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Idiopathic CD4+ lymphocytopenia and extranodal marginal zone lymphoma of mucosa‐associated lymphoid tissue (MALT lymphoma) are rare diseases in children. We report the first case of a child with STK4‐mutated CD4+ lymphocytopenia who developed Epstein–Barr virus associated MALT lymphoma arising in the salivary gland. The child achieved complete remission with rituximab, and her immunodeficiency was...
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