CASE PRESENTATION
We present the case of a 54-year-old woman diagnosed with Systemic Lupus Erythematosus (SLE) in 1996 with systemic involvement: lupus glomerulonephropathy, cutaneous, serous and joint involvement. Prior to the diagnosis of LAHPS, the patient was serologically positive por ANA, anti-DNA, anti-SSA, anti-Scl70, LA, ACAs and anticardiolipin antibodies (aCL) and was under follow-up by rheumatology in our centre. She had no personal or family history of thrombotic or haemorrhagic events and was on prophylactic antiplatelet treatment with acetylsalicylic acid (ASA) 100mg daily given the triple positivity for antiphospholipid antiboides.
In August 2020, after four days of headache, the patient went to the hospital emergency department where an urgent cranial CT scan was performed showing a left cerebellar haematoma. Antiplatelet therapy was withdrawn at that time, and she progressed favourably without new haemorrhagic complications or secondary neurological sequelae. At that time, she was receiving immunosuppressive treatment with prednisone 5mg daily and cyclosporine 50mg every 12 hours due to her previous diagnosis of SLE. The patient had previously received multiple lines of immunosuppressive treatment as part of the treatment for her previous diagnosis of SLE and had a history of serious adverse events to immunosuppressants, including agranulocytosis secondary to treatment with azathioprine and severe infusional reaction secondary to the administration of rituximab.
The patient was referred to the thrombosis and haemostasis department for a study, which was carried out in October 2020 (Table 1).
On his first evaluation at our clinic, his platelet count was in the normal range, INR 1.44; prothrombin activity (PA) 61%; cephalin time (PTT) 83.1 sec; cephaline mixture test (PTT) 74.9 sec; antigenic fibrinogen 408 mg/dl and Clauss fibrinogen 360 mg/dl. In view of these findings, a study of extrinsic coagulation pathway factors (coagulometric method) was performed and a deficit in FII activity (28%) was observed. Persistent triple positivity for antiphospholipid antibodies was also observed. The study of factors of the intrinsic coagulation pathway with ellagic acid was within the normal range. The study of von Willebrands disease, filling times and platelet aggregations did not show abnormal results.
Given the suspicion of LAHPS, the dose of oral prednisona was incresed to 50mg (1mg/kg/day) daily in December 2020, after which FII activity normalised to levels of 68% (Figure 1) in January 2021. A tapering course of corticosteroids was started two weeks after the start of treatment, reaching a dose of 10mg of prednisone daily in March 2021, with a parallel progressive decrease in FII activity to levels of 34%. The patient continued to be monitored by the rheumatology department and in consensus with them, with the aim of starting a drecreasing course of corticosteroids until reaching her usual doses, the increased the daily dose of cyclosporine A and associated mycophenolate mofetil with a further increase in the levels of FII to 46% on 10/29/2021. Given the previous adverse effects presented by the patient, it was not posible to use other immunosuppressants commonly used in other cases of LAHPS, such as azathioprine or rituximab.
On 10/29/2021 antiplatelet therapy was reintroduced with ASA 100mg daily. The patient did not present haemorrhagic or thrombotic complications.