Conţinutul numărului revistei |
Articolul precedent |
Articolul urmator |
260 0 |
SM ISO690:2012 TOMACINSCHI, Cristina, SELEVESTRU, Rodica, SINIŢÎNA, Lilia, PETROVICH, Virgil, GOLBAN, Rodica, TSURYA, Valentin, ŞCIUCA, Svetlana. ABCL-265 Hematologic Malignancies in Pediatrics Inborn Errors of Immunity. In: Clinical Lymphoma, Myeloma and Leukemia, 2022, nr. 22, pp. S367-S368. ISSN 2152-2650. DOI: https://doi.org/10.1016/S2152-2650(22)01518-X |
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Clinical Lymphoma, Myeloma and Leukemia | ||||||
Numărul 22 / 2022 / ISSN 2152-2650 /ISSNe 2152-2669 | ||||||
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DOI:https://doi.org/10.1016/S2152-2650(22)01518-X | ||||||
Pag. S367-S368 | ||||||
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Context: Inherited errors of immunity (IEI) or primary immunodeficiencies are a group of genetic rare diseases predisposing to severe infections, autoimmunity, and malignancies. The overall risk for malignancies in pediatric IEI-patients is estimated to range from 4–25%, of which 60% of cases are of hematological origin. Objective: Evaluation of hematological malignancies in pediatrics IEI. Methods: Were included 13 patients 3 of which developed hematological malignancies and were evaluated for demographics, clinical features, and prognosis. Results: The prevalence of hematological malignancies among our patients was 23% (3/13). The male-to-female ratio was 2:1. The mean age at diagnosis of malignancy was 8.6 years (range 5-14). Non-Hodgkin lymphoma was the malignancy type diagnosed in all the pediatric patients (n=3, of which Wiskott-Aldrich-1, ataxia-telangiectasia-1, common variable immunodeficiency (CVID)-1. However, 2 patients (52.5%) died (1-during first line-chemotherapy, 1-NHL was established postmortem). 1 patient underwent bone marrow transplantation and is currently under supervision. Conclusions: In this study, we related an association between hematological malignancies and IEI. Lymphoid malignancies were the most common malignancy, this warns of the need for multidisciplinary monitoring of these patients. |
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Cuvinte-cheie ABCL, children, immunodeficiency, lymphoma, malignancy, Wiskott-Aldrich |
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