TY  - JOUR
TI  - Autoimmune Lymphoproliferative Syndrome in Children with
Nonmalignant Organomegaly, Chronic Immune Cytopenia, and
Newly Diagnosed Lymphoma
AB  - 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.
AU  - ORUKLU, Nihan
AU  - Kocak, Ulker
AU  - Pinarli, Faruk Guclu
AU  - D AZ-MADRO ERO, Mar a J.
AU  - Kirkiz, Serap
AU  - IŞIK, Melek
AU  - Kaya, Zuhre
AU  - RUİZ-GARC A, Raquel
AU  - GÖÇÜN UYAR, Pınar
AU  - ALLENDE, Luis M.
AU  - BAĞRIAÇIK, Emin Ümit
DO  - 10.4274/tjh.galenos.2020.2020.0618
PY  - 2021
JO  - Turkish Journal of Hematology
VL  - 38
IS  - 2
SN  - 1300-7777
SP  - 145
EP  - 150
DB  - TRDizin
UR  - http://search/yayin/detay/522471
ER  -