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Plasma exchange in the hyperviscosity syndrome due to immunoglobulins

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GRAPHICS

INTRODUCTION

The hyperviscosity syndrome results from high levels of proteins capable of increasing the serum viscosity. Classically, the syndrome is a result of increased amounts of IgM, as seen in Waldenstrom's macroglobulinemia; however, hyperviscosity has also been described in certain cases of multiple myeloma in which abnormal polymers of IgA, IgG, or kappa light chains are produced [1-4].

Hyperviscosity can lead to impairment in the microcirculation of the central nervous system, possibly resulting in headache, dizziness, vertigo, nystagmus, hearing loss, visual impairment, somnolence, coma and seizures. Other possible findings include mucosal hemorrhage due to reduced platelet function, heart failure, which has been attributed to an expanded plasma volume (see below), renal failure, and sausage-like beading in the retinal veins (picture 1).

The diagnosis is established by measuring serum viscosity with an Ostwald viscosimeter. Normal serum viscosity is between 1.4 and 1.8 (measured as the flow time through the viscometer of the patient's serum divided by that of water or saline). Patients with values between 2 and 4 are only rarely symptomatic, while symptoms occur in most patients with values between 5 and 8 [1]. Values above 10 are invariably associated with symptoms. Corresponding values of IgM are commonly between 4 and 8 g/dL but the correlation with viscosity values is not linear [5]. Total serum protein levels usually exceed 10 g/dL. (See "Recognition of monoclonal proteins", section on 'Serum viscosity' and "Epidemiology, pathogenesis, clinical manifestations and diagnosis of Waldenstrom macroglobulinemia", section on 'Overview' and "Epidemiology, pathogenesis, clinical manifestations and diagnosis of Waldenstrom macroglobulinemia", section on 'Hyperviscosity syndrome'.)

ROLE OF PLASMA EXCHANGE

Patients presenting with severe neurologic impairment, such as stupor or coma, should be treated with plasma exchange (plasmapheresis) on an emergency basis [1,5-8]. A reasonable initial prescription would be a one plasma volume exchange (see below), replaced with albumin, repeated daily until symptoms subside or until serum viscosity is normal. (See "Prescription and technique of therapeutic plasma exchange".)

  • Five to 20 liters of exchanged plasma may be required for IgM related disease [5].
  • IgA or IgG related syndromes may require a greater volume and a more repetitive treatment schedule, since a larger percentage of these smaller immunoglobulins are extravascular. As a result, lowering of serum values will be followed by extravascular to intravascular redistribution [9-11].
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References Top
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  2. Carter, PW, Cohen, HJ, Crawford, J. Hyperviscosity syndrome in association with kappa light chain myeloma. Am J Med 1989; 86:591.
  3. Bachrach, HJ, Myers, JB, Bartholomew, WR. A unique case of kappa light chain disease associated with cryoglobulinemia, pyroglobulinemia and hyperviscosity syndrome. Am J Med 1989; 86:596.
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  9. Barth, WF, Wochner, D, Waldmann, TA, Fahey, JL. Metabolism of human gamma macroglobulins. J Clin Invest 1964; 43:1036.
  10. Cohen, S, Freeman, T. Metabolic heterogeneity of human gamma globulin. Biochem J 1960; 76:475.
  11. Kaplan, AA. Towards a rational prescription of plasma exchange: The kinetics of immunoglobulin removal. Semin Dial 1992; 5:227.
  12. Valbonesi, M, Mosconi, L, Montani, F, et al. Cascade filtration: Clinical application in 26 patients with immune complex and IgM mediated diseases. Int J Art 1983; 6:303.
  13. Alexanian, R. Blood volume in monoclonal gammopathy. Blood 1977; 49:301.
  14. Kaplan, AA, Halley, SE. Plasma exchange with a rotating filter. Kidney Int 1990; 38:160.
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