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Journal of Clinical Oncology, Vol 26, No 4 (February 1), 2008: pp. 674-675 © 2008 American Society of Clinical Oncology. DOI: 10.1200/JCO.2007.14.3560
Extreme HyperlymphocytosisDepartment of Hematology/Oncology, Brown University; and Providence Veterans Administration Medical Center, Providence, RI
Department of Hematology/Oncology, Providence Veterans Administration Medical Center; and Department of Medicine, Brown University, Providence, RI
A 73-year-old asymptomatic man was diagnosed with the small-cell variant of T-cell prolymphocytic leukemia (T-PLL; WBC, 12,000/µL; 61% lymphocytes); he remained stable during 15 months. Flow showed CD4+ lymphocytes phenotyped as CD2+CD3+CD7+CD5+CD29+CD45RO-HLADR-CD25+, CD11b–CD57–. The smear subsequently favored classic T-PLL. Six months later he was diagnosed with a stroke syndrome, which resolved. His WBC was 182,000/µL with 82% prolymphocytes (Fig 1, x10 peripheral smear when WBC was
Hyperleukocytosis is defined as a WBC count in excess of 100,000 cells/µL.1 Extremes of high WBCs can result in microcirculatory compromise. The pathophysiologic basis is a combination of whole-blood hyperviscosity and leukostasis, with the latter being a function of interleukocyte and leukocyte–endothelial cell interaction. This has been described in myeloid and, less frequently, lymphoid leukemias.2 Despite the fact that pheresis has a well-defined role in certain diseases, in the setting of hyperleukocytosis it has been implemented mainly in myeloid leukemias.3-7 The role of lymphapheresis for hyperlymphocytosis has not been clearly defined in the literature. Successful treatment with lymphapheresis, resulting in symptomatic improvement, have been described8,9 predominantly in chronic lymphocytic leukemias (CLLs). A threshold has not been clarified, and in the reported scenarios, the decision to perform lymphapheresis has been guided by symptoms suggesting a leukostatic syndrome. The incidence of hyperviscosity and/or leukostasis syndromes in lymphocytic leukemias is rare. The cells in B- and T-CLLs are small and mostly well differentiated. Hence, to reach a leukocrit of 20%, where, in vitro, whole-blood viscosity increases, the cell count has to reach 1,000,000 cells/µL. The cell counts in CLLs are rarely above 500,000 cells/µL. In addition, the leukocrit is inversely correlated with the hematocrit. Previous observations noted a 1.5% decrease in the erythrocrit for every 1% increase of the leukocrit.10-12 In this way, the developing anemia improves the whole-blood hyperviscosity, and leukostatic phenomena are rarely observed until the total whole bloodcrit value (hematocrit plus leukocrit) exceeds 60%.13 We present a case of T-PLL, a rare disease, which represents approximately 3% of all T-cell lymphoproliferative disorders. It is distinct from chronic B-cell lymphocytic leukemias. T-PLL frequently demonstrates an aggressive course, resistance to conventional therapeutic measures, and has a median survival of 7.5 months. Although WBC counts are classically higher than those seen in B-CLL, lymphocytosis in excess of 500,000 cells/µL has rarely been reported; only one of 39 patients in a recently published case series had a lymphocyte count of that magnitude.13 Our case is noteworthy for several reasons. First, after review of the literature, it appears to represent the highest recorded WBC in T-PLL (1,052,000 cells/µL). Second, the plasma viscosity value was within normal limits, confirming that the actual mechanism of the microvascular compromise is related to whole blood viscosity and a cell-mediated phenomenon (cell-cell and cell–endothelial cell interaction). The fact that the patient became severely symptomatic after a rapid increase of his WBC to 400,000/µL, then much less so after leukapheresis to a WBC of 700,000/µL, supports the view that the clinical syndrome we observed was not strictly a leukocrit-induced occurrence. The correlation of the rapid WBC increase with a fulminant clinical presentation at these lower WBC counts suggests that the velocity of the WBC increase is of additional pathophysiologic relevance. AUTHORS’ DISCLOSURES OF POTENTIAL CONFLICTS OF INTEREST The author(s) indicated no potential conflicts of interest.
ACKNOWLEDGMENTS Presented at the Annual College of Physicians Annual Meeting, Rhode Island Chapter, May 10, 2006. REFERENCES 1. Porcu P, Cripe L, Ng EW, et al: Hyperleukocytic leukaemia and leukostasis: A review of pathophysiology, clinical presentation and management. Leuk Lymphoma 39:1-18, 2000[Medline] 2. McKee LC Jr, Colins RD: Intravascular leukocyte thrombi and aggregates as a cause of morbidity and mortality in leukaemia. Medicine 53:463-478, 1974[CrossRef][Medline] 3. Bloom R, Taveira DaSilva AM, Bracey A: Reversible respiratory failure due to intravascular leukostasis in chronic myelogenous leukemia: Relationship of oxygen transfer to leukocyte count. Am J Med 67:679-683, 1979[CrossRef][Medline] 4. Eisenstaedt RS, Beckman EM: Rapid cytoreduction in acute leukaemia: Management of cerebral leukostasis by cell pheresis. Transfusion 18:113-115, 1978[CrossRef][Medline] 5. Karp DD, Beck JR, Cornell CJ Jr: Chronic granulocyte leukemia with respiratory distress: Efficacy of emergency leukapheresis. Arch Intern Med 141:1353-1354, 1981 6. Lane TA: Continuous-flow leukapheresis for rapid cytoreduction in leukemia. Transfusion 20:455-457, 1980[CrossRef][Medline] 7. Mehta AB, Goldman JM, Kohner E: Hyperleucocytic retinopathy in chronic granulocytic leukaemia: The role of intensive leucapheresis. Br J Haematol 56:661-667, 1984[Medline] 8. Strobel E, Howe J, Backer U, et al: Therapeutic lymphapheresis in leukostasis-induced priapism [in German]. Dtsch Med Wochenschr 112:1984-1985, 1987[Medline] 9. Steinberg MH, Charm SE: Effect of high concentrations of leukocytes on whole blood viscosity. Blood 38:299-301, 1971 10. Lichtman MA: Rheology of leukocytes, leukocyte suspensions, and blood in leukemia: Possible relationship to clinical manifestations. J Clin Invest 52:350-358, 1973[Medline] 11. Lichtman MA, Rowe JM: Hyperleukocytic leukemias: Rheological, clinical, and therapeutic considerations. Blood 60:279-283, 1982 12. Dearden C, Matutes E, Cazin B, et al: High remission rate in T-cell prolymphocytic leukemia with CAMPATH-1H. Blood 98:1721-1726, 2001 13. Bartlett NL, Longo DL: T-small lymphocyte disorders. Semin Hematol 36:164-170, 1999[Medline]
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Copyright © 2008 by the American Society of Clinical Oncology, Online ISSN: 1527-7755. Print ISSN: 0732-183X
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200,000/µL) and quickly increased to 266,000/µL (hemoglobin normal, platelets 87,000/µL). His spleen was newly enlarged. A bone marrow clot section (
