Diagnostic and prognostic value of CD163 for infection-associated hemophagocytic syndrome in children / 中华实用儿科临床杂志

Objective To investigate the diagnostic and prognostic value of serum soluble CD163 (sCD163 )and the positive rate of membrane -bound CD163 (mCD163 )in peripheral blood mononuclear cells (PBMC)in children with infection -associated hemophagocytic syndrome (IAHS).Methods Between July 2012 and June 2016,26 pediatric patients with IAHS (IAHS group)and 28 pediatric patients with sepsis(sepsis group)admitted to Children′s Hospital Affiliated to Shanghai Jiaotong University were selected,and 20 healthy children were taken as healthy control group. Sandwich enzyme linked immunosorbent assay was used to detect serum sCD163 .The population of circulating mCD163 positive monocytes was determined by using flow cytometry.Receiver operating characteristic (ROC)curves were used to evaluate the diagnostic and prognostic values of sCD163 and mCD163 in children with IAHS compared with the diagnos-tic and prognostic values of plasma ferritin,and so on.Results The serum levels of sCD163 in patients of IAHS group, sepsis group and healthy control group were (1264 ±538)mg/L,(862 ±332)mg/L,(610 ±316)mg/L,respective-ly.And the population of mCD163 -positive PBMC in patients of IAHS group,sepsis group and healthy control group was (88.3 ±9.7)%,(68.5 ±18.3)%,(28.9 ±5.2)%,respectively.Both serum sCD163 and the population of mCD163 -positive PBMC were significantly higher in IAHS group compared with those of sepsis group (t =2.031 ,P =0.048;t =3.191 ,P =0.002,respectively).The serum sCD163 and population of mCD163 -positive PBMC in sepsis group were higher than controls (t =3.848,P =0.002;t =4.049,P =0.000,respectively).Moreover,the areas under the ROC curve (AUC)for the mCD163 ,sCD163 ,were 0.853(P =0.013),0.762(P =0.004),0.755(P =0.049),respec-tively.mCD163 at a cutoff of 83.7% had a high diagnosis sensitivity (81 .8%)and specificity (72.4%).The optimal cutoff values of sCD163 and ferritin for predicting IAHS was 888 mg/L (sensitivity 66.7% and specificity 63.3%)and 2880 μg/L (sensitivity 80.0% and specificity 54.5%).In addition,the serum level of sCD163 and the population of mCD163 -positive PBMCs were significantly increased in acute phase and decreased in recovery phase[(1553 ±542) mg/L vs.(866 ±92)mg/L,(91 .0 ±6.4)% vs.(79.0 ±4.6)%,t =2.450,χ2 =3.419,P =0.036,0.007]in IAHS group.Furthermore,subgroup analysis indicated that the serum level of sCD163 and the population of mCD163 -positive PBMCs were significantly higher in dead patients than those in survived patients [(1748.91 ±518.17)mg/L vs. (909.69 ±171 .35)mg/L,t =3.070,P =0.011 ;(93.50 ±8.42)% vs.(77.30 ±3.28)%,χ2 =3.005,P =0.024, respectively].Conclusion Serum sCD163 and the population of mCD163 -positive PMSCs are specific and validity bio-markers for early diagnosis of IAHS,which also are associated with treatment response assessment and prognostic analy-sis in IAHS.

Adenovirus-induced Hemophagocytic Lymphohistiocytosis in a Previously Healthy Boy: A Case Report and Literature Review / 임상소아혈액종양

A 3-year-old previously healthy boy was admitted because of a 1-week history of fever, abdominal pain, vomiting, and diarrhea. The initial laboratory tests showed hepatic dysfunction with disseminated intravascular coagulation. There was a large amount of pleural effusion, periportal edema, minimal ascites, and splenomegaly. He was initially managed with broad spectrum antibiotics with transfusion. Despite 2 days of treatment, the fever persisted and the results of the laboratory tests had worsened. Bacterial cultures from the blood, urine, pleural effusion, and ascites were all negative. He was finally diagnosed with hemophagocytic lymphohistiocytosis (HLH) based on the diagnostic criteria. Adenovirus was detected in the initial diarrhea and nasal swab specimens using polymerase chain reaction-based method. One year after chemotherapy with dexamethasone, cyclosporine, and etoposide, he is now healthy without evidence of disease recurrence. This is the first Korean case report of adenovirus-induced HLH in a previously healthy child.

