Neutrophil and monocyte adhesion molecules in bronchopulmonary dysplasia, and effects of corticosteroids

Aims: To study a longitudinal change in the expression of adhesion molecules CD11b, CD18, and CD62L on neutrophils and monocytes in very low birth weight babies who develop respiratory distress syndrome, to compare these levels between bronchopulmonary dysplasia (BPD) and non-BPD infants, and to assess the effect of corticosteroid treatment on these adhesion molecules. Methods: Of 40 eligible neonates, 11 neonates were oxygen dependent at 36 weeks (BPD 36 weeks), 16 infants were oxygen dependent at 28 days, but not at 36 weeks (BPD d28), and 13 infants did not develop BPD. Seventeen neonates received a six day course of steroid treatment. Expression of CD11b, CD18, and CD62L was measured on neutrophils and monocytes in arterial blood on days 1, 3, 7, 14, 21, and 28, and before and 2–3 days after initiation of dexamethasone treatment by flow cytometry. Results: CD18 expression on neutrophils and monocytes and CD62L on neutrophils, measured as mean fluorescent intensity, was significantly decreased in BPD neonates compared to non-BPD neonates on days 1–28. Dexamethasone treatment significantly decreased CD11b, CD18, and CD62L expression on neutrophils, and CD11b and CD18L expression on monocytes. Conclusions: Decreased CD18 expression on neutrophils and monocytes, and decreased CD62L expression on neutrophils, measured as mean fluorescent intensity during the first four weeks of life in micropremies may be risk factors and early predictors of BPD. Dexamethasone use was associated with decreased expression of CD11b, CD18, and CD62L.     

Expression of very late antigen-4 and lymphocyte function-associated antigen-1 on peripheral blood lymphocytes from patients with graves disease

The aim of the study was to estimate the expression of beta1 integrin CD49d (very late antigen-4), beta2 integrin CD11a (lymphocyte function-associated antigen 1 alpha), L-selectin (CD62L), and intercellular adhesion molecule-1 [ICAM-1 (CD54)] on peripheral blood mononuclear cells in patients with Graves disease (GD) (n = 45, mean age 15.9 +/- 5.9 y), in patients with nontoxic nodular goiter (NTNG) (n = 25, mean age 15.2 +/- 5.7 y), and in sex- and age-matched healthy control subjects (n = 25, mean age 15.9 +/- 2.4 y). The expression of the lymphocyte adhesion molecules was analyzed by the three-color flow cytometry. Decreased percentages of CD49d+ and CD11a+ lymphocytes were observed in untreated Graves' patients in comparison to healthy controls (p < 0.007, p < 0.036) and non-toxic nodular goiter patients (p < 0.006, p < 0.019). The analysis of CD62L+ and CD54+ antigen expression on peripheral blood lymphocytes revealed increased percentages of L-selectin- and ICAM-1-positive cells in patients with GD (p < 0.005 for CD62L, p < 0.008 for ICAM-1) before methimazole therapy, compared with healthy controls. These abnormalities were absent in children with NTNG. In patients with untreated GD, a positive correlation was found between serum levels of free thyroxine and the percentage of CD54+ lymphocytes (r = 0.47, p < 0.036). Statistically significant negative correlations existed between free thyroxine concentration in blood serum and the percentage of CD11a+ lymphocytes (r = -0.39, p < 0.033). No such correlation was detected between the percentage of lymphocyte markers and serum levels of antithyroid antibodies. We conclude that the alterations in the expression of adhesion molecules on peripheral blood mononuclear cells might suggest their role in the development of GD.     

