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CASE REVIEW
Prolonged survival of these cells is believed to result from a dysregulation of apoptosis. Course is generally indolent. Suppressive therapy is generally successful, but no cure has been described. The most common life-threatening complication is infection secondary to neutropenia.4
Presentation generally involves neutropenia and/or anemia. Up to 50 percent of LGL leukemia patients may present with splenomegaly, and up to 36 percent may have rheumatoid arthritis.4 For this reason, it is important to differentiate this disease from Felty’s syndrome, which is characterized by neutropenia, splenomegaly, and rheumatoid arthritis, but not generally by any hematological neoplastic process, although there may be some overlap in pathophysiology.5
Diagnosis of LGL leukemia is made by increased numbers of clonal large granular lymphocytes in an appropriate clinical context. Clonal gene rearrangement differentiates from reactive LGL proliferation. The morphology of these cells is characterized by size of 15-18um, abundant cytoplasm, azurophilic granules, and reniform or round nuclei. Granules contain perforin and cells. Immunohistochemistry may support the diagnosis, with a majority of cells being CD3+/CD8+/CD57+.4 Phenotypically, LGL cells are terminal effector memory T cells (CD45RA+CD62L-).6
If LGL leukemia becomes complicated by symptomatic anemia, neutropenia, or rheumatoid arthritis symptoms, initial therapy may consist of oral methotrexate or cyclophosphamide.7 If one therapy is unsuccessful, the other is attempted. If both fail, cyclosporine may be started. Evidence-based data on an ideal therapeutic regimen is scarce.4
In the above cases, both patients exhibited the T-cell phenotype of LGL leukemia. Both had a work-up that was guided by the point witnessed during the course, of each patient’s disease was remarkably different. SM experienced life-threatening suppression of hematopoiesis due to constitutively activated TD did not suffer even a mild decrease in blood counts, and
CONCLUSION
Because of its highly variable presentation, it is important to evaluate each potential case of LGL leukemia in its own right, approach.
CONTRIBUTING AUTHORS
■ SHAUN HANSON, MD is an Internal Medicine Resident at Christiana Care Health System in Newark, Del.
■ MICHAEL GUARINO, MD is an Oncologist and a partner in Medical Oncology Hematology Consultants, PA, located at Christiana Care’s Helen F. Graham Cancer Center in Newark, Del.
■ PAMELA SIMPSON, MD is an Oncologist who practices at Regional Hematology and Oncology in Newark, Del.
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