Long-term remission of T-cell large granular lymphocyte leukemia associated with rheumatoid arthritis after rituximab therapy.

نویسندگان

  • Divi Cornec
  • Valérie Devauchelle-Pensec
  • Sandrine Jousse-Joulin
  • Thierry Marhadour
  • Valérie Ugo
  • Christian Berthou
  • Nathalie Douet-Guilbert
  • Alain Saraux
چکیده

T-cell large granular lymphocyte leukemia (LGLL) is a rare clonal disease often associated with rheumatoid arthritis (RA) and manifests chiefly as neutropenia and recurrent infections. Immunosuppressive agents are the mainstay of treatment, but long-term remissions are rare. We report 2 cases of LGLL in patients with RA successfully treated with rituximab, a monoclonal antibody specific of B cells and approved for treating RA. The first patient experienced a complete LGLL remission that was sustained during the 8-year follow-up after the first rituximab infusion. In the second patient, rituximab therapy was followed by immediate neutropenia recovery and then by marked shrinkage of the LGLL clone 1 year later. The paradoxical efficacy of this specific anti-B-cell drug on a monoclonal T-cell disease suggests that some cases of LGLL may be reactive manifestations of chronic autoantigen stimulation rather than true malignancies.

برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید

ثبت نام

اگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید

منابع مشابه

Acquired pure red cell aplasia associated with lymphoproliferative disease of granular T lymphocytes.

Acquired pure red cell aplasia (PRCA) can be associated with lymphoproliferative disease of granular T lymphocytes (T-LDGL), also known as T-cell large granular lymphocyte leukemia. Fifteen adult patients with PRCA associated with T-LDGL comprise this study. Neutropenia and rheumatoid arthritis were uncommon. All patients responded to immunosuppressive therapy. The 2 most commonly used treatmen...

متن کامل

Large granular lymphocyte leukemia as a complication of rheumatoid arthritis.

Large granular lymphocyte leukemia is a rare entity belonging to same spectrum of diseases than Felty's syndrome, which might occur in patients with long-standing rheumatoid arthritis. It is clinically characterized by persistent neutropenia and recurrent bacterial infections associated with the presence in both peripheral blood and bone marrow of clonal expansion of atypic lymphocytes with a c...

متن کامل

BHS guidelines for the treatment of large granular lymphocyte and chronic prolymphocytic leukaemias

Large granular lymphocyte and prolymphocytic leukaemias are rare chronic lymphoproliferative disorders. Large granular lymphocyte leukaemias consist of indolent disorders such as T-cell large granular lymphocyte and chronic lymphoproliferative disorder of natural killer cells and the very rare but aggressive natural killer cell leukaemia. Treatment of the indolent large granular lymphocyte leuk...

متن کامل

Large Granular Lymphocyte Leukemia as a Complication of Rheumatoid Arthritis

Large granular lymphocyte leukemia is a rare entity belonging to same spectrum of diseases than Felty’s syndrome, which might occur in patients with long-standing rheumatoid arthritis. It is clinically characterized by persistent neutropenia and recurrent bacterial infections associated with the presence in both peripheral blood and bone marrow of clonal expansion of atypic lymphocytes with a c...

متن کامل

Emerging biologic therapies in rheumatoid arthritis: cell targets and cytokines.

PURPOSE OF REVIEW Biologic therapy for rheumatoid arthritis targets specific molecules, both cell-bound and soluble, that mediate and sustain the clinical manifestations of this complex disease. The aim of all the therapeutic strategies is to achieve complete and sustained suppression of inflammation, in the absence of unacceptable short-term and long-term toxicity. Despite the success of the c...

متن کامل

ذخیره در منابع من


  با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید

برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید

ثبت نام

اگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید

عنوان ژورنال:
  • Blood

دوره 122 9  شماره 

صفحات  -

تاریخ انتشار 2013