Pediatric Hematology/Oncology and ImmunopathologyPediatric Hematology/Oncology and ImmunopathologyВопросы гематологии/онкологии и иммунопатологии в педиатрии1726-17082414-9314Fund Doctors, Innovations, Science for Children15010.24287/1726-1708-2016-15-1-61-65ИММУНОЛОГИЯAnalysis of the incidence of lymphomas in children with primary immunodeficienciesАнализ частоты развития лимфом у детей с первичными иммунодефицитными состояниямиDeripapaElena V.ДерипапаЕлена Васильевна
Russian Federation
deripapa@mail.ruShvetsOksana A.ШвецОксана Анатольевна
Russian Federation
shv18081979@gmail.comAbramovDmitry S.АбрамовДмитрий Сергеевич
Russian Federation
pathmorf@mail.ruMyakovaNatalya V.МяковаНаталья Валерьевна
Russian Federation
nmiakova@mail.ruShcherbinaAnna Yu.ЩербинаАнна Юрьевна
Russian Federation
shcher26@hotmail.comFederal Research Center of Pediatric Hematology, Oncology, and Immunology named after Dmitry RogachevФедеральный научно-клинический центр детской гематологии, онкологии и иммунологии им. Дмитрия Рогачева Минздрава России19032016151616519092018Copyright ©; 2016, «D. Rogachev NMRCPHOI»Copyright ©; 2016, ФГБУ «НМИЦ ДГОИ им. Дмитрия Рогачева» Минздрава России2016«D. Rogachev NMRCPHOI»ФГБУ «НМИЦ ДГОИ им. Дмитрия Рогачева» Минздрава Россииhttps://creativecommons.org/licenses/by/4.0https://hemoncim.com/jour/article/view/150

Primary immunodeficiencies (PIDs) are genetically determined irreversible dysfunctions of the immune system. Along with infectious and autoimmune complications, patients with PIDs have high incidence of various malignancies, primarily lymphomas. We analyzed histories of the disease of 27 patients with lymphomas and PIDs, observed at the Federal Research Center of Pediatric Hematology, Oncology, and Immunology named after Dmitry Rogachev (Moscow), and compared our results with the data from American Immunodeficiency Cancer Registry (ICR). According to our data, patients with chromosomal breakage syndromes and unspecified PIDs predominate in the group of patients with lymphomas and PIDs. High proportion of patients with Nijmegen breakage syndrome (29%) in our group of patients with lymphomas could be explained by the Slavic mutation in the East European population. PIDs in patients with lymphomas are often diagnosed late - only after the development of lymphoma. Early diagnosis of PIDs and alertness with respect to malignancies in patients with certain PIDs are very important and dictate the need of conduction of studies of malignant lymphoproliferative diseases in these patients.

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