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《Journal of Applied Clinical Pediatrics》 2012-24
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Splenic Hamartoma with Immune Disorders in Multiple Systems in One Child

CHEN Xi1,WANG Shu-hong2,ZHANG Jing2(1.Department of Neurology,Children′s Hospital of Urumqi,Urumqi 830002,Xinjiang Uygur Autonomous Region,China;2.Department of Hematology,Children′s Hospital of Urumqi,Urumqi 830002,Xinjiang Uygur Autonomous Region,China)  
Objective To suggest the new ideas on the possibility that splenic hamartoma(SH) in children could be complicated with immune disorders in multiple systems on account of a novel case with typical clinical and histopathology presentation,as well his satisfied outcome after splenectomy.Methods Clinic data of 1 child with SH were collected.The diagnosis of autoimmune hemolytic anemia(AIHA)was made according to routine tests of blood,bone-marrow,Coombs,and red blood cell(RBC) fragility tests.The diagnosis of SH was made after splenectomy with histopathology and the specific immunohistochemistry stains for splenic tumors.The cerebrospinal fluid(CSF) routine test,cranial magnetic resonance imaging(MRI) and video electroencephalogram(VEEG) studies were performed during the activity stage of convulsion and the diagnosis of demyelination encephalitis was supported.The remote outcome for the all related complications was confirmed in 2.5 years follow-up after the surgical remove of spleen and SH.Results The patient suffered from recurrent episodes of jaundice,anemia complicated with chronic and progress splenomegaly at 3 months after birth.The inferior margin of the magnified liver was found at the 4 cm under the right ribs region and severe splenomegaly was presented at the age of 12 years when he was admitted to Children′s Hospital of Urumgi.Routine blood analysis showed severe anemia with elevated reticular RBCs(0.147).Direct Coombs test was negative,but indirect test was positive.Bone marrow test showed active proliferation in bone marrow but abnormal RBC genesis obviously,and elevated RBC osmotic fragility,confirming the diagnosis of AIHA.A single,circular like tumor(7.5 cm×8.0 cm) was detected with contrast CT.Histologic findings consist of unorganized vascular channels with intervening red pulp-like disorganized stroma.Immunohistochemistry on the lining cells of the splenoma vascular had a low proliferation index due to the antibody Ki-675%.The endothelial markets CD8,CD31,CD34(+),CD68(+),factor Ⅷ-related antigen and vimentin were positive in the endothelial cells of blood vessels of the SH.A diagnosis of SH was confirmed.Recurrent episodes of convulsion and disturbance of consciousness were occurred at the 11th day of this hospitalization.VEEG showed diffuse abnormal δ wave activity.Brain MRI showed abnormal foliated signals in the white matter at the right occipital lobe and around the bilateral anterior-posterior ventriculi lateralis.CSF routine and biochemical tests were normal,but myelin sheath alkaline protein was positive,supporting the diagnosis of demyelinative encephalitis.The patient underwent surgical removal of spleen and SH on the 25th day of this hospitalization.All medications were stopped after surgery with 2.5 years follow-up.No episodes of anemia and seizure occurred.Growth was significantly improved at the 0.5 years follow-up after surgery with normal blood count,hemoglobin and Coombs test.VEEG and brain MRI were also returned to normal after 1 year,further supporting the critical role of SH in the autoimmune anemia of the patient.Conclusions SH in children are more common to have some complications such as thrombocytopenia or other blood cell reduction than that in adults,the cause is always believed as hypersplenism.This is the second case reported in the world,but this is the first case reported who suffered from SH complicated with indirect Coombs test positive AIHA in the world,and highly indicates the possibility of AIHA as the complication of SH.Any patient with unexplained AIHA should rule out SH.Although the positive relationship between SH and encephalitis cannot be confirmed,but more attention should be given to the possibility of SH associated with multiple immune disorders.
【CateGory Index】: R733.2
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