Refractory thrombocytopenia in antiphospholipid syndrome

Thrombocytopenia is the second most common manifestation of antiphospholipid syndrome (APS). It is found in approximately 22% of the patients with this disease. Often it is not severe, platelet counts usually range between 50 x 10(9)/L and 150 x 10(9)/L without bleeding problems. Yet, it does not protect patients against thrombotic events. It rarely requires treatment and, due to similarities to idiopathic thrombocytopenic purpura (ITP), similar treatment rules usually apply. In this report two patients with APS are described who presented with severe thrombocytopenia that did not respond to standard treatment regimen namely glucocorticoids (GC) followed by intravenous immunoglobulin therapy (IVIG). Splenectomy had to be resorted to relieve the condition.