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Published on: November 1, 2015
[Lupus anticoagulant in children. Report of 4 cases]
M C de Hoyos López1, J M Pascual Pérez, A Blanco Quirós
1Sección de Hematología infantil, Hospital universitario Valladolid.
Insights
Lupus anticoagulant (AL) in children is challenging to diagnose and often underestimated. Early detection is crucial as it can indicate bleeding risks or thrombotic events.
Area of Science:
- Pediatric Hematology
- Immunology
Background:
- Lupus anticoagulant (AL) is a pro-thrombotic autoantibody.
- Literature on pediatric AL is scarce, particularly in certain regions.
Observation:
- Four pediatric cases of AL were identified over a decade.
- Nine children had antiphospholipid antibodies, with four confirmed as lupus anticoagulant.
- Patients ranged from 4 to 13 years old, with a male predominance.
Findings:
- Half of the children had a family history of bleeding disorders, correlating with hemorrhagic or thrombotic symptoms.
- Three of four AL cases were transient and associated with acute respiratory infections.
- One child experienced recurrent lower extremity thromboses without other symptoms.
- Anticardiolipin antibodies were present in two patients, associated with recurrent AL episodes, one with thrombosis and one asymptomatic.
Implications:
- Diagnosing pediatric AL is difficult and likely underreported.
- While often transient, AL can manifest in recurrent episodes.
- Early identification of AL is vital due to potential links with factor II deficiency, bleeding, and thrombosis.
Objective:
We present four cases of lupus anticoagulant (AL) in children. In addition, as a result of the lack of literature published on the subject in our country. We also evaluate the epidemiological, clinical and prognostic characteristics of AL in children.
Patients And Methods:
The diagnostic criteria established by the "Subcommittee for the Standardization of Lupus Anticoagulant" were followed.
Results:
Over a ten-year period (1988-1998), 46 cases of children with TTPA prolongation were documented. Nine children showed circulating anticoagulant, 4 of which were lupus type anticoagulants. The age of the patients ranged from 4 to 13 and there was a prevalence of males (3/4). Half of the children had a family history of bleeding dyscrasia and it was these who showed hemorrhage or thrombotic symptoms. The case that started with ecchymosis and hemorrhaging showed prothrombin prolongation due to factor II deficit. In 3 of the 4 children, AL was linked to acute respiratory infections and was transitory. The other coincided with two thrombotic episodes of the lower extremities in a healthy child. Positive anticardiolipin antibodies were detected in two patients, both showing repeated AL episodes, one with thrombosis and the other always asymptomatic.
Conclusions:
Diagnosis of AL in children is difficult and has probably been underestimated. Although it is usually transitory, it can appear in repeated episodes. Its early detection is important as it can be linked to both prothrombin deficit, as well as significant symptoms of hemorrhaging.