Epigenetic changes in the DAP-kinase CpG island in pediatric lymphoma

BACKGROUND: Hypermethylation of CpG islands in the promoter region of death-associated protein kinase (DAP-kinase) coupled with the loss of gamma-interferon-induced apoptosis have been reported in B-cell malignancies suggesting a role in pathogenesis or prognosis. Along with B-cell malignancies, pediatric lymphomas also include T-cell non-Hodgkin lymphoma (NHL), anaplastic large cell lymphoma, and Hodgkin disease, each with unique prognoses. The purpose of this study was to elucidate epigenetic changes in the DAP-kinase promoter region of pediatric cases to determine associations with aberrant hypermethylation. PROCEDURES: Thirty-nine cases of different lymphoid pathology [10 Burkitt lymphoma, 1 B-cell NHL; 7 T-cell lymphoblastic lymphoma; 4 anaplastic large cell lymphoma (LCL); 2 B-cell LCL; 14 nodular sclerosing Hodgkin disease (NSHD); and 1 B-cell acute lymphoblastic leukemia (ALL)] had methylation-specific polymerase chain reaction performed on bisulfite-treated DNA, which distinguishes the methylation status of the promoter region, and DAP-kinase mRNA expression assays performed on available specimens. RESULTS: In normal lymphocytes, the CpG islands in the promoter region were unmethylated, as were the T-cell lymphoblastic lymphoma and anaplastic LCL. In contrast, 100% of the Burkitt lymphoma (10/10) and B-cell ALL (1/1) were hypermethylated. Of the specimens with mRNA available, 7/8 Burkitt lymphoma had no DAP-kinase mRNA expression compared to normal expression in 3/3 and 4/4 T-cell lymphoblastic lymphoma and NSHD, respectively. CONCLUSIONS: In these pediatric lymphoid tumors, hypermethylation of the DAP-kinase promoter region with associated loss of DAP-kinase gene expression was associated with B-cell malignancies and thus may be important in the development and/or provide a prognostic tool in B- cell lymphomas.     

Ki-1 positive (anaplastic, large cell) lymphoma (case reports and review).

Ki-1 positive (anaplastic, large cell) lymphoma is a subgroup of non-Hodgkin lymphomas identified recently by Ki-1 (or BER-H2) (CD 30) monoclonal antibody. The clinicopathological features of two such pediatric cases of lymph node origin described here, and also the available literature emphasize the heterogenous nature of Ki-1 positive lymphomas, in almost every respect. Nevertheless, the Ki-1 antibody serves as an important diagnostic tool to differentiate lymphomas from other anaplastic, large malignancies.     

A unique case of adolescent CD56-negative extranodal NK/T-cell lymphoma, nasal type.

Mature T-cell neoplasms are unusual in the pediatric population. The majority of these neoplasms in the United States are anaplastic large cell lymphomas (ALCL) characterized by CD30 and anaplastic lymphoma kinase-1 expression. Extranodal natural killer/T (NK/T)-cell lymphomas, nasal type, are extremely rare. Extranodal NK/T-cell lymphomas often express CD56, are associated with Epstein-Barr virus, and are negative for CD30. Clinically, extranodal NK/T-cell lymphomas are much more aggressive than ALCL, and require different treatment strategies. The authors present an adolescent male with a CD56 negative extranodal NK/T-cell lymphoma, nasal type. The lymphoma was partially positive for CD30, diffusely positive for EBV by in situ hybridization, and clonal for T-cell receptor gene rearrangement and cytogenetic abnormalities. The patient was aggressively treated with chemotherapy, surgery, and radiation. More than 2 years from completion of the therapy, the patient remains disease free. This case highlights the importance and difficulty of accurate identification of this type of rare tumor. We further present the literature review and discuss the diagnostic criteria for extranodal NK/T lymphoma using morphologic, immunologic, molecular, and cytogenetic information.     

粘附分子CD62P和CD44在毛细支气管炎患儿外周血中的表达及意义

目的 探讨黏附分子CD62P和CD44在毛细支气管炎(简称毛支炎)患儿外周血中的表达及意义。方法 选取2014 年11 月至2015 年5 月住院治疗的毛支炎发病期患儿33例和恢复期患儿19例为研究对象,同期选取支气管肺炎患儿30例为支气管肺炎组,非感染患儿26例为对照组。采用流式细胞术检测各组患儿外周血CD62P的表达百分比,ELISA法测定血清CD44的水平。结果 毛支炎发病期组CD62P和CD44水平显著高于毛支炎恢复期组、支气管肺炎组及对照组(PPr=0.91,P结论 黏附分子CD62P、CD44参与了毛细支气管炎的发病过程,其水平高低可反映毛细支气管炎炎症反应的严重程度。     

