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Leukostasis in Sézary Syndrome

Shu-Dean Hsu, MD; James T. May, MD; Gary B. Weiss, MD, PhD; John J. Costanzi, MD; Richard E. Slavin, MD
JAMA. 1979;241(22):2378-2379. doi:10.1001/jama.1979.03290480012005.
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To the Editor.—  The occurrence of intravascular leukocyte thrombi and aggregates is well documented in both acute and chronic myelogenous leukemia, especially when the total WBC count is more than 50,000/cu mm. Leukostasis is an infrequent complication of lymphocytic leukemias, even with WBC counts of more than 200,000/cu mm.1 We had a patient with Sézary syndrome in whom leukostasis was believed to be the cause of death.

Report of a Case.—  A 53-year-old man was first seen in July 1974 with a two-year history of diffuse dry, scaling, pruritic skin lesions. The leukocyte count was 30,000/cu mm, with 65% lymphocytes. One half of the lymphocytes were distinctive, large lymphoid cells with convoluted nuclei and PAS-positive cytoplasmic granules, which were resistant to diastase decoloration (typical Sézary cells). Skin and lymph node biopsy specimens showed infiltration by similar cells. The bone marrow was spared. Combination chemotherapy was unsuccessful. The WBC count

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