T cell large granular lymphocytic leukemia in association with Sjögren's syndrome.
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.
Franco
Giovanni
G
Division of Hematology with BMT, Department of Oncology, University of Palermo, Palermo, Italy. giovifranco@gmail.com
Palazzolo
Roberto
R
Liardo
Eliana
E
Tripodo
Claudio
C
Mancuso
Salvatrice
S
D000368:Aged; D000740:Anemia; D001327:Autoimmune Diseases; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D054066:Leukemia, Large Granular Lymphocytic / Q000150:complications* / Q000175:diagnosis; D009503:Neutropenia; D012859:Sjogren's Syndrome / Q000150:complications* / Q000175:diagnosis
D007166:Immunosuppressive Agents