Immune status of patients with haemophilia A before exposure to factor  VIII : first results from the  HEMFIL  study | Zendy

Jardim Letícia L. | Zendy; Chaves Daniel G. | Zendy; SilveiraCassette Amanda C. O. | Zendy; Simões e Silva Ana Cristina | Zendy; Santana Marcio P. | Zendy; Cerqueira Monica H. | Zendy; Prezotti Alessandra | Zendy; Lorenzato Claudia | Zendy; Franco Vivian | Zendy; Bom Johanna G. | Zendy; Rezende Suely M. | Zendy

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Immune status of patients with haemophilia A before exposure to factor VIII : first results from the HEMFIL study

Author(s) -

Jardim Letícia L.,

Chaves Daniel G.,

SilveiraCassette Amanda C. O.,

Simões e Silva Ana Cristina,

Santana Marcio P.,

Cerqueira Monica H.,

Prezotti Alessandra,

Lorenzato Claudia,

Franco Vivian,

Bom Johanna G.,

Rezende Suely M.

Publication year - 2017

Publication title -

british journal of haematology

Language(s) - English

Resource type - Journals

SCImago Journal Rank - 1.907

H-Index - 186

eISSN - 1365-2141

pISSN - 0007-1048

DOI - 10.1111/bjh.14799

Subject(s) - interleukin 10 , chemokine , medicine , immunology , haemophilia , immune system , interleukin 8 , platelet , interleukin 4 , interleukin , inflammation , eotaxin , cytokine , pediatrics

Summary Previous cross‐sectional studies showed that some patients with haemophilia A ( HA ) without inhibitor presented a pro‐inflammatory profile during factor VIII ( FVIII ) replacement therapy. Furthermore, an anti‐inflammatory/regulatory state was described in HA patients after inhibitor development. However, no study investigated the levels of these biomarkers before exposure to exogenous FVIII . This study investigated the immunological profile of previously untreated patients ( PUP s) with HA in comparison with non‐haemophiliac boys. A panel of chemokines and cytokines was evaluated in the plasma of 40 PUP s with HA and 47 healthy controls. The presence of microparticles was assessed in the plasma of 32 PUP s with HA and 47 healthy controls. PUP s with HA presented higher levels of CXCL 8 ( IL 8), IL 6, IL 4, IL 10, IL 2, IL 17A ( IL 17), and lower levels of CXCL 10 ( IP ‐10) and CCL 2 ( MCP ‐1) than the age‐matched healthy controls ( P < 0·05). We also observed higher levels of microparticles derived from endothelium, erythrocytes, platelets, leucocytes, neutrophils, and T lymphocytes in patients in comparison with controls ( P < 0·05). Compared with controls, PUP s with HA presented a distinct immunological profile, characterized by a prominent pro‐inflammatory status that appears to be regulated by IL 4 and IL 10.

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