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Pitfalls in Coagulation Testing: A Pediatric Case of Lupus Anticoagulant Hypoprothrombinemia Syndrome Following
Taiki Homma1, Takuya Kamio1, Kimihiko Oishi1
1Department of Pediatrics, The Jikei University School of Medicine, Tokyo, JPN.
Insights
Lupus anticoagulant (LA) can transiently occur in children post-infection, rarely causing LA-hypoprothrombinemia syndrome (LAHPS). This case highlights LA interference with coagulation tests, mimicking other bleeding disorders.
Area of Science:
- Pediatric Hematology
- Clinical Coagulation
- Infectious Disease Complications
Background:
- Lupus anticoagulant (LA) in children is typically transient post-infection.
- LA can rarely lead to LA-hypoprothrombinemia syndrome (LAHPS), causing bleeding tendencies.
- LAHPS presents with complex coagulation abnormalities that can be misdiagnosed.
Abstract:
In children, the lupus anticoagulant (LA) is transiently produced after infection and rarely causes bleeding tendency, known as LA-hypoprothrombinemia syndrome (LAHPS). In this case report, we describe a child presenting with difficulty standing and walking after enterocolitis and complicated false coagulation abnormalities. Prolonged prothrombin time and activated partial thromboplastin time (APTT); decreased factors II, VIII, IX, XI, and XII; and inhibitors for factors VIII and IX were detected. On the basis of such findings as inhibitor pattern in the APTT cross-mixing test and positivity for serum LA, LAHPS was diagnosed. Physicians should be aware of the interference by LA with phospholipid-based coagulation tests, leading to the misinterpretation of the unusual coagulation abnormalities in LAHPS.
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