Case Report
Report of 6 Cases of Large Granular Lymphocytic Leukemia and Plasma Cell Dyscrasia

https://doi.org/10.1016/j.clml.2014.04.001Get rights and content

Introduction

Large granular lymphocytic leukemia (LGLL) is a rare lymphocytic malignancy that was discovered in 1985.1, 2 There are 2 types of LGLL: the type with T-cell large granular lymphocytes (LGLs), called T-LGLL (85%), and the type with natural killer–cell LGLs, called NK-LGLL (15%). Clinical features of LGLL include cytopenias and rheumatoid arthritis. The majority of patients have an indolent clinical course.

Chronic B-cell dyscrasia is an important clinical feature of LGLL, including monoclonal gammopathy of undetermined significance (MGUS), chronic lymphocytic leukemia, and B-cell lymphoma.3 One study reported 15 patients with LGLL and coexisting hairy cell leukemia.4 In a French registry, 12 of 229 patients with LGLL had associated B-cell lymphoid neoplasms.5 Moreover, monoclonal B-cell lymphocytosis has been found to be frequently associated with LGLL.6

This report describes 6 patients with coexistent LGLL and multiple myeloma (MM). The concomitant presence of the 2 disorders presented the opportunity to retrospectively observe the effect of bortezomib and lenalidomide, 2 novel agents used for the treatment of MM, on the LGLL clone.

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Patients and Methods

The authors screened a clinical database of 629 patients with LGLL followed up at Penn State Hershey Cancer Institute and 229 patients with LGLL at the French LGL Registry between January 2007 and December 2011. This search identified 6 patients with concomitant LGLL and MM, 5 from the Penn State Hershey Cancer Institute Registry (cases 1 to 5) and 1 from the French LGL Registry (case 6). The study was approved by the institutional review boards of both institutes. The authors retrospectively

Results

The basic clinical characteristics of the 6 patients are summarized in Table 1. The median follow-up of the 6 patients with LGLL and MM was 76 months (range, 30-121). A brief history of each case is given in subsequent sections.

Discussion

This study identified 6 patients with both LGLL and MM from a combined registry of 858 patients with LGLL. The previous medical literature contains 6 similar cases.7 Because of the very rare incidence of these 2 hematologic disorders, the present authors suspect that the coexistence of LGLL and MM in the present 6 patients is not just coincidental.

The simultaneous occurrence of LGLL and MM in a patient offered the opportunity to report for the first time the effect of novel anti-MM drugs on the

Conclusion

In light of the very rare incidence of both LGLL and MM, the present cases suggest that there is an association between these 2 hematologic malignancies. However, the exact pathophysiologic mechanism awaits exploration, and the common triggers need to be elucidated.

Several studies have found that clonal T-cell expansions develop in association with monoclonal gammopathies,15 and the present authors believe that the association between LGLL and MM may be greater than is suspected. In fact, the

Disclosure

The authors have stated that they have no conflicts of interest.

References (15)

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