Fletcher Factor Deficiency, Source of Variations of the Activated Partial Thromboplastin Time Test

Entes, Kenneth; LaDuca, Frank M.; K, Tourbaf

doi:10.1093/ajcp/75.4.626

Cited by 39 publications

(28 citation statements)

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“…Twenty-one out of the 89 patients had hypertension or hypertension-related conditions [6,8,12,14,15,[17][18][19][20][21][22][23][24][25][26][27][28][29][30]. Twelve were men and nine women (Table 1).…”

Section: Resultsmentioning

confidence: 99%

Prevalence of hypertension and its complications in congenital prekallikrein deficiency

Girolami¹,

Ferrari²,

Cosi³

et al. 2015

Blood Coagulation &Amp; Fibrinolysis

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The extra coagulation effects of prekallikrein and of the other factors of the contact phase of blood clotting have received great attention in the past few years.The clinical observation that hypertension was present in two families with congenital prekallikrein deficiency prompted a survey of all reported cases of this disorder.Altogether, 89 cases of proven prekallikrein deficiency have been described in the literature. Hypertension or vascular complications of it were found in 21 patients (12 men and nine women). If the analysis is limited to patients over 25 years of age, the number becomes 21 out of 64 cases (38.2%).This prevalence is much higher than that seen for other conditions occasionally found in patients with prekallikrein deficiency, namely hyperthyroidism, lupus erythematosus, chronic lymphocytic leukemia, kidney malformation, peptic ulcer, and myelofibrosis (1-2%).These results indicate the need to investigate further the relation between prekallikrein deficiency and hypertension.

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Section: Resultsmentioning

confidence: 99%

Prevalence of hypertension and its complications in congenital prekallikrein deficiency

Girolami¹,

Ferrari²,

Cosi³

et al. 2015

Blood Coagulation &Amp; Fibrinolysis

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“…The detection level of HMWK deficiency in the studies by Turi et al and Marlar et al varied from 2 to 34% (32,34). PK deficiency was not detected by some APTT methods (34,36,37) whereas with other reagents only levels <1.5% were detected (32,34). Entes et al describe a clearly prolonged APTT at PK levels of 1.7% but the lowest detection level is not known (37).…”

Section: Discussionmentioning

confidence: 94%

“…The detection rate of contact factor deficiencies however may be considerably lower. To our knowledge, there are five studies reporting on the sensitivity of different APTT assays for HMWK and PK deficiency (29,32,34,36,37). Hathaway et al only examined patients with PK and HMWK levels <1% which were detected by all reagents (29).…”

Section: Discussionmentioning

confidence: 99%

Contact factor deficiencies and cardiopulmonary bypass surgery: detection of the defect and monitoring of heparin

Veen

Laidlaw

Swanevelder

et al. 2009

European J of Haematology

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Contact factor pathway deficiencies do not cause surgical bleeding but make heparin monitoring by the activated partial thromboplastin time (APTT) and activated clotting time (ACT) unreliable. Heparin monitoring during cardiopulmonary bypass (CPB) surgery in these patients is particularly challenging. Here we describe heparin monitoring during CPB using the chromogenic anti Xa assay in two patients with severe factor XII deficiency (FXII < 0.01 U/mL) and one patient with severe prekallikrein (PK) deficiency (PK < 0.01 U/mL). Anti Xa levels of the three patients during CPB varied between 3.8 and 4.8 U/mL in keeping with a control group (mean anti Xa 4.5 U/mL and ACT > 480 s). There were no bleeding or thrombotic complications. We also found that detection of severe PK deficiency by the APTT in the PK deficient patient was dependent on the reagent used and discuss the sensitivity of different APTT reagents for contact factor deficiencies. We conclude that the sensitivity of APTT methods for contact pathway deficiencies is highly variable and although insensitivity is not a clinical problem in terms of bleeding, it can be a cause of discrepancy between different APTT reagents and the ACT. This can lead to confusion about a possible haemorrhagic tendency and delays in surgery. If these patients need to undergo cardiac surgery requiring high dose heparin treatment, monitoring by chromogenic anti Xa assay is a good alternative.

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“…Die überwiegende Anzahl der Personen mit Präkallikreinmangel wies keine klinische Symptomatik auf, so dass es sich meist um zufällige Entdeckungen bei der Durchführung einer gerinnungsanalytischen Untersuchung handelte [2,3,5,6,9,13]. Über Operationen bei diesen Personen wurde vereinzelt berichtet [13,16].…”

Section: Prekallikrein (Fletcher Factor) Deficiency and Prolongation unclassified

Pr�kallikrein-(Fletcher-Faktor-)Defekt als Ursache einer pr�operativen APTT-Verl�ngerung

Dietzel

Lutze

Kätzel

et al. 2003

Medizinische Klinik

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Fletcher Factor Deficiency, Source of Variations of the Activated Partial Thromboplastin Time Test

Cited by 39 publications

References 0 publications

Prevalence of hypertension and its complications in congenital prekallikrein deficiency

Prevalence of hypertension and its complications in congenital prekallikrein deficiency

Contact factor deficiencies and cardiopulmonary bypass surgery: detection of the defect and monitoring of heparin

Pr�kallikrein-(Fletcher-Faktor-)Defekt als Ursache einer pr�operativen APTT-Verl�ngerung

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