A Case Report on Refractory Moschcowitz Syndrome

Although completely identified later on, Thrombotic thrombocytopenic purpura was first described by Dr. Moschcowitz in 1924, therefore the name 'Moschcowitz syndrome'. It is a microangiopathy associated with thrombocytopenia and hemolysis which causes organ dysfunction secondary to widespread microvascular thrombosis. The spectrum of the organ dysfunction in thrombotic thrombocytopenic purpura is quite diverse and not limited to nervous system and kidneys as classically described thus making the diagnosis more difficult. Use of plasmapheresis has led to improvement in mortality rates associated with this disease. However, multiple organ failure and presence of shock indicates likelihood of severe disease activity or refractoriness which can be avoided with earlier initiation of immunosuppressive therapy such as Rituximab.