Successful treatment of life-threatening Evans syndrome due to antiphospholipid antibody syndrome by rituximab-based regimen: a case with long-term follow-up

Abstract

An association of antiphospholipid antibody syndrome with antibodies directed against either phospholipids or plasma proteins strongly suggest that B-cell dysfunction may be involved in its pathogenesis. Antiphospholipid antibody syndrome with autoimmune cytopenias shows a poor response rate to conventional treatment with anticoagulants, glucocorticosteroids, immunosuppressive agents, intravenous immunoglobulin or plasmapheresis. We report a case of life-threatening antiphospholipid antibody syndrome with Evans syndrome receiving successful multimodal treatment including anti-CD20 monoclonal antibody rituximab with long-term follow-up.

Keywords

antiphospholipid antibody syndrome hemolytic anemia rituximab thrombocytopenia

Get full access to this article

View all access options for this article.

References

1 Erdozain JG Ruiz-Irastorza G Egurbide MV Aguirre C . Sustained responses to rituximab of autoimmune hemolytic anemia associated with antiphospholipid syndrome. Haematologica 2004; 89: ECR34 .

2 Veldhuis W Janssen M Kortlandt W van Houte A van de Ree M . Coombs-negative severe haemolytic in an immunocompetent adult following cytomegalovirus infection. Eur J Clin Microbiol Infect Dis 2004; 23: 844 – 847 .

3 Kerr R Rawlinson PSM Cachia PG . Direct antiglobulin test negative, non spherocytic autoimmune haemolytic anaemia. Clin Lab Haem 2000; 22: 365 – 367 .

4 Cervera R Piette J-C Font J Khamashta MA Shoenfeld Y . Antiphospholipid syndrome. Clinical and immunologic manifestations and patterns of disease expression in a cohort of 1000 patients. Arthritis Rheum 2002; 46: 1019 – 1027 .

5 Lang B Straub RH Weber S . Elevated anticardiolipin antibodies in autoimmune haemolytic anemia irrespective of underlying systemic lupus erythematosus. Lupus 1997; 6: 652 – 655 .

6 Lockshin MD Erkan D . Treatment of the antiphospholipid syndrome. N Engl J Med 2003; 349: 1177 .

7 Statkut L Traynor A Oyama Y . Antiphospholipid syndrome in patients with systemic lupus erythematosus treated by autologous hematopoietic stem cell transplantation. Blood 2005; 106: 2700 – 2709 .

8 Youinou P Renaudineau Y . The antiphospholipid syndrome as a model for B cell induced autoimmune diseases. Thromb Res 2004; 114: 363 – 369 .

9 Reff ME Carner K Chambers KS . Depletion of B cells in vivo by chimeric mouse human monoclonal antibody to CD 20. Blood 1994; 83: 435 – 445 .

10.

10 Asherson RA Cervera R de Groot PG . Catastrophic antiphospholipid syndrome: international censensus statement on classification criteria and treatment guidelines. Lupus 2003; 12: 530 – 534 .

11.

11 Mantadakis E Danilatou V Stiakaki E Kalmanti M . Rituximab for refractory Evans syndrome and other immune-mediated hematologic diseases. Am J Hematol 2004; 77: 303 – 310 .

12.

12 Josdottir T Gunnarsson I Risselada A . Treatment of refractory SLE with rituximab plus cyclophosphamide: clinical effects, serological changes, and predictors of response. Ann Rheum Dis 2008; 67: 330–334.

13.

13 Asherson R . Multiorgan failure and antiphospholipid antibody: the catastrophic antiphospholipid (Ashersons’s) syndrome. Immunobiology 2005; 210: 727 – 733 .

14.

14 Rubenstein E Arkfeld DG Metyas S . Rituximab treatment for resistant antiphospholipid syndrome. J Rheumatol 2006; 33: 355 – 357 .

15.

15 Trappe R Loew A Thuss-Patience P . Successful treatment of thrombocytopenia in primary antiphospholipid antibody syndrome with the anti-CD20 antibody rituximab-monitoring of antiphospholipid and anti-GP antibodies: a case report. Ann Hematol 2006; 85: 134 – 135 .