Low‐affinity immunoglobulin gamma Fc region receptor III‐B (FcγRIIIB, CD16B) deficiency in patients with blood and immune system disorders

• Published in: British journal of haematology 2021-12, Vol.195 (5), p.743-747
• Main Authors: Minguela, Alfredo, Salido, Eduardo J., Soto‐Ramírez, María F., Olga, Montes‐Ares, Leal, Juan D., García‐Garay, María C., Periago, Adela, Berenguer, Mercedes, Blanque, Miguel, Campillo, José A.
• Format: Article
• Language: English
• Subjects: acute myeloid leukaemia; Acute myeloid leukemia; Adult; Affinity; Aged; Aged, 80 and over; Blood; Blood diseases; Bone Marrow - pathology; Disease Progression; Fc receptors; Female; GPI-Linked Proteins - analysis; Hematologic Diseases - pathology; Hematology; Humans; Idiopathic thrombocytopenic purpura; Immune system; Immune System Diseases - pathology; immune system disorders; Immunoglobulins; Leukemia, Myeloid, Acute - pathology; low‐affinity immunoglobulin gamma Fc region receptor III‐B (FcγRIIIB or CD16B) deficiency; Male; Myelodysplastic syndrome; Myelodysplastic Syndromes - pathology; Neutrophils - pathology; Purpura, Thrombocytopenic, Idiopathic - pathology; Receptors, IgG - analysis; Young Adult
• ISSN: 0007-1048; 1365-2141
• DOI: 10.1111/bjh.17828

Summary Low‐affinity immunoglobulin gamma Fc region receptor III‐B (FcγRIIIB) deficiency is present in ˜0·05% of the general population. Among our patients, FcγRIIIB deficiency was less frequent in those with immune‐system disorders (one of 1815 patients, 0·05%) than in those with blood disorders (nine of 2147 patients, 0·42%, P = 0·023): mainly primary immune thrombocytopenia (4·34%), therapy related myeloid neoplasms (1·16%) and myelodysplastic syndrome with excess blasts (1·28%). Four of the nine (44·4%) patients with blood disorders were diagnosed with or quickly evolved to acute myeloid leukaemia (AML), suggesting that FcγRIIIB deficiency could be an adverse prognostic factor for progression to AML that should be confirmed in large multicentre studies.