WALDENSTROMS MACROGLOBULINEMIA WITH AN IGM PARAPROTEIN THAT IS BOTH ACOLD AGGLUTININ AND A CRYOGLOBULIN AND HAS A SUPPRESSIVE EFFECT ON PROGENITOR-CELL GROWTH
M. Zagonovaretti et al., WALDENSTROMS MACROGLOBULINEMIA WITH AN IGM PARAPROTEIN THAT IS BOTH ACOLD AGGLUTININ AND A CRYOGLOBULIN AND HAS A SUPPRESSIVE EFFECT ON PROGENITOR-CELL GROWTH, Transfusion, 34(10), 1994, pp. 910-914
Background: A patient with Waldenstrom's macroglobulinemia was admitte
d to the hospital with fever, leg pain, and dyspnea. The patient had g
as gangrene of the left leg that required above-the-knee amputation. P
lasmapheresis was instituted to treat hyperviscosity. Study Design and
Methods: The patient's serum contained an IgM-kappa paraprotein, a cr
yoglobulin, and a cold agglutinln. The serum was studied. Results: The
patient's red cells typed as A(1), Rh-positive. The direct antiglobul
in test was negative. The serum contained a cold agglutinin with anti-
Pr cold agglutinin specificity (titer 4096). Maximal thermal range was
30 degrees C. Following dithiothreitol treatment, the cold agglutinin
activity disappeared. The serum IgM concentration in the tested sampl
e was 62.3 g per L. The cold agglutinin titer in the supernatant after
removal of the cryoglobulin was 256, and the IgM level was 0.31 g per
L. Redissolving the cryoglobulin in a equivalent volume of saline res
ulted in a cold agglutinin titer of 4096 and an IgM level of 68.4 g pe
r L. These results indicate that the cryoglobulin and the cold aggluti
nin are the same paraprotein. Serum protein electrophoresis using agar
ose gel and immunofixation of the serum revealed an IgM-kappa monoclon
al band. Progenitor cell assays were performed by adding the patient's
serum at final concentrations of 0, 1, 5 and 10 percent (vol/vol) to
patient's and normal donor's peripheral blood mononuclear cells. Inhib
ition of burst-forming units-erythroid and colony-forming units-granul
ocyte/macrophage by the patient's serum was demonstrated. Appropriate
controls and the use of the serum of another patient with Waldenstrom'
s macroglobulinemia did not suppress progenitor cell growth. The patie
nt's serum inhibited colony formation in a dose-response fashion. Conc
lusion: Reports of cryoprecipitable cold agglutinins are rare. This ca
se is unusual because the IgM-kappa paraprotein was also a cold agglut
inin with anti-Pr specificity and erythroid and granulocyte-macrophage
progenitor cell-suppressive properties.