Objective The aim of our study was to study the CT findings of cryptococcosis in immunocompetent children.Methods CT scan and clinical data of 21 immunocompetent children with proven pulmonary cryptococcosis were retrospectively collected and analyzed.Results The CT scans demonstrated 1 mm subpleural nodule in the lingula of left lung in 1 patient and multiple nodules in 20 patients.Of 20 patients with multiple nodules,peripheral or subpleural distribution was found in 12 patients,and diffuse distribution in 8 patients.Of 20 patients with multiple nodules,Nodules of ＜ 10 mm was found in 18 patients,＜3 mm in 14 patients,and ＞ 10 mm in 2 patients.Round nodular with smooth margin was detected in 15 of 20 patients with multiple nodules.Lymphadenopathy was found in 17 patients including 3 patients with mild contrast enhancement and 2 patients with circular enhancement.Extrapulmonary lesions distributing in liver,spleen,kidney,and the nervous system were found in 14 patients.In follow-up,1 patient died and 20 patients fully recovered.ConclusionsPulmonary multiple nodules with lymphadenopathy is the characteristic CT findings in immunocompetent children with pulmonary crytococcosis which is prone to involve multiple extra-pulmonary organs.

ObjectiveTo investigate the diagnostic significance of different pathology techniques (Immunohistochemistry and ISH)to detect EBV on bone marrow biopsy tissues of hemophagocytic syndrome. Methods Histological,immunohistochemicalandinsituhybridizationwereusedtostudythe hemophagocytic features and expression of LMP-1,EBER and other markers.Results25 out of 51 cases (49.0 ％)showed an active proliferation growth pattern while other 12 cases(23.5 ％)demonstrated a deterioration morphological character compared with their same age group. The left 14 cases (27.5 ％) showed a normal myeloproliferative pattern. 91.4 ％ (32/35) expressed CD68/KP-1 and 89.5 ％ (17/19) cases expressed CD68/PG-M1, which showed an abnormal increase of monocytes. 26 out of 51 cases (51.0 ％) were positive for EBER,while EBER and immunohistochemistry on LMP-1 were detected simultaneously in 41 cases.The EBER were positive in 19 cases(46.3 ％)but none for LMP-1(P =0.000).ConclusionHypoplasia or hemophagocytic features could be detected on bone marrow biopsy tissues of hemophagocytic syndrome, which might be slightly difficult to diagnose. So immunohistochemistry would be essential. Meanwhile, the detection of EBER is more helpful than LMP-1 for diagnosis.

Objective To investigate the CT features of inflammatory myofibroblastic tumor in children. Methods Eighteen patients with inflammatory myofibroblastic tumor proven by surgery and pathology were examined with plain and contrast medium enhancement CT scan. Results Of 18 cases,16 had isolated lesions located at lung (n =4), mesentery (n =3), kidney (n =2) and trachea (n = 1 ),left main bronchus ( n = 1 ), right thoracic cavity ( n = 1 ), peritoneum cavity ( n = 1 ), pancreas ( n = 1 ),left thigh ( n = 1 ), prostate ( n = 1 ), superclvicle soft t tissue ( n = 1 ) , bladder ( n = 1 ). The other 2 cases were with multiple lesions on omentum and mesentery, and in intraperitoneal and side of split of right hepatic lobe, respectively. The CT findings of 18 cases included 16 solid mass with calcifications in 3 of them, and 2 solid-cystic mass. After contrast enhancement, moderate or marked homogeneous or heterogeneous enhancement were shown in all the solid parts of tumor on dynamic CT. Mass can compress surround great vessel and tube-like structure. On pathological examination, the tumor was mainly composed of spindleshaped fibrous cells and inflammatory cells, and the immunohistochemically staining for SMA was observed positively. Conclusion CT can provide specific information for diagnosis of inflammatory myofibroblastic tumor, yet definite diagnosis relies on pathology.