State of the Art and Future Needs in Cytogenetic/Molecular Genetics/Arrays in childhood lymphoma: summary report of workshop at the First International Symposium on childhood and adolescent non-Hodgkin lymphoma, April 9, 2003, New York City, NY

BACKGROUND: A significant number of studies describe the cytogenetics and molecular genetics of adult non-Hodgkin lymphoma (NHL); however, similar knowledge is lacking regarding pediatric NHL. METHODS: A workshop to discuss the "State of the Art and Future Needs in Cytogenetic/Molecular Genetics/Arrays" in pediatric NHL was held in conjunction with the First International Symposium on Childhood and Adolescent Non-Hodgkin Lymphoma on April 9, 2003 in New York City. RESULTS: Cytogenetic characteristics of pediatric NHL include 14q11.2 rearrangements in T-cell lymphoblastic leukemia/lymphomas (LBL), ALK rearrangements in anaplastic large cell lymphomas (ALCL), and CMYC translocations in both Burkitt and Burkitt-like lymphomas (BL/BLL). Pediatric diffuse large B-cell lymphoma (DLBCL) is cytogenetically different from DLBCL in adults, suggesting a different disease in children. Microarray studies demonstrate three types of T-cell leukemia, the leukemic counterpart of LBL, that block T-cell differentiation at different stages of T-cell development, corresponding to LYL, TAL1, and HOX-expressing leukemias. ALCL cell lines have a unique expression profile compared to normal T-cells. Germinal centers of BL have CMYC expression signatures, indicating that CMYC expression is ectopic and does not reflect the physiology of the normal cell counterpart. CONCLUSIONS: Additional cytogenetic, molecular and microarray investigations of NHL in children are vital to better understand these diseases, their etiology, and differences from adult NHL. A greater understanding of pediatric NHL will lead to disease-specific and patient-individualized therapies of these diseases.     

CD54、CD40和B7-1分子在桥本甲状腺炎患儿甲状腺组织中的表达

目的探讨T细胞共刺激分子CD54、CD40和B7_1在儿童桥本甲状腺炎发病中的作用。方法采用免疫组化(HRP)法观察11例儿童桥本甲状腺炎(HT)和11例非毒性结节性甲状腺肿(NTG)甲状腺组织的T细胞共刺激分子CD54、CD40和B7_1的表达,应用Mias99图像分析系统进行定量分析。结果CD54、CD40和B7_1分子在所有HT患儿的甲状腺组织中均呈强阳性表达;而在NTG的甲状腺组织几乎不着色;其阳性颗粒面积、平均吸光度及积分吸光度值,在HT组明显高于NTG组(P均<0.01)。结论T细胞共刺激分子CD54、CD40和B7_1的异常表达与儿童自身免疫性甲状腺炎的发病有关,并可扩大和加重免疫损伤。     

No Influence of bcl-2, p53, and p21waf1 protein expression on the outcome of pediatric Hodgkin lymphomas

In Argentina, lymphomas account for 13.6% of all pediatric tumors and 47% of them are Hodgkin lymphoma. Previous studies of lymphoma series have reported the expression of apoptotic and cell cycle proteins. Our aim was to study these markers in our pediatric patients and correlate them with their outcome. Immunohistochemical staining with monoclonal antibodies anti-p53, bcl-2, p21, and mdm2 were performed on formalin-fixed paraffin-embedded Hodgkin lymphoma lymph node biopsies from 54 pediatric patients. The analyzed oncogenes p53, bcl-2, p21, and mdm2 exhibited 81%, 44%, 76%, and 90% positive staining, respectively. The most prevalent p53/p21 expression pattern was p53+/p21+, in 57% of cases, whereas concerning p53/mdm2 expression pattern p53+/mdm2+ was observed in 61% of cases. We failed to find any statistically significant correlation between oncogene expression and patient's survival. It seems that p53 plays an important role in lymphomagenesis in our studied population, because it is overexpressed in 81% of Hodgkin lymphoma cases and in more than 50% of cases, it might be able to activate its cellular effectors. Bcl-2 staining observed in 44% of our cases could represent a failure in bcl-2 down-regulation that leads to a rescue event in defective germinal center B-cells, that allows them to develop into Reed-Sternberg and Hodgkin cells.     

