Autoimmune Lymphoproliferative Syndrome in Children with Nonmalignant Organomegaly, Chronic Immune Cytopenia, and Newly Diagnosed Lymphoma.

Abstract

This study investigated the frequency of and predictive factors for autoimmune lymphoproliferative syndrome (ALPS) in children with lymphoma, chronic immune cytopenia, and nonmalignant organomegaly. Thirty-four children with suspected ALPS (n=13, lymphoma; n=12, immune cytopenia; n=9, nonmalignant organomegaly) were included. Double-negative T-cells, lymphocyte apoptosis, and genetic findings were analyzed. Patients were stratified into two groups as proven/probable ALPS and clinically suspected patients according to the ALPS diagnostic criteria. Of the 34 patients, 18 (53%) were diagnosed with proven/probable ALPS. One patient had a mutation (c.652-2A>C) in the FAS gene. The remaining 16 (47%) patients were defined as clinically suspected patients. Predictive factors for ALPS were anemia and thrombocytopenia in patients with lymphoma, splenomegaly and lymphadenopathy in patients with immune cytopenia, and young age in patients with nonmalignant organomegaly. ALPS may not be rare in certain risk groups. Our study indicates that screening for ALPS may be useful in children having lymphoma with cytopenia at diagnosis, in those having nonmalignant organomegaly with immune cytopenia, and in those having chronic immune thrombocytopenic purpura or autoimmune hemolytic anemia with organomegaly developing during follow-up.

• Authors: Zühre Kaya, Melek Işık, Nihan Oruklu, Serap Kirkiz, Emin Ümit Bağrıaçık, Luis M. Allende, María J. Díaz-Madroñero, Raquel Ruiz-García, Faruk Güçlü Pınarlı, Pınar Göçün Uyar, Ülker Koçak
• Journal: Turkish journal of haematology : official journal of Turkish Society of Haematology (Turk J Haematol) Vol. 38 Issue 2 Pg. 145-150 (Jun 01 2021) ISSN: 1308-5263 [Electronic] Turkey
• PMID: 33375216 (Publication Type: Journal Article)

Chemical References

• FAS protein, human
• fas Receptor

Topics

• Adolescent
• Anemia (diagnosis, etiology, immunology)
• Anemia, Hemolytic, Autoimmune (diagnosis, etiology, immunology)
• Apoptosis (immunology)
• Autoimmune Lymphoproliferative Syndrome (complications, diagnosis, immunology, pathology)
• Child
• Child, Preschool
• Chronic Disease
• Cross-Sectional Studies
• Female
• Humans
• Infant
• Leukopenia (diagnosis, etiology, immunology)
• Lymphadenopathy (diagnosis, etiology, immunology)
• Lymphoma (diagnosis, etiology, immunology)
• Male
• Mutation
• Predictive Value of Tests
• Purpura, Thrombocytopenic, Idiopathic (diagnosis, etiology, immunology)
• Splenomegaly (diagnosis, etiology, immunology)
• T-Lymphocytes (immunology, pathology)
• Thrombocytopenia (diagnosis, etiology, immunology)
• fas Receptor (genetics)