Objective To study clinical characteristics and prognostic factors in primary systemic anaplastic large cell lymphoma (S-ALCL). Methods Clinical data of 56 S-ALCL were retrospectively analysed, who were diagnosed in Lymphoma Lab of Peking University Health Science Centre. Immunohistochemical staining for ALK-1 and bcl-2 were performed by standard SP method. Results The median age of patients is 17 years, and the ratio of sex was1.67:1 (male : female) in 56 cases of S-ALCL. Among of the 49 cases who were followed up, 32.65 % (16/49) of patients died, and all of them died within two years after diagnosis. The 3-year and 5-year overall survival were 64.28 %. 41 out of 56 cases (73.21 %) was positive for ALK-1 protein, while 10 cases out of 56 S-ALCL cases (17.86 %) positive for bcl-2. Clinical staging, extranodal sites of involvement or with extranodal sites of involvement and ALK were important prognostic factors with statistic significance by Long-rank test. Among of them, Clinical staging was the most independent prognostic factor by COX multivariate analysis. Conclusion S-ALCL was mostly seen in the young and middle-aged male patients. The death were most frequently occurred within two years after diagnosis. Most of the patients who have good responses to chemotherapy can get the complete remission and long-term survival. Clinical staging, extranodal sites of involvement or with extranodal sites of involvement and ALK were very important prognostic factors which can be used to predict the patients long term survival, and guide the treatment.

Objective To investigate the chest HRCT characteristics of pediatric perinuclear anti-neutrophil cytoplasmic antibody (p-ANCA) associated systemic vasculitis. Methods Retrospectively analysis of the clinical and HRCT data of 15 pediatric cases diagnosed as p-ANCA related pneumonia according to the classification and diagnostic criteria of Chapel Hill meeting of 1994 in our hospital were retrospectively analyzed. They were 13 girls and 2 boys. The age range was 1—17 years and the median was about 10 years old, In the 15 patients, 12 were diagnosed as primary MPA, 2 were secondary MPA, 1 was CSS. Two radiologists with 10 years of radiological experience read the CT imaging together. Results 1 of 12 primary MPA were found large opacities and ground glass shades in both sides of lung,especially the posterior parts on CT images with bilateral pleural effusion; 1 case had focal opacities in left lower lobe, around with thickened interlobular septa and pleural effusion; Another 1 case only had focal thickened interstitial in right middle lobe; The other 9 cases showed scattered or diffused ground glass opacity, with thickened pulmonary interstitial and (or) pleura. 1 of the 2 secondary MPA who had hemoptysis showed ground glass opacity on CT images with no thickened septal nodules or pleural effusion. The other one who had renal anomalies and no respiratory symptoms showed subpleural nodules with halo sign in right low lobe. The CSS showed diffused patchy ground glass opacity, consolidation of both lingular lobes, nodules of centri-and peri-lobular, thickened septa near the pleura, obviously thickened bronchial wall, slightly widened bronchial lumen and thickened pleura. Conclusion The classic imaging features of p-ANCA pneumonia on HRCT is the non-specific small pulmonary vessel hemorrhage.

Objective To investigate the immunophenotype and molecular genetics of mature aggressive B-cell lymphomas in Chinese pediatric patients and provide the criteris for the diagnosis of them.Methods We collected 97 paraffin-embeded tissue samples of pediatric cases of mature aggressive B-cell lymphomas including 81 Burkitt lymphoma (BL) cases, 8 diffuse large B cell lymphoma (DLBCL) cases and 8unclassifiable B cell lymphoma with featares intermediate between BL and DLBCL (BL/DLBCL) cases. The immunophenotype and genetic features of them were detected by immunohistochemistry and interphase FISH.Results The expression of bcl-2 [3 %(2/66) in BL, 50 % (4/8) in DLBCL, 50 % (4/8) in BL/DLBCL], MUM1 [17 % (12/71) in BL, 63 % (5/8) in DLBCL, 63 % (5/8) in BL/DLBCL] and mean Ki-67 proliferation index [(93±4.4)% in BL, (83±14.3)% in DLBCL, (80±11.5)% in BL/DLBCL] were significantly different between BL and DLBCL and between BL and BL/DLBCL. The frequency of c-myc rearrangement [98 % (79/81) in BL,38 % (3/8) in DLBCL, 50 % (4/8) in BL/DLBCL] and an extra copy of bcl-6 [0 % in BL, 38 % (3/8) in DLBCL, 25 % (2/8) in BL/DLBCL] were also significantly different between BL and DLBCL and between BL and BL/DLBCL. Conclusion Diagnosis of the mature aggressive B cell lymphomas in pediatrics should be based on the comprehensive review and integration of morphologic, immunohistochemical and molecular genetic features. BL/DLBCL is more likely a subgroup of the DLBCL in pediatric population. The expression of CD10 and bcl-6 but not bcl-2, a high Ki-67 PI (＞90 %) and a c-myc rearrangement but not bcl-2 or bcl-6rearrangement are the features of BL. Regardless of the expression of CD10 and bcl-6, positive staining for bcl2, Ki-67 PI below 90 % and an extra copy of the bcl-6 favor a diagnosis of DLBCL or BL/DLBCL.

