We present a case of granular lymphocytic leukemia (GLL) associated with pure red cell aplasia (PRCA). Twenty-seven percent of the peripheral blood mononuclear cells (PBMC) were positive for TCRδ1 but not for δTCS1 (Vδ2), and a rearrangement of the TCR chain (Jδ1) gene was detected. The PBMC and serum from the patient slightly suppressed CFU-E but not BFU-E colony formation in vitro. These findings suggest that monoclonal proliferation of TCRγδ-positive granular lymphocytes (GL) can be associated with PRCA even in a patient having a small proportion of leukemia GL, which we propose to designate as ‘smoldering GLL’.

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