Serum levels and differential expression of CD44 in childhood leukemia and malignant lymphoma: Correlation with prognostic criteria and survival

BACKGROUND: The CD44, a cell surface proteoglycan, participates in a variety of function including tumor dissemination and metastasis. However, there are no available data on the prognostic significance of CD44 expression of tumor tissue correlated with serum sCD44 level in childhood leukemias and lymphomas. METHODS: Serum levels and leukemic cell tumor tissue expression of CD44 were detected in 54 children with acute leukemia and malignant lymphoma. Serum samples were obtained from all patients before treatment and during remission. Twelve age-matched healthy children were included as a control group. RESULTS: The serum CD44 levels were significantly higher in patients with Hodgkin's disease (HD), non-Hodgkin's lymphoma (NHL), Burkitt's lymphoma (BL) and acute lymphoblastic leukemia (ALL) than those in the control group. The median values were 1627.0, 1336.0, 1318.5, 1730.4, 902.7 ng/mL, respectively, and P<0.001, P<0.01, P<0.01, P<0.05 in comparisons, respectively. However, there was no significant difference between acute myeloid leukemia (AML) and the control group (median values: 900.3 and 902.7 ng/mL, respectively, P>0.05). Serum sCD44 levels significantly declined in HD, NHL and ALL patients who were in complete remission (median values: 684.0, 573.8 and 1101.1 ng/mL, respectively, P<0.05 in each comparison). Patients with HD had higher levels of serum sCD44 and correlated well with higher erythrocyte sedimentation rate (ESR), B-symptoms and advanced-stage disease (P<0.05, P<0.05 and P<0.01, respectively). Expression of CD44 was significantly high in patients with HD and NHL who were in advanced stages of disease. High serum CD44 level was also associated with high tumor tissue expression of CD44 in patients with HD and BL. In addition, patients with higher levels of serum sCD44, had a poorer outcome and survival than those with lower sCD44 levels in HD and NHL groups. CONCLUSIONS: A high serum sCD44 level and/or tumor tissue expression at diagnosis is associated with poor prognostic criteria and/or unfavorable outcome in childhood leukemias and lymphomas.     

小儿原发性胃肠道非霍奇金淋巴瘤的临床病理分析

目的探讨小儿原发性胃肠道非霍奇金淋巴瘤（primary gastrointestinallymphoma,PGIL）的临床病理特征及免疫表型。方法对10例小儿PGIL采用sP法行CD45、CD3、CD3e、CD5、CD10、CD15、CD20、CD30、CD45R01CD56、CD68,CD99、Bcl-2、Bcl-6、ALK、CyelinD1、Mum-1、MPO、PAX-5、TdT、TIA-1、Ki-67及细胞角蛋白（CK）免疫组织化学染色,运用原位杂交检测Epstein—Barr病毒编码的早期RNA（EBER）,按2008年WHO淋巴造血系统肿瘤分类标准进行分类。结果①10例瘤细胞均表达CIM5、Ki-67,无一例表达CK;10例均经病理、免疫组化及原位杂交检测等确诊为不同类型PGIL;②临床表现为腹痛、腹泻、腹部包块、发热、贫血和消瘦等。发生在小肠5例、回盲部2例、结肠1例、直肠2例。6例为溃疡型,4例为隆起型;③B细胞性NHL7例,其中伯基特淋巴瘤5例,B淋巴母细胞性淋巴瘤1例,弥漫性大B细胞淋巴瘤1例;T细胞NHL3例,其中NK／T细胞淋巴瘤1例,肠病相关T细胞淋巴瘤1例,ALK阳性间变性大细胞淋巴瘤1例。结论小儿PGIL临床症状无特异性,以B细胞淋巴瘤多见,T细胞淋巴瘤少见,预后差,须与其他胃肠道恶性肿瘤相鉴别。     

