Semin Thromb Hemost 2007; 33(4): 350-354
DOI: 10.1055/s-2007-976170
Copyright © 2007 by Thieme Medical Publishers, Inc., 333 Seventh Avenue, New York, NY 10001, USA.

Therapeutic Apheresis in Hyperleukocytosis and Hyperviscosity Syndrome

William Blum1 , Pierluigi Porcu1
  • 1Department of Internal Medicine, Division of Hematology/Oncology, The Ohio State University, Columbus, Ohio
Further Information

Publication History

Publication Date:
24 May 2007 (online)

ABSTRACT

Therapeutic apheresis (TA), as a way of rapidly eliminating harmful or excessive blood components such as plasma proteins (plasma exchange) or cells (leukapheresis and platelet apheresis), has found broad application in a vast array of hematologic disorders. The most common hematologic indications for TA are leukocytosis in acute leukemias and hyperviscosity syndrome secondary to plasma cell dyscrasia. Leukapheresis is indicated in the initial management of leukostasis in patients with hyperleukocytosis in acute leukemias, particularly myeloid leukemias, or in patients who are at high risk of developing such a complication. Patients with lymphoid malignancies rarely develop leukostasis but may undergo cytoreduction with leukapheresis as prophylaxis for tumor lysis. The use of leukapheresis in acute promyelocytic leukemia is discouraged, given the possibility of increased risk of coagulopathy and bleeding. Similarly, therapeutic plasma exchange (TPE) represents an effective but temporary way of managing hyperviscosity syndrome secondary to immunoglobulin M paraproteins in patients with Waldenström macroglobulinemia. This review provides an overview of the pathophysiology of leukostasis and its management with leukapheresis. The use of TPE in the management of hyperviscosity syndrome is also discussed.

