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.