Case 14: Prolonged prothrombin time, acquired factor X deficiency, hemarthrosis and skin bleeding – Amyloidosis

1999 ◽  
Vol 56 (9) ◽  
pp. 523-525 ◽  
Author(s):  
Bohler ◽  
Lämmle
Keyword(s):  
Prothrombin Time ◽  
Vitamin K ◽  
Factor X ◽  
Hämorrhagische Diathese ◽  
Prolonged Prothrombin Time ◽  
Factor X Deficiency

Wir beschreiben den Fall einer 68jährigen Patientin, welche im April 1994 spontan einen Hämarthros am rechten Ellenbogengelenk erlitt. Eine Neigung zu kutanen Ekchymosen bestand seit Sommer 1993. Die damals gefundenen tiefen Quickprozentwerte um 40–50% ohne Korrektur nach prolongierter Gabe von Vitamin K wurden erst 1994 abgeklärt. Die Gerinnungsanalysen zeigten einen isolierten Faktor X (FX)-Mangel ohne Nachweis eines zirkulierenden FX-Inhibitors. Dies suggerierte die Diagnose einer systemischen Amyloidose, welche retrospektiv anhand der ein Jahr zuvor entnommenen Dünndarmbiopsien bestätigt wurde. Wegen einer Amyloidose-bedingten beidseitigen Hüftkopfnekrose und beidseitiger stark schmerzhafter Schenkelhalsfraktur mußte die Patientin im Juni 1994 operiert werden und verstarb vier Wochen später nach mehrfachen Blutungskomplikationen. Ein isolierter FX-Mangel kann hereditär bedingt sein, sollte aber immer auch an eine systemische Amyloidose denken lassen. Die mögliche hämorrhagische Diathese bei Amyloidose ist nicht nur durch den FX-Mangel, sondern auch durch die Amyloidablagerung in den (kleinen) Blutgefäßen bedingt (akquirierte vaskuläre hämorrhagische Diathese).

The Heterogeneity of Congenital factor X deficiency. A study of two unrelated patients

1979 ◽  
Author(s):  
N.R. Porter ◽  
R.G. Malia ◽  
P.C. Cooper ◽  
F.E. Preston
Keyword(s):  
Prothrombin Time ◽  
Gastrointestinal Haemorrhage ◽  
Factor X ◽  
Prolonged Prothrombin Time ◽  
Factor X Deficiency ◽  
The Family ◽  
Rate Of Decline ◽  
Consanguinous Marriage ◽  
Caucasian Female ◽  
Congenital Factor

Two unrelated patients with congenital factor X deficiency are described. R.N. a four year old Pakistani child of consanguinous marriage presented with gastrointestinal haemorrhage. Routine tests of coagulation revealed a grossly prolonged prothrombin time and KCCT. All factor assays, other than factor X, were normal. Factor X, measured by three unrelated methods, was < 1%. Factor X related antigen (FXRAg) measured by antibody neutralisation was undetectable. In other, less affected members of the family FXC/FXRAg = 1 An infusion of factor X concentrate raised the factor X to 140%. The rate of decline was biphasic with an initial rapid component, T½ = 4 hours, and a slower second component T½ = 33 hours. The second patient, P.P. a Caucasian female presented with persistent post-dental extraction haemorrhage. Routine tests of coagulation revealed a prolonged prothrombin time and KCCT. All factor assays, other than factor X, were normal. Factor X, measured by two unrelated methods was 20 - 32% (normal 58- 134%) FXRAg was 71% (normal 60 - 120%). FXC/FXRAg = 0.35. Similar ratios were obtained on three other affected members of the family. The ratio represents an Important difference between the two families. Studies using the modified thrombotest strongly suggest an abnormal molecular complex in P.P. The results provide further evidence of the heterogenity of factor X deficiency states.

