Successful Management of Refractory Severe Thrombocytopenia Associated with Antiphospholipid Syndrome Using rituximab

Background: Rituximab, a monoclonal antibody against the pan B-cell antigen CD20, that induces a rapid in vivo depletions of normal B lymphocytes. Although this agent was originally developed for use in patients with B-cell-NHL, its use has been successfully extended to many autoimmune disorders. Thrombocytopenia associated with antiphospholipid syndrome can be mild to severe. Severe cases can be associated with significant morbidity and mortality if refractory to the usual therapy for autoimmune thrombocytopenia.

Aim: To report an effective regimen in treating a case of severe bleeding thrombocytopenia associated with antiphospholipid syndrome.

Patient and Methods: A 49 year old female was diagnosed with thrombocytopenia associated with antiphospholipid syndrome and antibodies to double-stranded DNA, controlled over the last 15 years. Unfortunately, over the last two years the patient has had frequent admissions for uncontrolled nose bleed, soft palate petechiae and generalized bruising.

Many treatments have been tried for this case of difficult refractory bleeding with platelets in the range 2.0–6.0 K/uL. These include systemic treatments such as corticosteroids, high pulse therapy of methylprednisolone, Imuran, i.v. immunoglobulin infusion and anti Rh-D intravenous therapy.

We report an adult female patient with severe refractory bleeding thrombocytopenia associated with antiphospholipid syndrome and successful treatment with Rituximab. Her platelets count have been maintained above 200.0K/uL over a 14 months period. Unfortunately, this patient has had side effects of steroid therapy.

In conclusion: Rituximab may be effective treatment on patients with refractory, severe thrombocytopenia associated with antiphospholipid syndrome and significant bleeding complications. Rituximab is a promising alternative option for the eradication of the autoantibodies and restoration of normal hemostasis while avoiding the use of high-dose steroid in refractory bleeding thrombocytopenia.