Objective To analyze the clinical characteristics and prognosis of disseminated cryptococcosis in children.Methods The data of disseminated cryptococcosis inpatients were reviewed retrospectively at Beijing Children's Hospital,Capital Medical University,from January 2002 to September 2014.The demographic data,clinical manifestations,laboratory findings,imaging,antifungal treatments and outcomes of all the patients were analyzed.Results Overall 25 children with disseminated cryptococcosis were enrolled including 17 boys(68.0％).The average age was 7 years old.Four cases (16.0％) had underlying conditions,among them 1 case had human immunodeficiency virus(HIV)-positive.The median time to diagnosis was 32 (23-47) days,44.0％ of the patients (11 cases) were misdiagnosed,and 8 cases misdiagnosed as tuberculosis.All patients had fever.Other common clinical manifestations included cough (16 cases),headache (10 cases),vomiting (10 cases),altered mental status (6 cases) and stomachache (6 cases).Respiratory system involvement was seen in all cases,central nervous system was involved in 18 cases,other organ involvement included lymph nodes,spleen,liver,kidney,skin,skeleton and costicartilage.Amphotericin B (AmB) + Fluconazole (Flu) ± 5-flucytosine (5-FC) was the most common therapy (15 cases),Flu ± 5-FC for 6 cases,AmB ± 5-FC for 2 cases,Voricanazole (VOR) for 1 case.Sixteen cases (66.7％) got recovery/improved on discharge,8 cases (33.3％) rejected to the advise and discharged with treatment failure,and the HIV-infected patient transferred to a special hospital.Fifteen patients (60.0％) were followed up,and 13 cases (87.0％) showed recovery,but 2 died in the long-term prognosis.Conclusions Disseminated cryptococcosis in children is more common in school-age boys.Most patients are without underlying conditions.Disseminated cryptococcosis can cause multiple organ damage.Patients with prolonged fever,cough,headache,with or without underlying diseases,can be suspected as disseminated cryptococcosis.Blood and cerebrospinal fluid culture for fungus,cryptococcal antigen should be performed for early diagnosis and treatment.

