the patient’s Plasma. However, 20 days after admission·to hospital both factor X activity and antigen spontaneously returned to normal. These results suggest that the synthesis of factor X was transiently defective. Since other conditions known to affect factor X activity could not be demonstrated, it is postulated that the acquired factor X deficiency in this patient was related to the infection with mycoplasma pneumoniae.


1962 ◽  
Vol 07 (03) ◽  
pp. 558-566 ◽  
Author(s):  
Kristoffer Korsan-Bengtsen ◽  
Peter F Hjort ◽  
Johan Ygge

SummaryA patient with extensive amyloidosis and a selective factor X deficiency is described. The following observations indicate that the factor X deficiency in this case is not inherited.1. The first symptoms of a bleeding tendency appeared at an age of 50 years.2. The patient’s four children had no clotting defect.3. After infusion of 1 liter of fresh plasma no increased factor X activity was observed. No anticoagulants could be demonstrated in vitro.


Author(s):  
Antonio Girolami ◽  
Diana Noemi Garcia de Paoletti ◽  
Marcelo Leonardo Nenkies ◽  
Silvia Ferrari ◽  
Hugo Guglielmone

Background: Investigation of rare bleeding disorders in Latin-America. Objective: The report of a new case of FX deficiency due to a compound heterozygosis. Methods: Accepted clotting procedures were used. Sequencing of DNA was carried out by means of Applied Biosystems Instruments. Results: A compound heterozygote due to the association of a new mutation (Gla72Asp) with an already known mutation (Gly154Arg) of the FX gene is reported. The proposita is a 38 year old female who had a moderate bleeding tendency (menorrhagia, epistaxis, easy bruising). The proposita has never received substitution therapy but in the occasion of a uterine biopsy. The mother was asymptomatic but was a heterozygote for the new mutation. The father was asymptomatic but had deserted the family and could not be investigated. After this abandonment the mother of the proposita re-married with an asymptomatic man and she gave birth to a son who was asymptomatic but was also heterozygous for the new mutation (Gla72Asp). As a consequence it has to be assumed that the first husband of the mother of the proposita was heterozygous for the known mutation (Gly154Arg). Conclusion: This is the third case of a new mutation in the FX gene reported, during the past few years, in Argentina.


Haemophilia ◽  
2009 ◽  
Vol 15 (1) ◽  
pp. 401-403 ◽  
Author(s):  
L. BOWLES ◽  
K. BAKER ◽  
K. KHAIR ◽  
M. MATHIAS ◽  
R. LIESNER

1984 ◽  
Vol 34 (3) ◽  
pp. 639-647
Author(s):  
Hiroyuki Shibuya ◽  
Norio Azumi ◽  
Fumihiko Abe ◽  
Masataka Deno ◽  
Shohki Sakurama

2018 ◽  
Vol 07 (01) ◽  
pp. e12-e15 ◽  
Author(s):  
Ahmed Elmahrouk ◽  
Mohamed Ismail ◽  
Abdulbadee Bugis ◽  
Nashwa Badawy ◽  
Hesham Aboelghar ◽  
...  

Background Factor X deficiency (also known as Stuart–Prower factor deficiency) is an autosomal recessive extremely rare hereditary hematologic disorder, affecting around 1:1,000,000 of the general population. Case Presentation This case report describes a patient with hypoplastic left heart syndrome and severe factor X deficiency, who underwent staged surgical palliation. From stage 1 Norwood palliation, through superior cavopulmonary anastomosis and ending with total cavopulmonary connection with satisfactory hemostasis and no significant perioperative bleeding complication. Conclusion The need to maintain hemostasis while aiming to prevent intracardiac thrombosis requires multidisciplinary team approach including hematologist, cardiac surgeon, pediatric cardiac intensivist, and anesthesiologist along with meticulous hemostasis during surgery and careful monitoring of coagulation profile in the postoperative period.


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