Combined immunodeficiency with marginal zone lymphoma due to a novel homozygous mutation in<i>IL-21R</i>gene and successful treatment with hematopoietic stem cell transplantation

Karaca, Neslihan Edeer; Özek, Gülcihan; Ataseven, Eda; Tökmeci, Nazan; Şenol, Handan Duman; Kıran, Ezgi; Aykut, Ayça; Durmaz, Asude; Aksu, Güzide; Aksoylar, Serap; Aydoğdu, Sema; Çetingül, Nazan; Kütükçüler, Necil

doi:10.1080/08880018.2021.1924326

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Cited by 4 publications

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Current genetic defects in common variable immunodeficiency patients on the geography between Europe and Asia: a single-center experience

Aygun,

Topyıldız,

Geyik

et al. 2023

Immunol Res

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Current genetic defects in common variable immunodeficiency patients on the geography between Europe and Asia: a single-center experience

Aygun,

Topyıldız,

Geyik

et al. 2023

Immunol Res

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Indolent B-cell non-Hodgkin lymphomas in children: high association with inborn errors of immunity

Kurucu,

Kutluk,

Sağlam

et al. 2024

Leukemia & Lymphoma

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A Rare Case of Refractory Nodal Marginal Zone Lymphoma in a Child, Successfully Cured by Allogeneic Stem Cell Transplant: A Case Report

Memon,

Almudayfir,

Elbashir

et al. 2024

Pediatric Transplantation

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BackgroundNodal marginal zone lymphoma (NMZL) is a rare form of non‐Hodgkin lymphoma (NHL), accounting for around 2% of all NHL in pediatrics and adults. It is considered the least common subtype of marginal zone lymphoma, which is rarely seen in pediatrics and is usually viewed as a mild disease. In adults, its presentation varies from mild to aggressive disease with multiple lymph node enlargement and systemic symptoms.Case ReportHere, we report an interesting case of a 31‐month‐old boy who developed refractory EBV‐positive NMZL with extensive disease. The tumor did not respond well to conventional chemotherapy and Rituximab. Therefore, we manage the disease with second‐line chemotherapy, including bortezomib, followed by an allogeneic hematopoietic stem cell transplant (HSCT) with an excellent outcome.ConclusionVery few cases have been reported for aggressive NMZL children. The tumor was refractory to first line chemotherapy. However, the successful outcome was achieved by allogeneic stem cell transplant with myeloablative conditioning chemotherapy. The significance of this particular case is that this is the first reported case from pediatrics for refractory NMZL, which responded well to allogeneic stem cell transplant.

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Combined immunodeficiency with marginal zone lymphoma due to a novel homozygous mutation inIL-21Rgene and successful treatment with hematopoietic stem cell transplantation

Cited by 4 publications

References 16 publications

Current genetic defects in common variable immunodeficiency patients on the geography between Europe and Asia: a single-center experience

Current genetic defects in common variable immunodeficiency patients on the geography between Europe and Asia: a single-center experience

Indolent B-cell non-Hodgkin lymphomas in children: high association with inborn errors of immunity

A Rare Case of Refractory Nodal Marginal Zone Lymphoma in a Child, Successfully Cured by Allogeneic Stem Cell Transplant: A Case Report

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