The Prognostic Significance of Beta2 Microglobulin in Patients with Hemophagocytic Lymphohistiocytosis.
OBJECTIVE
To determine the prognostic significance of beta2 microglobulin (β2-m) concentrations in patients with hemophagocytic lymphohistiocytosis (HLH), a rare disorder caused by pathologic activation of the immune system.


METHODS
The study population consisted of 74 patients diagnosed with HLH and 35 healthy controls. Serum β2-m levels were measured using a latex agglutination photometric immunoassay.


RESULTS
Median serum β2-m levels were significantly higher in HLH patients than in healthy controls (4.05 versus 1.5 mg/L; P < 0.001) and were significantly higher in patients with lymphoma associated hemophagocytic syndrome (LAHS) than in patients with benign disease-associated HLH (4.2 versus 3.3 mg/L; P < 0.001). Higher serum β2-m levels were positively correlated with LAHS (P = 0.005), abnormal lactate dehydrogenase concentrations (P = 0.009), and hypoalbuminemia (P = 0.003). ROC analysis showed that overall survival (OS) was significantly shorter in LAHS patients with serum β2-m levels ≥4.03 mg/L compared to <4.03 mg/L (P < 0.001). Moreover, multivariate analysis showed that serum β2-m level was an independent prognostic of OS (P = 0.034) in patients with LAHS.


CONCLUSIONS
High serum β2-m levels and LAHS were associated with markedly poorer OS in patients with HLH. Serum β2-m concentration was a powerful and independent prognostic factor for OS in patients with LAHS.
Department of Cardiology, Beijing University of Chinese Medicine Third Affiliated Hospital, Beijing 100029, China.
Tiantong
T
Jiang
Departments of Clinical Laboratory, Beijing Youan Hospital, Capital Medical University, Beijing 100069, China.
Xiurong
X
Ding
Department of Cardiology, Beijing University of Chinese Medicine Third Affiliated Hospital, Beijing 100029, China.
Weixing
W
Lu
D000328:Adult; D005260:Female; D006801:Humans; D051359:Lymphohistiocytosis, Hemophagocytic / Q000378:metabolism* / Q000401:mortality; D008223:Lymphoma / Q000209:etiology / Q000378:metabolism* / Q000401:mortality; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D012372:ROC Curve; D016019:Survival Analysis; D015854:Up-Regulation; D055815:Young Adult; D001613:beta 2-Microglobulin / Q000097:blood*
D001613:beta 2-Microglobulin