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IgA and IgG hypogammaglobulinemia in Waldenström’s macroglobulinemia
11/10/2009
By ZR Hunter, R Manning, C Hanzis, B Ciccarelli, L Ioakimidis, C Patterson, M Lewicki, H Tseng, P Gong, X Liu, Y Zhou, G Yang, J Sun, L Xu, P Sheehy, M Morra, SP Treon
Publisher: Haematologica
Background
Hypogammaglobulinemia is common in Waldenström’s macroglobulinemia (WM), and has been attributed to disease-related suppression. The etiology for this finding remains unclear, and has been speculated to be on the basis of tumor-induced suppression.
Design and Methods
We evaluated the incidence of IgA and IgG hypogammaglobulinemia in 207 untreated WM patients and addressed the associated clinicopathological findings, and impact of therapy. We also sequenced 8 genes (AICDA; BTK; CD40; CD154; NEMO, TACI, SH2D1A, UNG) implicated in immunoglobulin deficiency in 19 WM patients with IgA and/or IgG hypogammaglobulinemia.
Results
At baseline 63.3%, 58.0% and 49.3% of the 207 patients had abnormally low serum levels of IgA, IgG, or both. No association between IgA and IgG hypogammaglobulinemia and disease burden, serum IgM levels, B2M, IPSS score, or incidence of recurrent infections was observed, though presence of adenopathy and/or splenomegaly was associated with a lower incidence of hypogammaglobulinemia. Lower IgA and IgG levels were associated with disease progression in watch and wait patients. IgA and/or IgG levels remained abnormally low despite response to treatment, including complete remissions. A missense mutation in the highly conserved catalytic site of UNG was observed in a patient with hypogammaglobulinemia warranting further study of this pathway in WM.
Conclusions
IgA and IgG hypogammaglobulinemia is common in WM which persists despite therapeutic intervention and response. IgA and IgG hypogammaglobulinemia do not predict for recurrent infection risk in WM patients, though lower levels of serum IgA and IgG are associated with disease progression in WM patients on watch and wait.
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