Secondary Fisher–Evans syndrome in a child with activated PI(3)kd syndrome and lymphoma

Zh. A. Kuzminova; Кузьминова Ж. А.; V. V. Fominykh; Фоминых В. В.; N. N. Kotskaya; Коцкая Н. Н.; K. V. Mitrakov; Митраков К. В.; A. A. Moiseeva; Моисеева А. А.; O. A. Shvets; Швец О. А.; A. M. Livshits; Лившиц А. М.; M. A. Kurnikova; Курникова М. А.

doi:10.24287/1726-1708-2023-22-1-152-155

Secondary Fisher–Evans syndrome in a child with activated PI(3)kd syndrome and lymphoma

Authors: Kuzminova Z.A.¹, Fominykh V.V.¹, Kotskaya N.N.¹, Mitrakov K.V.¹, Moiseeva A.A.¹, Shvets O.A.¹, Livshits A.M.¹, Kurnikova M.A.¹
Affiliations:
1. The Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology of Ministry of Healthсare of the Russian Federation
Issue: Vol 22, No 1 (2023)
Pages: 152-155
Section: CLINICAL OBSERVATIONS
Submitted: 21.01.2023
Accepted: 30.01.2023
Published: 14.02.2023
URL: https://hemoncim.com/jour/article/view/686
DOI: https://doi.org/10.24287/1726-1708-2023-22-1-152-155
ID: 686

Cite item

Full Text

Abstract
About the authors
References
Supplementary files
Statistics

Abstract

Evans syndrome, a combination of autoimmune hemolytic anemia and immune thrombocytopenia, is a rare disease in children. In childhood, it may turn out to be one of the first manifestations of a primary immunodeficiency or an immune dysregulation syndrome. Here we present a clinical case of a patient who was initially diagnosed with Evans syndrome and did not respond well to therapy. Based on the results of genetic testing, the child was then diagnosed with primary immunodeficiency, namely, activated PI(3)kd syndrome. During follow-up, the patient developed lymphoma and had to undergo radical treatment (allogeneic hematopoietic stem cell transplantation). The patient's parents gave consent to the use of their child's data, including photographs, for research purposes and in publications.