Abstract

Infections are a major cause of morbidity and mortality in patients with chronic lymphocytic leukemia (cll), who typically have increased susceptibility because of hypogammaglobulinemia (hgg) related to their disease and its treatment. Immunoglobulin replacement therapy (igrt) has been shown to reduce the frequency of bacterial infections and associated hospitalizations in patients with hgg or a history of infection, or both. However, use of igrt in cll is contentious. Studies examining such treatment were conducted largely before the use of newer chemoimmunotherapies, which can extend lifespan, but do not correct the hgg inherent to the disease. Thus, the utility of igrt has to be re-evaluated in the current setting. Here, we discuss the evidence for the use of igrt in cll and provide a practical approach to its use in the prevention and management of infections.

Highlights

  • Chronic lymphocytic leukemia is a hematopoietic neoplasia, marked by the proliferation and accumulation of small, mature-appearing, immunologically incompetent B lymphocytes in blood, bone marrow, lymph nodes, and spleen[1]

  • Primary immunodeficiency disorders marked by hgg are caused by inborn errors of B-cell development or maturation and are associated with increased susceptibility to the same types of infections associated with hgg in cll[27]

  • The beneficial effect of ivig was later demonstrated in 1988 in a randomized controlled double-blind clinical trial conducted by the Cooperative Group for the Study of Immunoglobulin in CLL39, whereby, compared with placebo, use of ivig was associated with fewer bacterial infections (p = 0.01, Table ii)

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Summary

BACKGROUND

Chronic lymphocytic leukemia (cll) is a hematopoietic neoplasia, marked by the proliferation and accumulation of small, mature-appearing, immunologically incompetent B lymphocytes in blood, bone marrow, lymph nodes, and spleen[1]. The spectrum of infections in patients with cll varies according to disease stage (early vs late) and treatment history, with a core susceptibility to bacterial respiratory tract infections, but potentially extending to other organisms as a result of cll-directed therapy. Primary immunodeficiency disorders (pids) marked by hgg are caused by inborn errors of B-cell development or maturation and are associated with increased susceptibility to the same types of infections associated with hgg in cll[27]. Given that hgg worsens with disease duration, clinical (infection history) and laboratory (Ig level) evaluations should be performed at least every 6–12 months, the timing should be tailored to each patient.

2–4 Weeks 2–4 Weeks
Results
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