A middle aged man with hx of DM2, BPH, multiple recent infections, who presented with Neutropenic fever with ANC of 0 and cellulitis of Left foot. After multiple hospitalizations was diagnosed with Large Granular Lymphocyte Leukemia (LGL Leukemia).
– Heme/Onc cases in general are often difficult due to our exposure and the rarity of cares.
– LGL Leukemia is rare, estimated to be 1 in 10 million in US
– Must differentiate this condition from reactive LGL expansions and chronic lymphomatous conditions, as well as chronic and aggressive NK cell leukemias.
– It is thought that autoimmunity plays a central role in its development
– Associated with infections (EBV, CMV) as well as RA, Schogren’s and other autoimmune conditions. 40% of cases are associated with RA or an autoimmune cytopenia (pure red cell aplasia, autoimmune hemolytic anemia, ITP.
– Diagnosis is clinical with undefined neutropenia, confirmed with bone marrow biopsy with increased number of LGLs (normal 200-400 micro/L).
– Treatment is primarily immune suppression with MTX, cyclophosphamide, Cyclosporine, +/- steroids. (this patient also received Neupogen with good response)
– Good prognosis, 85% survival at 4 years