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A 57-year-old man presents with fatigue and dyspnea on exertion. Initial laboratory workup shows hemoglobin 1 0.5 g/dl with an MCV of 85 fl. leukocyte count 11 .2 00/ .uL. and platelet count 1 67 ,000/ .uL. No lymphadenopathy or splenomegaly was noted on physical exam. Peripheral blood smear is shown below.
What best describes the usual clinical course ofT-LGL leukemia?
- Rapid progression with overall survival measured in months
- Spontaneous regression with recurrence in about one-half of patients
- Indolent course which often does not require immediate therapy
- Cure of the disease with successful treatment directed toward an underlying autoimmune condition
Medicine
C. The natural history ofT-LGL leukemia is one of indolence, with median survival of more than 10 years. Treatment is initiated when cytopenias become significant or associated autoimmune conditions prompt intervention. Spontaneous regression has rarely been described. Although treat-ment directed toward an underlying autoimmune condition is part of the approach to LGL, control rather than cure is the norm. Recent data suggest that aberrant STAT3 and STAT5b signaling underlie the pathogenesis of this disease. Perhaps treatment with STAT inhibitors may be of benefit (NEJM. 2012;366: 1 905-1913. Blood. 2012;120:3048-3057).
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