SUCCESSFUL MANAGEMENT OF REFRACTORY SEVERE
THROMBOCYTOPENIA ASSOCIATED WITH ANTIPHOSPHOLIPID
SYNDROME USING RITUXIMAB.
Soad Khalil Al Jaouni, MD, FRCPC King Adbulaziz University Hospital, College
of
Medicine, King Abdulaziz University, Jeddah, Kingdom of Saudi Arabia
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.
saljaouni@kau.edu.sa
Keywords: Antiphospholipid syndrome, Rituximab
* Accepted in Blood November 16, 2008.