Autoimmune Lymphoproliferative Syndrome in Children with Nonmalignant Organomegaly, Chronic Immune Cytopenia, and Newly Diagnosed Lymphoma
Yıl: 2021 Cilt: 38 Sayı: 2 Sayfa Aralığı: 145 - 150 Metin Dili: İngilizce DOI: 10.4274/tjh.galenos.2020.2020.0618 İndeks Tarihi: 21-06-2022
Autoimmune Lymphoproliferative Syndrome in Children with Nonmalignant Organomegaly, Chronic Immune Cytopenia, and Newly Diagnosed Lymphoma
Öz: 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.
Anahtar Kelime: Malign Olmayan Organomegali, Kronik İmmün Sitopeni ve Yeni Tanı Lenfomalı Çocuklarda Otoimmün Lenfoproliferatif Sendrom
Öz: Bu çalışmanın amacı malign olmayan organomegali, kronik immün
sitopeni ve lenfomalı çocuklarda otoimmün lenfoproliferatif sendrom
(OILS) sıklığını ve belirleyici faktörlerini araştırmaktır. Bu çalışmaya
OILS şüpheli 34 hasta dahil edildi (13 hasta lenfoma, 5 hasta otoimmün
hemolitik anemi, 7 hasta kronik immün trombositopenik purpura
ve 9 hasta malign olmayan organomegali). Çift negatif T-hücreler,
lenfosit apoptozis ve genetik bulgular analiz edildi. Hastalar OILS
tanı kriterlerine göre kesin ve yüksek olasılıklı OILS’li hastalar ve
klinik şüpheli OILS’li hastalar olarak iki gruba ayrıldı. Çalışmaya dahil
edilen 34 hastanın, 18’i (%53) kesin ve yüksek olasılıklı OILS’di. Malign
olmayan organomegalisi olan bir çocukta FAS geninde mutasyon
(c.652-2A>C) saptandı. Klinik şüpheli hasta 16 (%47) idi. OILS için
belirleyici faktörler; lenfomalı hastalarda anemi ve trombositopeni,
kronik immün sitopenili hastalarda splenomegali ve lenfadenopati;
malign olmayan organomegalisi olan hastalarda genç yaş idi. OILS
belirli risk gruplarında nadir olmayabilir. Çalışmamız, ilk tanıda
sitopenisi olan lenfomalı çocuklarda, immün sitopenisi olan nonmalign
organomegalili çocuklarda ve izlem sırasında organomegalisi gelişen
kronik immün trombositopenik purpura ve otoimmün hemolitik
anemili çocuklarda OILS taramasının yararlı olabileceğine işaret
etmektedir.
Anahtar Kelime: Belge Türü: Makale Makale Türü: Araştırma Makalesi Erişim Türü: Erişime Açık
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| APA | Kaya Z, IŞIK M, ORUKLU N, Kirkiz S, BAĞRIAÇIK E, ALLENDE L, D AZ-MADRO ERO M, RUİZ-GARC A R, Pinarli F, GÖÇÜN UYAR P, Kocak U (2021). Autoimmune Lymphoproliferative Syndrome in Children with Nonmalignant Organomegaly, Chronic Immune Cytopenia, and Newly Diagnosed Lymphoma. , 145 - 150. 10.4274/tjh.galenos.2020.2020.0618 |
| Chicago | Kaya Zuhre,IŞIK Melek,ORUKLU Nihan,Kirkiz Serap,BAĞRIAÇIK Emin Ümit,ALLENDE Luis M.,D AZ-MADRO ERO Mar a J.,RUİZ-GARC A Raquel,Pinarli Faruk Guclu,GÖÇÜN UYAR Pınar,Kocak Ulker Autoimmune Lymphoproliferative Syndrome in Children with Nonmalignant Organomegaly, Chronic Immune Cytopenia, and Newly Diagnosed Lymphoma. (2021): 145 - 150. 10.4274/tjh.galenos.2020.2020.0618 |
| MLA | Kaya Zuhre,IŞIK Melek,ORUKLU Nihan,Kirkiz Serap,BAĞRIAÇIK Emin Ümit,ALLENDE Luis M.,D AZ-MADRO ERO Mar a J.,RUİZ-GARC A Raquel,Pinarli Faruk Guclu,GÖÇÜN UYAR Pınar,Kocak Ulker Autoimmune Lymphoproliferative Syndrome in Children with Nonmalignant Organomegaly, Chronic Immune Cytopenia, and Newly Diagnosed Lymphoma. , 2021, ss.145 - 150. 10.4274/tjh.galenos.2020.2020.0618 |
| AMA | Kaya Z,IŞIK M,ORUKLU N,Kirkiz S,BAĞRIAÇIK E,ALLENDE L,D AZ-MADRO ERO M,RUİZ-GARC A R,Pinarli F,GÖÇÜN UYAR P,Kocak U Autoimmune Lymphoproliferative Syndrome in Children with Nonmalignant Organomegaly, Chronic Immune Cytopenia, and Newly Diagnosed Lymphoma. . 2021; 145 - 150. 10.4274/tjh.galenos.2020.2020.0618 |
