Systemic Amyloidosis Associated With Factor X Deficiency

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


1977 ◽  
Vol 297 (2) ◽  
pp. 81-85 ◽  
Author(s):  
Bruce Furie ◽  
Edith Greene ◽  
Barbara C. Furie

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.


1992 ◽  
Vol 68 (06) ◽  
pp. 648-651 ◽  
Author(s):  
I Schwarzinger ◽  
M Stain-Kos ◽  
R Bettelheim ◽  
I Pabinger ◽  
P Kyrle ◽  
...  

SummaryWe describe the case of a 64-year-old woman with isolated severe factor X deficiency associated with kappa light chain myeloma. At the time of diagnosis there was no evidence for amyloidosis. Complete remission (CR) of myeloma as well as normalization of factor X levels were achieved after cytostatic chemotherapy. Subsequently, factor X deficiency recurred twice without any evidence for relapse of myeloma. The first time factor X normalized again following cytostatic treatment, the second time, however, factor X deficiency was refractory to chemotherapy. Finally, relapse of myeloma became evident associated with rapidly progressing, systemic amyloidosis, which was fatal within a few months. Initially, factor X infusion studies showed a normal recovery, but when amyloidosis became overt the recovery decreased to 0%. We assume that factor X deficiency was due to a binding of factor X to kappa light chains associated with the proliferation of the malignant myeloma cell clone.


1987 ◽  
Vol 54 (5) ◽  
pp. 399-406 ◽  
Author(s):  
F. V. Lucas ◽  
A. J. Fishleder ◽  
R. C. Becker ◽  
D. S. Cavalier ◽  
R. R. Tubbs

Haemophilia ◽  
2013 ◽  
Vol 19 (6) ◽  
pp. e375-e376 ◽  
Author(s):  
P. Chaudhary ◽  
J. Wakim ◽  
H. Khadim ◽  
K. K. Karumbaiah ◽  
A. Gajra

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