肺炎患儿外周血白细胞表面粘附分子CD11b、CD54、CD62L表达的研究

目的 探讨肺炎患儿外周血白细胞表面CD11b、CD54、CD62L在发病中的作用及其与病情的关系。方法 采用全血直接免疫荧光流式细胞术检测34例肺料患儿及22例正常小儿外周血白细胞粘附分子（AMS）CD11b、CD54、CD62L的表达。结果 1．肺炎组与对照组比较：淋巴细胞（L）、单核细胞（M）、多形核细胞（PMN）表面CD11b、CD54、CD62L的阳性细胞百分率（PPC）和（或）平均荧光强度（MFI）普遍上调（P＜0．05或＜0．0001），其中以M的AMS牙调最为显著；2．重型肺料组与轻型组比较；PGE表面CD11b、L表面CD62L的PPC和（或）MFI表达上调，3类白细胞表面CD54的PPC和MFI表达均显著上调（P＜0．05或P＜0．001）。结论 1．AMS在肺炎的发病中发挥了重要作用；在三类白细胞表面CD54表达均同时上调的水平有可能作为判断肺炎病情程度的免疫学指标；3．此结果可能为肺炎的抗粘附疗法提供了一些依据。     

CD147和基质金属蛋白酶9在非霍奇金淋巴瘤中的表达及其与预后的关系

目的 探讨CD147和基质金属蛋白酶9(MMP-9)在非霍奇金淋巴瘤(NHL)中的表达,分析表达水平与临床分期、肿块大小、血清乳酸脱氢酶(LDH)水平及与预后的关系.方法采用免疫组化方法研究病理标本中CD147、MMP-9的表达及其与临床指标之间的关系,探讨两者的相关性及其与NHL预后的关系.结果 ①免疫组化结果 :73%病例(45/62)CD147表达阳性,其中(+)11例,(++)13例,(+++)21例,阴性表达17例;81%病例(50/62)MMP-9表达阳性,其中(+)13例,(++)18例,(+++)19例,阴性表达12例;CD147与MMP-9的表达有明显相关性.②CD147的表达与临床骨髓浸润、肿块大小、LDH值以及临床分期有关;MMP-9的表达与骨髓浸润和临床分期有关.③CD147(-)～(+)组5年生存率明显高于(++)～(+++)组,同样MMP-9(-)～(+)组5年存活率显著高于(++)～(+++)组.Cox多因素风险分析提示CD147和MMP-9都是影响预后的重要因素.结论 CD147和MMP-9的表达与NHL的预后相关,高表达者预后不良.     

儿童肝脾γδT细胞淋巴瘤的临床病理分析

目的探讨儿童肝脾γδT细胞淋巴瘤的临床病理和免疫表型特征。方法应用组织病理和免疫组化方法分析2例儿童肝脾γδT细胞淋巴瘤的骨髓和肝脏活检标本,并对相关文献进行调研。结果本文报道的2例肝脾γδT细胞淋巴瘤为9岁女孩,主要表现发热、肝脾肿大和血细胞减少,无淋巴结肿大。其中1例合并慢性活动性EB病毒感染和噬血细胞综合征,诊断时有骨髓受累。2例肝脏针刺活检病理均示中等大小非典型淋巴细胞的窦内浸润,免疫组化示CD3+CD4-CD8-Granzyme B+EBER-。结论肝脾γδT细胞淋巴瘤是一类罕见的结外侵袭性外周T细胞淋巴瘤,典型特征包括:全身症状,肝脾肿大,血细胞减少,肝脾和骨髓的窦内有CD2+CD3+CD4-CD5-CD7+CD8-TCRγδ+表型淋巴细胞的浸润,7号染色体长臂等臂畸形和8号染色体三体同时出现的细胞遗传学改变。骨髓或肝脏活检组织的免疫表型和TCR受体分析有助于此型肿瘤的诊断,目前尚无有效治疗方法。     

Improvement in complete response rate,duration of response and survival with adriamycin combination chemotherapy for non-Hodgkin lymphoma: A prognostic factor comparison of two regimens

