Serum and urine beta‐‐‐2‐microglobulin in hemophagocytic syndrome

Abstract

Background. Previous reports suggesting a correlation between lymphoproliferative disease and serum levels of beta‐2‐microglobulin (beta‐2M) and in vitro data indicating a role of cytokines in the production of beta‐2M prompted a study of serum and urine beta‐2M concentration in patients with hemophagocytic syndrome (HPS), because no data were previously available for HPS, a pathologic state associated with excessive cytokines secreted from activated reactive/malignant lymphocytes and histiocytes. Methods. Serum and urine beta‐2M levels were measured in six children with HPS during active and convalescent phase and in other diseases. Results. Serum and urine beta‐2M levels during active phase HPS were significantly high not only in serum (median, 7.5 mg/l; range, 2.3‐16.0 mg/l; P < 0.01), but also in urine (median, >31,650 m̈g/gram Creatinine (gCr); range, 8179‐236,333 m̈g/gCr; P < 0.01), compared with levels during convalescent phase HPS (median, 2.0 mg/l; range, 0.9‐2.5 mg/l in serum and median, 338 m̈g/gCr; range, 223‐585 m̈g/gCr in urine) and in control subjects with diseases such as acute lymphocytic leukemia (median, 2.3 mg/l; range, 1.0‐2.8 mg/l in serum and median, 327 m̈g/gCr; range, 48‐2684 m̈g/gCr in urine), infectious mononucleosis (median, 2.9 mg/l; range, 2.5‐5.5 mg/l in serum and median, 348 m̈g/gCr; range, 80‐1325 m̈g/gCr in urine), and Kawasaki disease (median, 2.8 mg/l; range, 1.5‐3.3 mg/l in serum and median, 1016 m̈g/gCr; range, 214‐4500 m̈g/gCr in urine). Noteworthy was the observation that urine beta‐2M levels correlated closely with HPS disease activity. Conclusions. Urine beta‐2M appears to be a useful marker for assessing disease activity in patients HPS. Cancer 1995;75:1700‐5. Copyright © 1995 American Cancer Society