REFERENCES

  • 1 Porcu P, Cripe L D, Ng E W et al.. Hyperleukocytic leukemias and leukostasis: a review of pathophysiology, clinical presentation and management.  Leuk Lymphoma. 2000;  39 1-18
  • 2 Vaughan W P, Kimball A W, Karp J E, Dragon L H, Burke P J. Factors affecting survival of patients with acute myelocytic leukemia presenting with high WBC counts.  Cancer Treat Rep. 1981;  65 1007-1013
  • 3 Hug V, Keating M, McCredie K, Hester J, Bodey G P, Freireich E J. Clinical course and response to treatment of patients with acute myelogenous leukemia presenting with a high leukocyte count.  Cancer. 1983;  52 773-779
  • 4 Dutcher J P, Schiffer C A, Wiernik P H. Hyperleukocytosis in adult acute nonlymphocytic leukemia: impact on remission rate and duration, and survival.  J Clin Oncol. 1987;  5 1364-1372
  • 5 Porcu P, Danielson C F, Orazi A, Heerema N A, Gabig T G, McCarthy L J. Therapeutic leukapheresis in hyperleucocytic leukaemias: lack of correlation between degree of cytoreduction and early mortality rate.  Br J Haematol. 1997;  98 433-436
  • 6 Creutzig U, Ritter J, Budde M, Sutor A, Schellong G. Early deaths due to hemorrhage and leukostasis in childhood acute myelogenous leukemia. Associations with hyperleukocytosis and acute monocytic leukemia.  Cancer. 1987;  60 3071-3079
  • 7 Bunin N J, Pui C H. Differing complications of hyperleukocytosis in children with acute lymphoblastic or acute nonlymphoblastic leukemia.  J Clin Oncol. 1985;  3 1590-1595
  • 8 Bunin N J, Kunkel K, Callihan T R. Cytoreductive procedures in the early management in cases of leukemia and hyperleukocytosis in children.  Med Pediatr Oncol. 1987;  15 232-235
  • 9 Cuttner J, Holland J F, Norton L, Ambinder E, Button G, Meyer R J. Therapeutic leukapheresis for hyperleukocytosis in acute myelocytic leukemia.  Med Pediatr Oncol. 1983;  11 76-78
  • 10 Ventura G J, Hester J P, Smith T L, Keating M J. Acute myeloblastic leukemia with hyperleukocytosis: risk factors for early mortality in induction.  Am J Hematol. 1988;  27 34-37
  • 11 Giles F J, Shen Y, Kantarjian H M et al.. Leukapheresis reduces early mortality in patients with acute myeloid leukemia with high white cell counts but does not improve long- term survival.  Leuk Lymphoma. 2001;  42 67-73
  • 12 Byrd J C, Mrozek K, Dodge R et al.. Pretreatment cytogenetic abnormalities are predictive of induction success, cumulative incidence of relapse, and overall survival in adult patients with de novo acute myeloid leukemia: results from Cancer and Leukemia Group B (CALGB 8461).  Blood. 2002;  100 4325-4336
  • 13 Frohling S, Schlenk R F, Breitruck J et al.. Prognostic significance of activating FLT3 mutations in younger adults (16 to 60 years) with acute myeloid leukemia and normal cytogenetics: a study of the AML Study Group Ulm.  Blood. 2002;  100 4372-4380
  • 14 Vahdat L, Maslak P, Miller Jr W H et al.. Early mortality and the retinoic acid syndrome in acute promyelocytic leukemia: impact of leukocytosis, low-dose chemotherapy, PMN/RAR-alpha isoform, and CD13 expression in patients treated with all-trans retinoic acid.  Blood. 1994;  84 3843-3849
  • 15 Wetzler M, Dodge R K, Mrozek K et al.. Prospective karyotype analysis in adult acute lymphoblastic leukemia: the cancer and leukemia Group B experience.  Blood. 1999;  93 3983-3993
  • 16 Hoelzer D, Thiel E, Loffler H et al.. Prognostic factors in a multicenter study for treatment of acute lymphoblastic leukemia in adults.  Blood. 1988;  71 123-131
  • 17 Estey E H, Keating M J, McCredie K B, Bodey G P, Freireich E J. Causes of initial remission induction failure in acute myelogenous leukemia.  Blood. 1982;  60 309-315
  • 18 Byrd J C, Mrozek K, Dodge R K et al.. Pretreatment cytogenetic abnormalities are predictive of induction success, cumulative incidence of relapse, and overall survival in adult patients with de novo acute myeloid leukemia: results from Cancer and Leukemia Group B (CALGB 8461).  Blood. 2002;  100 4325-4336
  • 19 Eguiguren J M, Schell M J, Crist W M, Kunkel K, Rivera G K. Complications and outcome in childhood acute lymphoblastic leukemia with hyperleukocytosis.  Blood. 1992;  79 871-875
  • 20 Smith M, Arthur D, Camitta B et al.. Uniform approach to risk classification and treatment assignment for children with acute lymphoblastic leukemia.  J Clin Oncol. 1996;  14 18-24
  • 21 Kuo K HM, Callum J, Brandwein J et al.. Management of hyperleukocytosis in acute myelogenous leukemia using hydroxyurea rather than leukopheresis.  Blood. 2006;  108 , (abst 2007)
  • 22 McKee Jr L C, Collins R D. Intravascular leukocyte thrombi and aggregates as a cause of morbidity and mortality in leukemia.  Medicine (Baltimore). 1974;  53 463-478
  • 23 Novotny J R, Nuckel H, Duhrsen U. Correlation between expression of CD56/NCAM and severe leukostasis in hyperleukocytic acute myelomonocytic leukaemia.  Eur J Haematol. 2006;  76 299-308
  • 24 Novotny J R, Muller-Beissenhirtz H, Herget-Rosenthal S, Kribben A, Duhrsen U. Grading of symptoms in hyperleukocytic leukaemia: a clinical model for the role of different blast types and promyelocytes in the development of leukostasis syndrome.  Eur J Haematol. 2005;  74 501-510
  • 25 Stucki A, Rivier A S, Gikic M, Monai N, Schapira M, Spertini O. Endothelial cell activation by myeloblasts: molecular mechanisms of leukostasis and leukemic cell dissemination.  Blood. 2001;  97 2121-2129
  • 26 Kwaan H C, Bongu A. The hyperviscosity syndromes.  Semin Thromb Hemost. 1999;  25 199-208
  • 27 Mehta J, Singhal S. Hyperviscosity syndrome in plasma cell dyscrasias.  Semin Thromb Hemost. 2003;  29 467-471
  • 28 Bachrach H J, Myers J B, Bartholomew W R. A unique case of kappa light-chain disease associated with cryoglobulinemia, pyroglobulinemia, and hyperviscosity syndrome.  Am J Med. 1989;  86 596-602
  • 29 Rosenson R S, McCormick A, Uretz E F. Distribution of blood viscosity values and biochemical correlates in healthy adults.  Clin Chem. 1996;  42 1189-1195
  • 30 Drew M J. Plasmapheresis in the dysproteinemias.  Ther Apher. 2002;  6 45-52
  • 31 Chirnside A, Urbaniak S J, Prowse C V, Keller A J. Coagulation abnormalities following intensive plasma exchange on the cell separator.  Br J Haematol. 1981;  48 627-634
  • 32 Dodd R Y, Notari 4th E P, Stramer S L. Current prevalence and incidence of infectious disease markers and estimated window-period risk in the American Red Cross blood donor population.  Transfusion. 2002;  42 975-979

William BlumM.D. 

The Ohio State University, B310 Starling Loving Hall

320 West 10th Ave., Columbus, OH 43210

Email: william.blum@osumc.edu

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