The results of a chemotherapy regimen utilizing adriamycin in combination with vincristine, prednisone plus or minus cyclophosphamide (CHOP-HOP) for the treatment of non-Hodgkin lymphoma are compared to those of a non-adriamycin containing combination (COP). The complete remission (CR) rate of 67% for CHOP-HOP in malignant lymphoma, diffuse histiocytic type (MLDH) is superior to 33% obtained with COP. In malignant lymphoma, nodular poorly differentiated lymphocytic (MLNPD), the CR rate was: CHOP-HOP 83%, COP 44%. The prognostic factors for both treatment groups were compared and found to be similar. Thus, adriamycin appears to be responsible for this improvement in CR rate. When analyzed as a group, the patients treated with CHOP-HOP had a longer survival than those treated with COP. This difference was also evident in the subgroup of patients with MLDH. The smaller subgroup of patients with MLNPD treated with CHOP-HOP did not show a significant improvement in survival. A trend for longer duration of CR in both diffuse and nodular lymphomas was also observed, but this difference did not reach statistical significance possibly due to the smaller numbers of patients. Combination chemotherapy with adriamycin has resulted in a higher CR rate and survival for patients with non-Hodgkin lymphoma particularly for the subgroup of patients with MLDH.     

Improvement in complete response rate, duration of response and survival with adriamycin combination chemotherapy for non-Hodgkin lymphoma: a prognostic factor comparison of two regimens.

The results of a chemotherapy regimen utilizing adriamycin in combination with vincristine, prednisone plus or minus cyclophosphamide (CHOP-HOP) for the treatment of non-Hodgkin lymphoma are compared to those of a non-adriamycin containing combination (COP). The complete remission (CR) rate of 67% for CHOP-HOP in malignant lymphoma, diffuse histiocytic type (MLDH) is superior to 33% obtained with COP. In malignant lymphoma, nodular poorly differentiated lymphocytic (MLNPD), the CR rate was: CHOP-HOP 83%, COP 44%. The prognostic factors for both treatment groups were compared and found to be similar. Thus, adriamycin appears to be responsible for this improvement in CR rate. When analyzed as a group, the patients treated with CHOP-HOP had a longer survival than those treated with COP. This difference was also evident in the subgroup of patients with MLDH. The smaller subgroup of patients with MLNPD treated with CHOP-HOP did not show a significant improvement in survival. A trend for longer duration of CR in both diffuse and nodular lymphomas was also observed, but this difference did not reach statistical significance possibly due to the smaller numbers of patients. Combination chemotherapy with adriamycin has resulted in a higher CR rate and survival for patients with non-Hodgkin lymphoma particularly for the subgroup of patients with MLDH.     

CD95 (APO-1/Fas) expression on naive CD4(+) T cells increases with disease progression in HIV-infected children and adolescents: effect of highly active antiretroviral therapy (HAART)

We studied the expression of the CD95 receptor (APO-1/Fas) on peripheral blood T cell subpopulations in 37 HIV-1-infected children and adolescents stratified according to disease stage or antiretroviral treatment regimen and compared the results to values obtained in 12 healthy age-matched control subjects. CD95 expression on CD45RA(+) CD45RO(-)/CD62L(+) (resting/naive) and CD45RO(+) CD45RA(-) (primed/memory) CD4(+) and CD8(+) T cells was assessed quantitatively by four-color and three-color flow cytometry. CD4(+) T cells contained a population of predominantly CD95(-) resting/naive cells and a population of CD95(high) primed/memory cells, whereas CD8(+) T cells had a more uniform pattern of CD95 expression. The percentage of CD95(+) CD4(+) T cells increased with disease progression because of both an augmented median fluorescence intensity on resting/na?ve cells and an increased percentage of CD95(high) cells. Patients with highly active antiretroviral combination therapy who maintained stable CD4 counts in the presence of elevated plasma viral load had nearly normal numbers of CD95(-) resting/naive CD4(+) T cells, whereas CD95 expression in the CD8(+) T cell subset was still elevated compared with control subjects. Low CD95 expression on resting/naive CD4(+) T cells may therefore indicate a low risk for disease progression in antiretrovirally treated and untreated patients.     