Objective:To analyze the clinicopathological features, treatment and prognosis of Epstein -Barr virus(EBV) positive diffuse large B cell lymphoma(DLBCL) in children, so as to improve the knowledge of pediatricians on this disease.Methods:The data of 6 cases of EBV positive DLBCL who were initially diagnosed and regularly treated in Beijing Children′s Hospital from January 2016 to December 2019 were collected, including basic information (gender, age, first symptom, and course of disease), pathological results [immunohistochemistry, EBV encoded RNA(EBER), latent membrane protein(LMP), and C- MYC gene], immune function, EBV index, treatment group, treatment plan and prognosis. Results:There were 4 males and 2 females, with the average age of 6.67 years.The uric acid was 266.2 μmol/L, lactic dehydrogenase(LDH) was 346.5 U/L at early stage, and 1 patient had immunodeficiency.The immune function test before chemotherapy indicated that the proportion of auxiliary T cells decreased in 4 cases, and the humoral immune function was normal in all patients.There was no evidence of recent infection in 6 patients, and EBV-DNA increased in 3 patients.There were 2 cases of stage Ⅲ, 4 cases of stage Ⅳ, 1 case of giant tumor, 2 cases of symptom B, 6 cases of extranodal invasion, 4 cases of central invasion and 1 case of bone marrow invasion.Three patients died and three survived.Immunohistochemistry showed that: (1) CD 19, CD 20, and CD 79a were expressed in all patients, and CD 30 was expressed in 5 patients.(2) C- MYC gene was detected by immunofluorescence in situ hybridization method in all patients, and no MYC break, Bcl-2 and Bcl-6 break and amplification were found.(3) EBV: EBER and LMP-1 were expressed in all patients. Conclusions:The pathological changes of EBV positive DLBCL are similar to those adults.The origin of non-germinal center and extranodal and central invasion are more common.The prognosis of the patients with central nervous system invasion is very poor, and the recurrence and progress of the disease often occur in the treatment or in the early stage of drug withdrawal.At present, there is no effective and feasible treatment plan.It is suggested that the patients in the late stage should receive allogeneic hematopoietic stem cell transplantation as soon as possible after intensive treatment, so as to improve the survival rate.

Objective To investigate the molecular genetic changes of anaplastic lymphoma kinase (ALK) gene and c-myc gene in anaplastic large cell lymphoma (ALCL). Methods The structural aberrations and changes of copy numbers in ALK and c-myc genes in 72 paraffin-embedded ALCL specimens were detected by interphase fluorescence in situ hybridization (FISH). Results Among 72 ALCL specimens, ALK protein was expressed in 42, ALK gene translocation was detected in 40 specimens in which extra copies of ALK gene were detected in 17. ALK gene translocation was not found in all 30 ALK negative specimens, but extra copies of ALK gene were detected in 14 cases. The difference of incidence rates of extra copies in ALK gene between ALK positive and ALK negative specimens was not significant (P＞0.05). c-myc gene translocation was not found in any of 72 ALCL specimens, but extra copies were detected in 24 cases.Conclusion Most (75.0%) ALCL have ALK gene aberration, in which ALK gene translocations are most common (55.6%), and the extra copies of ALK gene are relatively common genetic changes (43.1%). The ALK gene aberration is only detected in ALK positive ALCL and the gene translocations are in either ALK positive and negative ALCL. There is no or rare c-myc gene translocation in ALCL, but extra copies of c-myc gene are relatively common (33.3%).

Objective To study the patients' clinical characteristics and prognosis when only C3 deposition exists in endocapillary proliferative glomerulonephritis and try to understand deeply the role of C3 in kidney damage deeply. Methods The patients who were diagnosed with endocapillary proliferative glomerulonephritis but only had C3 deposited in immunofluorescence(to avoid false positive,C3≥2 ﹢ was included)were selected from Beijing Children's Hospital Affiliated to Capital Medical University during November 2010 to October 2014. Their clinical manifestations,la-boratory examinations,treatments,prognosis,and pathological changes were analyzed,and literature review was performed. Their clinical characteristics and prognosis were summarized. Results There were 11 patients diagnosed with endocapil-lary proliferative glomerulonephritis which had only C3 deposition(≥2 ﹢ ). Nine of them had onset with acute nephritis syndrome(81. 8% ),and 2 cases presented recurrent paroxysmal gross hematuria(18. 2% ). Seven cases were diagnosed with acute post streptococcal glomerulonephritis(63. 6% ). Eleven cases' clinical manifestations were relatively severe, and the complement C3 was significantly lower than the normal(100. 0% ). Their light microscope showed capillary proli-ferative glomerulonephritis,and the electron microscope showed the immune complexes were deposited in the endothelium,the epithelium or the mesangial area. The patients received corresponding treatment respectively,and all the patients had good prognosis during following up of 7 months up to 39 months. Conclusions Streptococcus infection is a common cause in endocapillary proliferative glomerulonephritis with only C3 deposition. The clinical manifestations of some children are similar to post streptococcal glomerulonephritis but relatively severe. Only deposition of C3 without IgG may be involved in another complement activation mechanism.