The Heterogeneity of Congenital factor X deficiency

1979 ◽  
Author(s):  
N Porter ◽  
R Malia ◽  
P Cooper ◽  
F Preston
Keyword(s):  
Prothrombin Time ◽  
Gastrointestinal Haemorrhage ◽  
Factor X ◽  
Prolonged Prothrombin Time ◽  
Factor X Deficiency ◽  
The Family ◽  
Rate Of Decline ◽  
Consanguinous Marriage ◽  
Caucasian Female ◽  
Congenital Factor

Two unrelated patients with congenital factor X deficiency are described. R.N. a four year old Pakistani child of consanguinous marriage presented with gastrointestinal haemorrhage. Routine tests of coagulation revealed a grossly prolonged prothrombin time and KCCT. All factor assays, other than factor X, were normal. Factor X, measured by three unrelated methods, was < 1%. Factor X related antigen (FXRAg) measured by antibody neutralisation was undetectable. In other, less affected members of the family FXC/FXRAg = 1 An infusion of factor X concentrate raised the factor X to 140%. The rate of decline was biphasic with an initial rapid component, T½ = 4 hours, and a slower second component T½ = 33 hours. The second patient, P.P. a Caucasian female presented with persistent post-dental extraction haemorrhage. Routine tests of coagulation revealed a prolonged prothrombin time and KCCT. All factor assays, other than factor X, were normal. Factor X, measured by two unrelated methods was 20 - 32% (normal 58 - 134%) FXRAg was 71% (normal 60 - 120%). FXC/FXRAg = 0.35. Similar ratios were obtained on three other affected members of the family. The ratio represents an important difference between the two families. Studies using the modified thrombotest strongly suggest an abnormal molecular complex in P.P. The results provide further evidence of the heterogenity of factor X deficiency states.

THE EFFECT OF PHTHALIC ACID ON THE PROTHROMBIN TIME OF DICUMAROL-TREATED DOGS

1945 ◽  
Vol 23e (6) ◽  
pp. 167-174 ◽  
Author(s):  
L. B. Jaques ◽  
A. P. Dunlop
Keyword(s):  
Prothrombin Time ◽  
Vitamin K ◽  
Phthalic Acid ◽  
Prolonged Prothrombin Time ◽  
Continuous Injection

Dicumarol (10 mgm./kgm.) was administered to dogs intravenously and the prothrombin time followed. The administration of vitamin K simultaneously markedly reduced the elevation of prothrombin time following dicumarol, while the administration of large doses of sodium phthalate in the form of single injections combined with a continuous injection caused a transient lowering of the prolonged prothrombin time. This effect of sodium phthalate was not altered by removal of the kidneys. The same effect was observed in the completely eviscerated normal dog. Sodium phthalate has no effect on the prothrombin time when added in vitro.

Prothrombin time prolongation in paracetamol poisoning: a relevant marker of hepatic failure?

2003 ◽  
Vol 22 (11) ◽  
pp. 617-621 ◽  
Author(s):  
C Payen ◽  
A Dachraoui ◽  
C Pulce ◽  
J Descotes
Keyword(s):  
Liver Failure ◽  
Prothrombin Time ◽  
Vitamin K ◽  
Hepatic Failure ◽  
Paracetamol Overdose ◽  
Paracetamol Poisoning ◽  
Clotting Factors ◽  
Prolonged Prothrombin Time ◽  
Few Data

The association between paracetamol overdose and prolonged prothrombin time due to hepatic failure is well recognized. However, little is known of the possibility that paracetamol overdose can prolong the prothrombin time without overt hepatic failure. The few data from the literature suggest this is either due to a reduction in the functional levels of the vitamin K-dependent clotting factors by elevated doses of paracetamol, or a consequence of the administration of the antidote N-acetylcystein. The three reported cases provide further evidence that paracetamol overdose can be associated with a prolongation in the prothrombin time without overt hepatic failure. Even though the prothrombin time provides useful prognosis information, decisions regarding the management of these patients should not solely be based on this endpoint to avoid misinterpretation of the accuracy and the severity of liver failure.