Adenovirus-induced Hemophagocytic Lymphohistiocytosis in a Previously Healthy Boy: A Case Report and Literature Review / 임상소아혈액종양

A 3-year-old previously healthy boy was admitted because of a 1-week history of fever, abdominal pain, vomiting, and diarrhea. The initial laboratory tests showed hepatic dysfunction with disseminated intravascular coagulation. There was a large amount of pleural effusion, periportal edema, minimal ascites, and splenomegaly. He was initially managed with broad spectrum antibiotics with transfusion. Despite 2 days of treatment, the fever persisted and the results of the laboratory tests had worsened. Bacterial cultures from the blood, urine, pleural effusion, and ascites were all negative. He was finally diagnosed with hemophagocytic lymphohistiocytosis (HLH) based on the diagnostic criteria. Adenovirus was detected in the initial diarrhea and nasal swab specimens using polymerase chain reaction-based method. One year after chemotherapy with dexamethasone, cyclosporine, and etoposide, he is now healthy without evidence of disease recurrence. This is the first Korean case report of adenovirus-induced HLH in a previously healthy child.

A Clinical Study of Childhood Histiocytosis Syndrome / 대한소아혈액종양학회지

PURPOSE: We surveyed this study to find the factors related to clinical aspects of patients with histiocytosis syndrome. METHODS: We analyzed the clinical data of thirty patients retrospectively who were diagnosed as histiocytosis syndrome from January 1992 to December 1997 at Keimyung University Dong San Hospital. RESULTS: There were nine cases of Class I patients, twenty cases of Class II patients and one case of Class III patient. Male patients were eighteen, and female patients were twelve. Mean age at diagnosis was 4 years. The most common clinical manifestation was fever, and others were hepatosplenomegaly, pallor, respiratory symptom, and lymphadenopathy in order. Bone was involved in seven cases out of nine Class I patients. Single organ involvement happened in five cases out of Class I patients, two organ involvement happened in two patients, three or four organ involvement happened in one case of Class I patient respectively. Etiology of Class II were EBV in four patients, bacterial infection in four patients, and the others were candida, mycoplasma, mycobacterium tuberculosis. There were pancytopenia, coagulation defect, abnormal liver function tests on laboratory examinations. Most common histologic finding of Class I was proliferation and infilteration of histiocytes. Hemophagocytosis was common in bone marrow examination of Class II patients. Chemotherapy was undergone for seven patients out of nine Class I patients. Six of them showed complete remission. One of them died during chemotherapy. Thirteen patients out of twenty Class II patients are on complete remission, and five of them died. One Class III patient died during chmotherapy. CONCLUSION: The survival rate depends on age, Lahey's organ dysfunction score, severity, and sites of involved organ. One year survival rate by Kaplan-Meier method of ClassI and II patients was 87.5% and 72.2% respectively. In this study, Class II patients showed high mortality rate, so early diagnosis and treatment will be important.

Two Cases of Infection-associated Hemophagocytic Syndrome with Review of Literature / 감염

The hemophagocytic syndrome is a disorder caused by systemic proliferation of benign histiocytes with avid phagocytosis of blood cells. Although this is an established disease entity, early clinical diagnosis is often difficult. The disease is known to be rare. The clinical and laboratory characteristics of 33 patients with the infection-associated hemophagocytic syndrome were reviewed which included previously reported 31 cases from the literature, and 2 recent cases presented in this study. The patients were 22 males and 11 females with a mean age of 20.8 years(range 1 to 69 years). Ten patients were associated with viral infection and seven with bacterial infection. The infection usually occurs in patients with preexisting immunological abnormalities, but in this study underlying illness was found in only 8 out of the 33 cases. The overall mortality rate was 63.6%, which is a higher percentage than in other countries. Since the clinical course can be fulminant, accurate diagnosis and effective treatment are needed.