title: Symptomatic Primary Selective IgM Immunodeficiency - B Lymphoid Cell Defect in Adult Man with Secondary HLH Syndrome.
abstract: Selective immunoglobulin M deficiency(sIgMD) is a rare form of dysgammaglobulinaemia characterized by an isolated low level of serum immunoglobulin M (IgM). It was an incidence of less than 0.03% in the general population and 1% in hospitalized patients. sIgMD may occur as a primary or secondary condition. sIgMD is much more common than primary .Hemophagocytic lymphohistiocytosis (HLH) is also a rare but potentially fatal disease of normal but overactive histiocytes and lymphocytes and can be primary or secondary, characterized by the overwhelming activation of normal T lymphocytes and macrophages, invariably leading to clinical and hematologic alterations. We report an adult case of primary sIgMD with absent B lymphoid cells and secondary HLH syndrome who presented with recurrent infections, fever and pancytopenia.
authors.affiliation: Senior Consultant and Head, Department of Internal Medicine.
authors.forename: Arun
authors.initials: A
authors.lastname: Agarwal
authors.affiliation: Consultant, Department of Pathology.
authors.forename: Samiksha
authors.initials: S
authors.lastname: Sharma
authors.affiliation: Clinical Director, Narayana Multispecialty Hospital, Jaipur, Rajasthan.
authors.forename: Mala
authors.initials: M
authors.lastname: Airun
mesh_terms: D000328:Adult; D001402:B-Lymphocytes; D004406:Dysgammaglobulinemia / Q000097:blood / Q000150:complications*; D006801:Humans; D007075:Immunoglobulin M / Q000172:deficiency*; D051359:Lymphohistiocytosis, Hemophagocytic / Q000097:blood / Q000150:complications* / Q000175:diagnosis*; D008297:Male
chemical_list: D007075:Immunoglobulin M