Difenacoum Poisoning as a cause of Haematuria

1992 ◽  
Vol 11 (6) ◽  
pp. 553-554 ◽  
Author(s):  
Graham P. Butchery ◽  
Martin J. Shearer ◽  
Alan D. MacNicoll ◽  
Michael J. Kelly ◽  
Philip W. Ind
Keyword(s):  
Serum Concentration ◽  
Prothrombin Time ◽  
Vitamin K ◽  
Prolonged Treatment ◽  
Prolonged Prothrombin Time

A man presented with frank haematuria and a grossly prolonged prothrombin time. He was later found to have taken an overdose of difenacoum — a 'superwarfarin' rodenticide. The diagnosis was confirmed by a serum concentration of difenacoum of 0.6 μg ml-1. Overdosage with superwarfarins is discussed and the need for prolonged treatment with vitamin K1 highlighted.

Prediction of Vitamin K Response Using the Echis Time and Echis-Prothrombin Time Ratio

1990 ◽  
Vol 64 (03) ◽  
pp. 353-357 ◽  
Author(s):  
C Solano ◽  
R G Cobcroft ◽  
D C Scott
Keyword(s):  
Prothrombin Time ◽  
Vitamin K ◽  
Factor X ◽  
Time Ratio ◽  
Factor Ii ◽  
Before And After ◽  
K Deficiency ◽  
Echis Carinatus ◽  
Factor X Activation ◽  
K Response

Summary Echis carinatus venom contains proteases capable of activating both normal and descarboxy prothrombin. We showed this venom (Sigma) principally activates prothrombin with almost no factor X activation. Echis time in combination with prothrombin time can predict vitamin K responsiveness since the Echis time is usually normal in the presence of descarboxy prothrombin associated with vitamin K deficiency.38 patients with abnormal routine prothrombin times (PT) had both coagulant and immunogenic factor II assays along with Echis times done before and after vitamin K. Of 22 patients responding to vitamin K, based on correction of PT, 21 had normal initial Echis times and of 16 not responding, 11 had abnormal Echis times, giving a sensitivity of 95.4% and specificity of 68.8% for vitamin K responsiveness. 90% of patients with a PT/Echis time ratio <1.3 and a prolonged Echis time did not correct their PTs with vitamin K therapy.The 5 non-responders with normal Echis times all showed normal initial coagulant and antigenic prothrombin, but 3 had low F V and/or F VII.

Vitamin K status of preterm infants with a prolonged prothrombin time

2007 ◽  
Vol 94 (12) ◽  
pp. 1822-1824 ◽  
Author(s):  
Paul Clarke ◽  
Simon J Mitchell ◽  
Shanmuga Sundaram ◽  
Vibha Sharma ◽  
Robert Wynn ◽  
...  
Keyword(s):  
Prothrombin Time ◽  
Preterm Infants ◽  
Vitamin K ◽  
Prolonged Prothrombin Time

scholarly journals Prolonged Prothrombin Time After Discontinuing Vitamin K Antagonist

2017 ◽  
Vol 63 (9) ◽  
pp. 1442-1444
Author(s):  
Mark W M Schellings ◽  
Moniek P M de Maat ◽  
Sacha de Lathouder ◽  
Floor Weerkamp
Keyword(s):  
Prothrombin Time ◽  
Vitamin K ◽  
Vitamin K Antagonist ◽  
Prolonged Prothrombin Time

Inaccurate therapeutic decisions in patient with lupus anticoagulant as a result of falsely elevated prothrombin time

2015 ◽  
Vol 51 (3) ◽  
pp. 199-202
Author(s):  
Maria Magdalena Jeleńska ◽  
Katarzyna Pawlak ◽  
Monika Budnik ◽  
Hanna Zborowska
Keyword(s):  
Prothrombin Time ◽  
Vitamin K ◽  
Lupus Anticoagulant ◽  
Reference Range ◽  
Cephalic Vein ◽  
Factor V ◽  
Prolonged Prothrombin Time ◽  
Vein Thrombosis ◽  
Therapeutic Decisions ◽  
Increased Risk

