A Case Report of Combined Type 2 Autoimmune Hepatitis and Antiphospholipid Syndrome Presenting With Intracranial Hemorrhage: Diagnostic and Therapeutic Dilemma.

backgroundAutoimmune hepatitis (AIH) is very frequently associated with many autoimmune diseases. Antiphospholipid syndrome (APS) is frequently associated with type 1 AIH but rarely seen in type 2 AIH patients. In this case, the association between type 2 AIH and APS is seen, along with complications, which made the management difficult. CASE PRESENTATION: This patient, who had a history of recurrent abortions, presented with complaints of recurrent epistaxis with severe thrombocytopenia. She was diagnosed with APS with concomitant type 2 AIH. After failing with conservative treatment, partial splenic artery embolization (PSAE) for recurrent thrombocytopenia was done, which led to improvement in platelet count and the resolution of recurrent epistaxis. But after a few days, the patient developed an intracranial hemorrhage (ICH) with a normal platelet count and coagulation profile. A platelet function study was done, and platelet dysfunction was found. Finally, after a prolonged period of admission, the patient developed sepsis and disseminated intravascular coagulation (DIC) and succumbed to death.

conclusionFrom this case, it may be kept in mind the possibility of the association of both types of AIH when dealing with a patient with APS. Unconventional methods such as partial splenic artery embolization can be useful in patients with recurrent thrombocytopenia. A platelet function study is a very important parameter to look for whenever a patient has bleeding with a normal coagulation profile and platelet count with isolated raised activated partial thromboplastin time (aPTT).