Lymphoid interstitial pneumonia(LIP) is an uncommon process in childhood, and is characterized by interstitial accumulation of mature lymphocytes, plasma cells and reticuloendothelial cells. LIP is believed to have autoimmune or immunologic
pathogenesis, because it is frequently associated with acquired immune deficiency syndrome, Epstein-Barr virus infection, common variable immunodeficiency.
The patient described in this in this report had idiopathic thrombocytopenic purpura, Evans syndrome and LIP . The diminished levels of igG1 IgG2 were the only immunologic abnormality, which suggest that this patient may be in the early phase of
common
variable immunodeficiency or selective IgG2 ＆IgG4 deficiency due to immunoglobulin heavy chain deletion. We can rule out the possibility of lymphoma by clonality study, which showed polyclonal lymphocyte infiltration. On our knowledge, this is
the
first report of LIP in Korean children.
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