IFR4/MUM1‐positive lymphoma in Waldeyer ring with co‐expression of CD5 and CD10

IRF4/MUM1‐positive lymphoma is a new subgroup of germinal center‐derived B‐cell lymphoma, predominantly involving the Waldeyer ring (WR) in children. CD5 expression is rare in these lymphomas. We report a 7‐year‐old Chinese male with B‐cell lymphoma. Evaluation of his specimen by morphology, immunohistochemistry, and FISH analysis demonstrated IRF4/MUM1‐positive lymphoma with strong and extensive CD5 and CD10 positivity. Despite the lack of t(14;18)(q32;q21) rearrangement, BCL2 protein was expressed. Our report highlights the clinicopathologic features of IFR4/MUM1‐positive lymphoma in WR with co‐expression of CD5 and CD10, and thereby provides insight into this newly recognized disease entity.     

Spectrum and presentation of pediatric malignancies in the HIV era: experience from Blantyre, Malawi, 1998-2003

BACKGROUND: Data on childhood cancers in Africa are sparse, particularly since the spread of HIV. We aimed to document the frequency of pediatric cancers presenting to a large central hospital in Malawi, detailing the presenting features, initial investigations, and HIV status of these children. PROCEDURE: A retrospective audit of the spectrum and clinical presentation of cancers among children (<16 years) seen at Queen Elizabeth's Central Hospital (QECH), between 1998 and 2003. RESULTS: Seven hundred seven children with cancer were seen, the number of cases per year increased over the time period; 50% (351) had Burkitt lymphoma, 13% (89) had retinoblastoma, and 9% (61) had Kaposi sarcoma, with a variety of other tumors comprising the remainder. Kaposi sarcoma markedly increased in frequency over time. Histological verification of diagnosis was available for 49% (348). The proportion of children with cancer who were tested for HIV increased over time, but varied by cancer type. Amongst those tested, the seroprevalence was 93% (52/56) for children with Kaposi sarcoma, 4% (11/289) for those with Burkitt lymphoma, 31% (8/26) for those with other non-Hodgkin lymphomas, 7% (1/15) for those with Hodgkin disease, and 5% (5/103) for those with other cancers. CONCLUSIONS: The number of cases seen per year has increased over the study period for almost all cancers, but in particular for Kaposi sarcoma. Burkitt lymphoma remains the commonest pediatric tumor in Malawi. In the case of Burkitt lymphoma, non-Hodgkin lymphoma, and Kaposi sarcoma there is a significant difference in the presentation of HIV-seropositive and -seronegative children.     

Mature B-cell lymphoma in children and adolescents: International group pathologist consensus correlates with histology technical quality

In pediatric mature B-cell non-Hodgkin lymphoma, international pathologist diagnostic agreement was previously evaluated using the Revised European-American Lymphoma Classification. Surgical biopsy histology technical quality (HTQ) is variable and may affect diagnostic accuracy. This study evaluated diagnostic agreement correlated with HTQ. Surgical biopsies obtained from international protocol FAB LMB96 Treatment of Mature B-Cell Lymphoma/Leukemia for Burkitt lymphoma (BL), diffuse large B-cell lymphoma (DLBCL), and high-grade B-cell lymphoma Burkitt-like (BLL), were independently reviewed by hematopathologists from 3 national groups (Children's Cancer Group, Société Fran?aise d'Oncologie Pédiatrique, and United Kingdom Children's Cancer Study Group) to determine each national diagnosis and a final diagnosis. HTQ grades for microscopic tissue sections included: good; medium; low; inconclusive. Final diagnoses in 187 cases included: BL 87 (47%); BLL 20 (11%); DLBCL 64 (34%); other 16 (9%). HTQ grades included: good 10 (5%); medium 100 (54%); low 75 (40%); inconclusive 2 (1%). The rate of uniform agreement between the national diagnoses was significantly higher with good or medium HTQ (62%) than with low HTQ (33%) (P = 0.001). In conclusion, in pediatric mature B-cell non-Hodgkin lymphoma, international pathologist diagnostic agreement is significantly higher in surgical biopsies with better HTQ. Poor HTQ may adversely impact diagnostic ability and affect prognosis and therapeutic management when different treatment regimens are employed for DLBCL versus BL/BLL.