期刊
JOURNAL OF PEDIATRIC HEMATOLOGY ONCOLOGY
卷 37, 期 7, 页码 507-508出版社
LIPPINCOTT WILLIAMS & WILKINS
DOI: 10.1097/MPH.0000000000000366
关键词
IL-2-inducible T-cell kinase (ITK) deficiency; EBV lymphoproliferative disorder; Hodgkin lymphoma
Background:Inherited or acquired immune dysregulation is associated with increased risk of lymphoproliferative disorders (LPDs), including classic Hodgkin lymphoma (cHL). A germline mutation in interleukin-2-inducible T-cell kinase (ITK) is described in individuals manifesting B-cell LPDs, cHL, and hemophagocytic syndromes following Epstein-Barr virus (EBV) infection.Observations:We report a novel ITK mutation in a child with EBV-associated cHL and multiple-site reactive polyclonal B-cell hyperplasia followed by relapsed cHL at another site. Following relapse, the child was successfully treated with allogeneic hematopoietic stem cell transplantation and EBV cytotoxic T cells.Conclusions:ITK-mutated T cells cause a defective antiviral immune response and the resulting immune dysregulation can lead to EBV-associated polyclonal hyperplasia with subsequent outgrowth of neoplastic B-cell clones, which in some instances may progress to LPDs, including cHL.
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