CONCLUSIONS.
LAHPS is an uncommon pathology associated with hemorrhagic complications, more common in pediatric age and in young women, with more than half of the patients being under sixteen years of age at diagnosis1. Most cases are associated with a diagnosis of SLE or occur secondary to viral infections, the latter especially in pediatric patients, who usually present spontaneous resolution of the picture more often than in cases diagnosed in adulthood2. The management, as it has been approached in this case, consists of steroids at high doses of mg/kg/day at the diagnosis of the disease, although there are no established parameters for the treatment of this pathology given the small number of cases described1.
The use of immunosuppressants is important for the progressive withdrawal of steroid therapy. Cyclophosphamide is one of the therapeutic options that have been used, either in combination with steroids or in monotherapy with generally favourable results6,7. Azathioprine has also been tried in this indication, always in combination with corticosteroids and with variable responses8,9. Non-specific intravenous immunoglobulins have been used as an alternative treatment in these patients, with variable responses and always in combination or after previous treatment with steroids10. Given the frequent coexistence of LAHPS and SLE in the same patient, in some cases these patients have been treated with hydroxychloroquine, with favorable results in combination with immunoglobulins11. Rituximab has been used in some patients, in combination with steroid therapy, especially in pediatric patients, with good response12,13. In our case it was not posible to use rituximab or azathioprine given the patient‘s history of severe adverse drug reactions to these drugs.
Combined treatment in our patient with cyclosporine A, mycophenolate mofetil and corticosteroids has so far allowed to maintain FII levels in the haemostatic range and to reintroduce antiplatelet therapy with ASA without documenting new haemorrhagic complications to date.