| Vancouver | Kaya Z,IŞIK M,ORUKLU N,Kirkiz S,BAĞRIAÇIK E,ALLENDE L,D AZ-MADRO ERO M,RUİZ-GARC A R,Pinarli F,GÖÇÜN UYAR P,Kocak U Autoimmune Lymphoproliferative Syndrome in Children with Nonmalignant Organomegaly, Chronic Immune Cytopenia, and Newly Diagnosed Lymphoma. . 2021; 145 - 150. 10.4274/tjh.galenos.2020.2020.0618 |
| IEEE | Kaya Z,IŞIK M,ORUKLU N,Kirkiz S,BAĞRIAÇIK E,ALLENDE L,D AZ-MADRO ERO M,RUİZ-GARC A R,Pinarli F,GÖÇÜN UYAR P,Kocak U "Autoimmune Lymphoproliferative Syndrome in Children with Nonmalignant Organomegaly, Chronic Immune Cytopenia, and Newly Diagnosed Lymphoma." , ss.145 - 150, 2021. 10.4274/tjh.galenos.2020.2020.0618 |
| ISNAD | Kaya, Zuhre vd. "Autoimmune Lymphoproliferative Syndrome in Children with Nonmalignant Organomegaly, Chronic Immune Cytopenia, and Newly Diagnosed Lymphoma". (2021), 145-150. https://doi.org/10.4274/tjh.galenos.2020.2020.0618 |
| APA | Kaya Z, IŞIK M, ORUKLU N, Kirkiz S, BAĞRIAÇIK E, ALLENDE L, D AZ-MADRO ERO M, RUİZ-GARC A R, Pinarli F, GÖÇÜN UYAR P, Kocak U (2021). Autoimmune Lymphoproliferative Syndrome in Children with Nonmalignant Organomegaly, Chronic Immune Cytopenia, and Newly Diagnosed Lymphoma. Turkish Journal of Hematology, 38(2), 145 - 150. 10.4274/tjh.galenos.2020.2020.0618 |
| Chicago | Kaya Zuhre,IŞIK Melek,ORUKLU Nihan,Kirkiz Serap,BAĞRIAÇIK Emin Ümit,ALLENDE Luis M.,D AZ-MADRO ERO Mar a J.,RUİZ-GARC A Raquel,Pinarli Faruk Guclu,GÖÇÜN UYAR Pınar,Kocak Ulker Autoimmune Lymphoproliferative Syndrome in Children with Nonmalignant Organomegaly, Chronic Immune Cytopenia, and Newly Diagnosed Lymphoma. Turkish Journal of Hematology 38, no.2 (2021): 145 - 150. 10.4274/tjh.galenos.2020.2020.0618 |
| MLA | Kaya Zuhre,IŞIK Melek,ORUKLU Nihan,Kirkiz Serap,BAĞRIAÇIK Emin Ümit,ALLENDE Luis M.,D AZ-MADRO ERO Mar a J.,RUİZ-GARC A Raquel,Pinarli Faruk Guclu,GÖÇÜN UYAR Pınar,Kocak Ulker Autoimmune Lymphoproliferative Syndrome in Children with Nonmalignant Organomegaly, Chronic Immune Cytopenia, and Newly Diagnosed Lymphoma. Turkish Journal of Hematology, vol.38, no.2, 2021, ss.145 - 150. 10.4274/tjh.galenos.2020.2020.0618 |
| AMA | Kaya Z,IŞIK M,ORUKLU N,Kirkiz S,BAĞRIAÇIK E,ALLENDE L,D AZ-MADRO ERO M,RUİZ-GARC A R,Pinarli F,GÖÇÜN UYAR P,Kocak U Autoimmune Lymphoproliferative Syndrome in Children with Nonmalignant Organomegaly, Chronic Immune Cytopenia, and Newly Diagnosed Lymphoma. Turkish Journal of Hematology. 2021; 38(2): 145 - 150. 10.4274/tjh.galenos.2020.2020.0618 |
| Vancouver | Kaya Z,IŞIK M,ORUKLU N,Kirkiz S,BAĞRIAÇIK E,ALLENDE L,D AZ-MADRO ERO M,RUİZ-GARC A R,Pinarli F,GÖÇÜN UYAR P,Kocak U Autoimmune Lymphoproliferative Syndrome in Children with Nonmalignant Organomegaly, Chronic Immune Cytopenia, and Newly Diagnosed Lymphoma. Turkish Journal of Hematology. 2021; 38(2): 145 - 150. 10.4274/tjh.galenos.2020.2020.0618 |
| IEEE | Kaya Z,IŞIK M,ORUKLU N,Kirkiz S,BAĞRIAÇIK E,ALLENDE L,D AZ-MADRO ERO M,RUİZ-GARC A R,Pinarli F,GÖÇÜN UYAR P,Kocak U "Autoimmune Lymphoproliferative Syndrome in Children with Nonmalignant Organomegaly, Chronic Immune Cytopenia, and Newly Diagnosed Lymphoma." Turkish Journal of Hematology, 38, ss.145 - 150, 2021. 10.4274/tjh.galenos.2020.2020.0618 |
| ISNAD | Kaya, Zuhre vd. "Autoimmune Lymphoproliferative Syndrome in Children with Nonmalignant Organomegaly, Chronic Immune Cytopenia, and Newly Diagnosed Lymphoma". Turkish Journal of Hematology 38/2 (2021), 145-150. https://doi.org/10.4274/tjh.galenos.2020.2020.0618 |
