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Vol. 124, No. 1, 2010
Issue release date: July 2010

T Cell Large Granular Lymphocytic Leukemia in Association with Sjögren’s Syndrome

Franco G. · Palazzolo R. · Liardo E. · Tripodo C. · Mancuso S.
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Abstract

T cell large granular lymphocytic (LGL) leukemia is a rare condition accounting for 2–3% of all mature lymphoid leukemias. Here, we present the case of a 73-year-old woman presenting with neutropenia and anemia (hemoglobin 9.9 g/dl). Hematological assessment revealed the presence of a T cell LGL leukemia. At the time of T cell LGL leukemia diagnosis, the patient developed xerophthalmia and xerostomia, and a diagnosis of Sjögren’s syndrome was made following salivary gland biopsy. The finding of large granular lymphocytes in the context of autoimmune disorders is well-known, though it often occurs with rheumatoid arthritis or in association with a positive autoantibody titer in the absence of an overt clinical picture. The concomitant presentation of T cell LGL leukemia with Sjögren’s syndrome is a rare event which is worth reporting. Our patient was managed with immunosuppressive therapy and is still alive.



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References

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    External Resources

  7. Loughran TP Jr: Clonal diseases of large granular lymphocytes. Blood 1993;82:1–14.
  8. Viny AD, Lichtin A, Pohlman B, Loughran T, Maciejewski J: Chronic B-cell dyscrasias are an important clinical feature of T-LGL leukemia. Leuk Lymphoma 2008;49:932–938.
  9. Ergas D, Tsimanis A, Shtalrid M, Duskin C, Berrebi A: T-gamma large granular lymphocyte leukemia associated with amegakaryocytic thrombocytopenic purpura, Sjögren’s syndrome, and polyglandular autoimmune syndrome type II, with subsequent development of pure red cell aplasia. Am J Hematol 2002;69:132–134.
  10. Saitoh T, Matsushima T, Kaneko Y, Yokohama A, Handa H, Tsukamoto N, Karasawa M, Nojima Y, Murukami H: T cell large granular lymphocyte (LGL) leukemia associated with Behcet’s disease: high expression of sFasL and IL-18 of CD8 LGL. Ann Hematol 2008;87:585–586.
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