71 year old patient was admitted to the hospital with a suspicion of infective endocarditis. Laboratory investigations using human recombined tromboplastin Innovin showed a considerably prolonged prothrombin time (INR above 4) and APTT. Due this vitamin K was administered (iv). During further investigations using Innovin significantly prolonged prothrombin time persisted, but using thromboplastin Technoplastin HIS was in reference range. Deficiency of vitamin K dependent coagulation factors (VII, X, II), factor V and fibrinogen were not found. Therefore a comparative test using Innovin and three other thromboplastins (Technoplastin HIS Tromborel S, RecombiPlasTin 2G) were performed. These gave prothrombin time in reference range. Prolonged aPTT resulted from presence of lupus anticoagulant. Thrombotic prophylaxis with low molecular weight heparin was applied (2 weeks after admission to the hospital). However, two days later, edema of upper limb has appeared due to cephalic vein thrombosis. There was no central catheter placed in any of central veins. The falsely elevated prothrombin time caused inaccurate therapeutic decisions such as iv supply of vitamin K, and delate antithrombotic treatment of patient with the increased risk of thrombosis (immobilization, inflammations and presence of the lupus anticoagulant). These decision surely contributed to the cephalic vein thrombosis. Our results demonstrated that protothrombin time/INR determined with thromboplastin Innovin can be prolonged also in patients with lupus anticoagulant not submitted to anticoagulation treatment. Therefore, in patients with LA before therapeutic decision, the verification of elevated prothrombin time using other than Innovin thromboplastins is necessary.

Coagulation Induced by Dilute Tissue Factor Depends More Critically on Factor II and X Concentration Than on FVII or FIX

Blood ◽  
2008 ◽  
Vol 112 (11) ◽  
pp. 4084-4084
Author(s):  
Brynja R. Gudmundsdottir ◽  
Alexia M Bjornsdottir ◽  
Pall T. Onundarson
Keyword(s):  
Prothrombin Time ◽  
Vitamin K ◽  
Platelet Rich Plasma ◽  
Coagulation Factor ◽  
Maximum Velocity ◽  
Coagulation Factors ◽  
Factor X ◽  
Platelet Poor Plasma ◽  
Stabilization Phase ◽  
Maximum Clot Firmness

Abstract Prothrombin time based tests used to monitor coumarin anticoagulation measure the initiation phase of fibrin formation (clotting time, CT) in platelet poor plasma. Additional information can be obtained using computerized rotational thromboelastometry (ROTEM) which also measures the consequent propagation phase (measured as maximum velocity, Vmax), and the stabilization phase (maximum clot firmness, MCF). We used ROTEM to study the effect of individual vitamin K dependent (VK) coagulation factor (F) concentration on clotting induced by dilute thromboplastin in platelet poor and platelet rich plasma (PPP and PRP). As expected, FII, FVII and FX in PPP equally affected the prothrombin time whereas FIX did not. In PPP the ROTEM CT, however, was affected more by the concentration of FII and FX than by FVII (data not shown). To imitate physiological clotting better, factor deficient platelet poor plasma was repleted by adding frozen platelets in an optimal concentration corresponding to 100 × 109/L.. In the PRP the ROTEM CT was more dependent on the concentration of FII than of FVII, more dependent on FX than on FII and not dependent on FIX at all (left figure). The Vmax (reflecting the propagation phase) was also more dependent on FII and factor X than on the concentration of FVII or FIX which both may demonstrate a threshold effect (right figure). The stabilization phase or maximum clot firmness (MCF, not shown) was influenced similarly by FII and FX but was not influenced by FVII or FIX. Figure Figure Conclusion: During deficiency of vitamin K dependent coagulation factors the concentration of FII and FX may be more critical for hemostasis than FVII or FIX concentration. This may have practical implications for the appropriate choice of monitoring assays during coumarin administration.

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