Paediatria Croatica, Vol. 68 No. 1, 2024.
Stručni rad
https://doi.org/10.13112/PC.2024.7
Acquired coagulopathy in a three-year-old boy with macrohaematuria – a case report
Karolina Malić Tudor
orcid.org/0000-0002-4951-0592
; KBC Split
*
Višnja Armanda
* Dopisni autor.
Sažetak
Acquired coagulopathy is a rare, potentially life-threatening disorder which should be suspected if acute bleeding in patients with a
negative previous hemorrhagic diathesis is encountered. This disease arises due to the formation of antibodies against clotting
factors in a previously healthy child or adult, which specifically partially or completely neutralize the procoagulant activity of clotting
factors or accelerate their removal from the circulating blood, reducing their plasma concentrations and increasing the bleeding
tendency.
The aforementioned disorder should be confirmed by determining global coagulation times, concentrations of individual clotting
factors, and determining the presence of specific or non-specific antibodies. Timely treatment aimed at prompt stopping of bleeding
and eradicating the resulting inhibitors will prevent the unfavorable outcome. It is recommended to continue monitoring patients in
the outpatient setting with determination of PT and APTT for a year, because in 20% of cases a relapse of the disease occurs, although
more often in adults.
In this paper, we presented a three-year-old boy in whom we determined prolonged global coagulation times and the presence of
non-specific inhibitors of the coagulation factors F II, F IX, F XI and F XII through the diagnostic workup of macrohematuria. The
treatment with fresh frozen plasma and corticosteroids resulted in clinical improvement and disease remission.
Acquired coagulopathy is a very rare disorder in childhood with scarce literature data. The aim of this paper is to demonstrate the
possible causes, clinical course and treatment of this life-threatening disorder in children.
Ključne riječi
Hemostasis; Coagulation Factors; Blood Coagulation Factor Inhibitors; Children; Bleeding
Hrčak ID:
317039
URI
Datum izdavanja:
31.3.2024.
Posjeta: 474 *