An infant with both autoimmune neutropenia and idiopathic thrombocytopenia with IgG2/IgA deficiency.
We report an infant with autoimmune neutropenia (AIN), idiopathic thrombocytopenia (ITP), and IgG2/IgA deficiency. The patient was referred to our hospital at 5 months of age because of epistaxis and generalized petechiae. Physical examination revealed moderate hepatosplenomegaly. A complete blood count revealed a platelet count of 2.0 x 10(3) cells/microliters, and a white cell count of 3,600 cells/microliters, with severe neutropenia (less than 1% bands and segmented cells). Neutrophils and platelets adhering to megakaryocytes were decreased in the bone marrow. Tests for serum neutrophil-binding IgG (NB-IgG) and platelet-associated IgG (PA-IgG) were positive. A diagnosis of both AIN and ITP was made and therapy with intact-type gamma-globulin and prednisolone was initiated. Improvement occurred, but was temporary. A lack of serum IgA and IgG2 was noted during the clinical course. The patient has not been susceptible to bacterial infections but has had a severe clinical course with rubella and chickenpox.
Sugita
K
K
Second Department of Pediatrics, Dokkyo University School of Medicine, Tochigi, Japan.
Owada
Y
Y
Ozawa
T
T
Sakakibara
H
H
Eguchi
M
M
Furukawa
T
T
Saitoh
K
K
D001327:Autoimmune Diseases / Q000150:complications*; D006801:Humans; D017098:IgA Deficiency / Q000150:complications*; D017099:IgG Deficiency / Q000150:complications*; D007223:Infant; D008297:Male; D009503:Neutropenia / Q000150:complications*; D013921:Thrombocytopenia / Q000209:etiology*