The Paradox : Bleeding Presentation in Antiphospholipid Syndrome
ISTH Academy. Selvaratnam V. Jul 9, 2019; 264570; PB1380 Topic: Acquired Hemorrhagic Coagulation Disorders
Dr. Veena Selvaratnam
Dr. Veena Selvaratnam
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PB1380

The Paradox: Bleeding Presentation in Antiphospholipid Syndrome

V. Selvaratnam, J. Sathar, P. Chelliah
Hospital Ampang, Haematology, Ampang, Malaysia

Main Topic: Hemophilia and Bleeding (including Transfusion)
Category: Acquired Hemorrhagic Coagulation Disorders

Background: Lupus anticoagulant-hypoprothrombinemia syndrome (LA-HPS) is an extremely rare acquired disorder caused by prothrombin antibodies. The predisposition is bleeding, unlike antiphospholipid syndrome (APS) characterised by thrombosis. Clinical manifestation of LA-HPS varies greatly and diagnosis is confirmed by identification of reduced prothrombin levels. There are no standardized treatment recommendations for hemorrhage associated with the syndrome; corticosteroids are usually the first-line treatment.
Aims: We present our first case of LA-HPS.
Methods: Patient´s history was retrieved from medical records.
Results: He is 12 years old, born term with unremarkable medical and family history. He presented with generalised bruising following 5 days of fever.
Examination revealed a palpable liver, 2cm subcostally and multiple bruises over his limbs.Blood investigations showed thrombocytopenia and coagulopathy. Blood flim was unremarkable. Complements were low with C3 (0.5g/dl) and C4(0.04g/dl). ANA was negative. Infectious screen was negative. Coagulopathy was not corrected with plasma transfusions and IV Vitamin K.
Bone marrow examination was suggested but parents refused. He was readmitted one month later with right thigh swelling after a trivial trauma and intermittent spontaneous gum bleeding and bruising of lower limbs.Ultrasound of right thigh revealed an intramuscular hematoma. He was transfused plasma daily but coagulation remained prolonged and heamatoma persisted.
He was then referred to our team for further counsult.


First Admission Second Admission
Date (2018) 6/7 8/7 9/7 14/8 15/8 17/8 19/8 20/8 22/8
Transfusion 4 FFP 8 FFP & 8 Cryo 4FFP & 4 cryo 4 FFP 4FFP 4FFP 8FFP 4 FFP 4FFP & 2 packed cells
PT(sec) 19.8 18.8 17.9 28.2 24.5 26 25.2 24.5 25.5
APTT (sec) 86.1 84.2 74.2 127.1 103.5 118.2 125.2 120.9 120.5
INR 1.71 1.6 1.5 2.71 2.26 2.44 2.34 2.26 2.33
[Blood results At Initial Presentation]




Date (2018) 30/8 19/9 18/10 29/11 31/1/2019
PT / PT mix 37.1 / 14.7 21.7 / - 22.9 / - 21.1 / - 21.5/ -
APTT-LA/ APTT-LA mix 159.7 / 129.2 70.0 / 58.9     73.5 / 46.5
DRVV/ DRVVmix(screen) 196.3 / 191.9 93.9 / 88.5     89.6 / 83.9
LA results Strong positive for LA Weak positive for LA     Weak positive for LA
Factor II Levels(%) 3.50 5.70 4.80 6.80 7.20
Factor levels with serial Dilution (%) on Presentation: Neat 1:2 1:4 1:8 1:16
FactorII 3.50 2.00 1.10 0.50 0.25
Factor IX 0.24 0.31 0.24 1.95 5.20
Factor XI 0.20 0.20 0.30 1.10 4.30
[Investigation results at Ampang Hospital]


Serial plasma dilution showed decreasing Factor II levels indicating true prothrombin deficiency while other factors increasing indicating non specific inhibitor due to LA. He was started with Prednisolone 0.5mg/kg and bleeding diathesis improved with no new bleeding. On follow up, lupus anticoagulant antibody is persistent but weakening with rising prothombin levels. He is currently well on tapering dose of prednisolone and is still on follow up.
Conclusions: HPS-LA is extremely rare; usually self-limiting and resolves spontaneously. However our patient had significant bleeding and required treatment intervention.

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