Large granular lymphocytic leukaemia
S Michalsen E Schrumpf K Beiske A Tierens V Stenberg G E Tjønnfjord
Background. Large granular lymphocytic leukaemia (LGL-leukaemia) is a rare disease. Knowledge of the disease may contribute to targeted and quick diagnosis and effective treatment of those affected.
Material and methods. We identified patients who had been diagnosed with LGL-leukaemia at Oslo University Hospital, Rikshospitalet in the period 01.10.2001–31.12.2007. Their medical records were assessed retrospectively.
Results. During the study period, LGL-leukaemia was diagnosed in 52 patients (26 women and 26 men), median age of 59 (26–86) years. The leukaemia displayed NK-cell phenotype in one patient and T-cell phenotype in the remaining 51 patients. Slightly more than one third of the patients were asymptomatic. Cytopenia, usually granulocytopenia, was the most common cause for the clinical phenotype in symptomatic patients. Concomitant disease with immune pathogenesis was common, and we also found a high prevalence of clonal B-cell disease (17 %).
Interpretation. Our patients with LGL-leukaemia are representative for presentations of the disease in the literature. The condition should be suspected when patients present with unexplained cytopenias. An early diagnosis may prevent an extensive and unnecessary diagnostic work-up and ensure that patients are offered a simple and effective treatment.