Abstract
Lupus anticoagulant-hypoprothrombinemia syndrome (LAHS) is a rare hemorrhagic disorder
that should be differentiated from classical antiphospholipid syndrome. Literature
review shows that LAHS may affect people at any age but approximately 40% are children
younger than 10 years. Autoimmune and infectious diseases are the most frequent triggering
causes, and the laboratory profile is characterized by a prolongation of prothrombin
time (PT) and activated partial thromboplastin time (aPTT) with a mild to severe reduction
in factor II levels. In more than half the patients, the other coagulation factors
are normal, while anti-cardiolipin and anti-β2-glycoprotein I antibodies show a high
titer. Lupus anticoagulant (LA) is positive in 100% of cases, as this represents a
defining feature. The majority of patients have mucocutaneous bleeding events (44%);
cerebral bleeding can occur in 10% of patients and other common bleeding sites are
the gastrointestinal and genitourinary tracts. There is no standard treatment for
LAHS. Supportive measures, such as fresh frozen plasma, packed red blood, and platelet
transfusion, are frequently administered in association with steroids alone or in
combination with intravenous immunoglobulin or cyclophosphamide, azathioprine, and
rituximab. Death, recurrent bleeding, and thrombosis can occur in approximately 3,
13, and 14% of patients, respectively. Our patient was an old man with a myocardial
infarction and a systemic infection from Candida parapsilosis. Thrombin generation and clot waveform analysis were performed before and after treatment.
Thrombin generation better reflected the role of prothrombin, revealing that a factor
II value of below around 15% can represent a risk for major bleeding. Treatment with
methylprednisolone and three-factor human prothrombin complex concentrate allowed
the patient to reach a complete recovery 1 month after initial diagnosis.
Keywords
LA-hypoprothrombinemia syndrome - bleeding management - thrombin generation - clot
waveform analysis
