The case of a 33 year old woman with a large granular lymphocytic leuk
emia is presented. The main symptoms were neutropenia and recurrent re
spiratory bacterial infections. No enlargement of the liver, spleen or
lymph nodes was noted. Circulating lymphocytes averaged 3000/mu l wit
h 35% of large granular cells. The bone marrow biopsy showed lymphatic
infiltration with both nodular and interstitial pattern. Lymphocytes
bore the T suppressor phenotype (CD8 +, CD45 RO +, CD20 -, kappa -, la
mbda -). Cytogenetic studies revealed a low expression clone with 7q -
: del (7) (q36). Gene rearrangements for immunoglobulins or T - cell r
eceptors could not be demonstrated by Southern Blot. Bone marrow cultu
res grew normally while both normal and patient bone marrow showed mar
ked inhibition when incubated with patients serum. Normalization of th
e peripheral granulocytic count was obtained with prednisone, while gr
anulocytic-stimulating factors, chlorambucil, and cyclosporine A were
partially active or inactive. We suggest that this case represents a f
orm of the lymphoproliferative disease of granular lymphocytes. To our
knowledge, the deletion of the long arm of chromosome 7 has not been
described in this disease.