Serum and urine beta-2-microglobulin in hemophagocytic syndrome.
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 micrograms/gram Creatinine (gCr); range, 8179-236,333 micrograms/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 micrograms/gCr; range, 223-585 micrograms/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 micrograms/gCr; range, 48-2684 micrograms/gCr in urine), infectious mononucleosis (median, 2.9 mg/l; range, 2.5-5.5 mg/l in serum and median, 348 micrograms/gCr; range, 80-1325 micrograms/gCr in urine), and Kawasaki disease (median, 2.8 mg/l; range, 1.5-3.3 mg/l in serum and median, 1016 micrograms/gCr; range, 214-4500 micrograms/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 with HPS.
Hibi
S
S
Division of Pediatrics, Children's Research Hospital, Kyoto, Japan.
Ikushima
S
S
Fujiwara
F
F
Hashida
T
T
Tsunamoto
K
K
Todo
S
S
Imashuku
S
S
D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D005260:Female; D015616:Histiocytosis, Non-Langerhans-Cell / Q000097:blood / Q000175:diagnosis* / Q000652:urine; D006801:Humans; D007371:Interferon-gamma / Q000097:blood; D008297:Male; D015375:Receptors, Interleukin-2 / Q000032:analysis; D001613:beta 2-Microglobulin / Q000032:analysis* / Q000652:urine
D015375:Receptors, Interleukin-2; D001613:beta 2-Microglobulin; D007371:Interferon-gamma