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Year : 2019  |  Volume : 44  |  Issue : 2  |  Page : 149-156

Correlation of B cell activating factor, B cell maturation antigen, and other cytokines profile with inhibitor status in hemophilia A

1 Department of Pathology, Dr Ram Manohar Lohia Institute of Medical Sciences, Gomti Nagar, Lucknow, Uttar Pradesh, India
2 Department of Biochemistry, Dr Ram Manohar Lohia Institute of Medical Sciences, Gomti Nagar, Lucknow, Uttar Pradesh, India

Correspondence Address:
Nuzhat Husain
Department of Pathology, Dr. Ram Manohar Lohia Institute of Medical Sciences, Gomti Nagar, Lucknow 226010, Uttar Pradesh
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ejh.ejh_6_19

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Background The role of cytokines in inhibitor-positive versus inhibitor-negative patients with hemophilia A (PWHA) is controversial. Raised levels of B cell activating factor (BAFF) and B cell maturation antigen (BCMA) have been recently reported and have been linked to presence of inhibitors. Aim To directly quantify serum BAFF and BCMA along with interleukin (IL)-10, IL-5, and tumor necrosis factor-α (TNF-α) in PWHA following an on-demand schedule for factor VIII replacement, and to correlate these with demographic, clinicopathological, and treatment parameters. Patients and methods The study group included 35 inhibitor-positive, 131 inhibitor-negative PWHA, and 20 healthy controls. Serum levels of BAFF, BCMA, IL-10, IL-5, and TNF-α were determined by enzyme-linked immunosorbent assay. Results The mean serum levels in inhibitor positive, inhibitor negative, and healthy controls for BAFF was 0.346, 0.218, and 0.118 pg/ml (P=0.280), for BCMA was 0.266, 0.176, and 0.187 pg/ml (P=0.298), for IL-10 was 13.71, 5.72, and 2.45 pg/ml (P≤0.001), for IL-5 was 2.90, 2.74, and 2.50 pg/ml (P=0.205) and for TNF-α was 5.20, 6.78, and 4.0 pg/ml (P=0.558), respectively. Six (30%) inhibitor-positive PWHA had elevated BCMA levels while none of the inhibitor-negative PWHA showed increased values. Conclusion The current study has, for the first time, analyzed BAFF and BCMA in PWHA with inhibitor status in a clinical setting of on-demand therapy. As a preliminary conclusion we feel that, while cytokines play a role in pathogenesis they do not seem to trigger inhibitor development in PWHA.

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