Improved prognosis of Epstein-Barr virus associated childhood Hodgkin's lymphoma: study of 47 South African cases

Engel, Mark E; Essop, M. Faadiel; Close, Pauline; Hartley, Patricia; Pallesen, Gorm; Sinclair-Smith, C

doi:10.1136/jcp.53.3.182

Cited by 63 publications

(47 citation statements)

References 36 publications

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“…A more recent study of adult patients from India also showed that 10-year relapse-free survival was significantly higher for EBV-positive HD patients when compared to the EBV-negative group (Naresh et al, 2000). A second smaller study from South Africa of 36 children with HD showed that EBV-positive HD was associated with longer median survival times (Engel et al, 2000).…”

Section: Discussionmentioning

confidence: 95%

Assessment of the prognostic impact of the Epstein–Barr virus-encoded latent membrane protein-1 expression in Hodgkin’s disease

et al. 2001

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SummaryWe have examined expression of the Epstein-Barr virus (EBV) latent membrane protein-1 (LMP1) in the malignant Hodgkin and Reed-Sternberg (HRS) cells of Hodgkin's disease (HD) and its impact on response to treatment and on survival. Paraffin tissue from 100 adult immunocompetent patients with HD were analysed using immunohistochemistry to identify LMP1 expression. According to the Rye classification, 8% of patients had lymphocyte predominance (LP) subtype, 48% had nodular sclerosis (NS) disease, 37% were of the mixed cellularity (MC) subtype and 7% were of the lymphocyte depletion (LD) subtype. During the five year follow-up period 27 patients died and 74 patients achieved a complete remission. Patients with LD subtype were older (P = 0.03), less frequently achieved complete remission (P = 0.01), had shorter disease-free survival (P = 0.01) and overall survival (P = 0.002) compared with the other subtypes of HD. LMP1 expression was found in the tumour cells of 26% of cases of HD. LMP1 expression was less common in NS disease than in the other subtypes (P = 0.05), whereas an association between MC subtype and LMP1 expression was not found (P = 0.22). Using the log-rank test there were no differences in overall survival or disease-free survival based on EBV status either when all patients were analysed or when LD and LP subtypes were excluded. However, the presence of EBV was associated with significantly longer disease-free survival in the subgroup of patients ≤ 30 years old (P = 0.02) and in those patients ≤ 34 years old (P = 0.05). In contrast, there was a trend for shorter disease-free survival for EBV-positive patients in the subgroup > 35 years old, but this difference was not statistically significant (P = 0.11). A similar trend was observed in patients > 50 years old. Analysis of the impact of LMP1 expression in patients who had different stage and B symptoms status showed that expression of EBV was associated with longer disease-free survival (P = 0.019) in early stage (1 + 2) patients without B symptoms. Significant differences in the other subgroups based on EBV status was not found. Factors adversely affecting the likelihood to achieve a complete remission were: absence of LMP1 expression (OR 6.4, 95% Cl 1.07-38.5, P = 0.04), age (OR 1.68, 95%Cl 1.15-2.5, P = 0.007) and subtype of HD (OR 3.32, 95%Cl 1.11-9.94, P = 0.03). Age and subtype of HD had an independent impact on overall survival (P = 0.01). We conclude that expression of LMP1 in HRS cells has a favourable impact on prognosis for HD patients, but that this effect may be restricted to young adult patients and those with early stage disease.

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Section: Discussionmentioning

confidence: 95%

Assessment of the prognostic impact of the Epstein–Barr virus-encoded latent membrane protein-1 expression in Hodgkin’s disease

et al. 2001

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“…In 47 South African children aged 3-14 years with classical HL, 68% were EBV positive and they presented with less aggressive symptoms and had longer median survival rates than EBV negative patients. (Engel et al, 2000).…”

Section: Ebv and Hodgkin Lymphomamentioning

confidence: 99%

Haematological cancers in African children: progress and challenges

et al. 2017

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SummaryCancer is increasingly important in low and middle-income settings where infectious diseases are declining. Childhood cancers treated in well-resourced centres have excellent outcomes with more than 80% survival. This success is not reflected in low-income settings where challenges involve every step on the care pathway. Access to diagnosis, delayed presentation, advanced disease, co-morbidities and underlying malnutrition make treatment difficult. Treatments are costly for impoverished families. Yet, the common haematological malignancies (Burkitt lymphoma, Hodgkin lymphoma, non Hodgkin lymphoma) are relatively easy to diagnose and, when managed with simple chemotherapy protocols, give limited but good results. As funding becomes available for cancer research we must ensure that the care and cure of these children is top of the agenda. There is already evidence of improved outcomes in middle-income countries. For others there is a long journey ahead.

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“…[1][2][3] The impact of the tumor-cell EBV status on the prognosis of patients with HL remains controversial. Most previous studies have not reported a significant association between EBV status and outcome, [4][5][6][7][8][9][10][11] while others have found that EBV-associated cases have either a more or less favorable prognosis [12][13][14][15][16][17][18] (Table 1). Some of the interstudy variation may be attributable to the different epidemiologic features of the disease in different geographic settings and some may be related to case selection.…”

Section: Introductionmentioning

confidence: 99%

Impact of tumor Epstein-Barr virus status on presenting features and outcome in age-defined subgroups of patients with classic Hodgkin lymphoma: a population-based study

Jarrett

Stark

White

et al. 2005

Blood

178

131

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The association between tumor EpsteinBarr virus (EBV) status and clinical outcome in Hodgkin lymphoma (HL) is controversial. This population-based study assessed the impact of EBV status on survival in age-stratified cohorts of adults with classic HL (cHL). Data from 437 cases were analyzed with a median follow-up of 93 months. Overall survival (OS) was significantly better for EBVnegative compared with EBV-positive patients (P < .001), with 5-year survival rates of 81% and 66%, respectively; diseasespecific survival (DSS) was also greater for EBV-negative patients (P ‫؍‬ .03). The impact of EBV status varied with age at diagnosis. In patients aged 16 to 34 years, EBV-associated cases had a survival advantage compared with EBV-negative cases, but differences were not statistically significant (P ‫؍‬ .21). Among patients 50 years or older, EBV positivity was associated with a significantly poorer outcome (P ‫؍‬ .

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Improved prognosis of Epstein-Barr virus associated childhood Hodgkin's lymphoma: study of 47 South African cases

Cited by 63 publications

References 36 publications

Assessment of the prognostic impact of the Epstein–Barr virus-encoded latent membrane protein-1 expression in Hodgkin’s disease

Assessment of the prognostic impact of the Epstein–Barr virus-encoded latent membrane protein-1 expression in Hodgkin’s disease

Haematological cancers in African children: progress and challenges

Impact of tumor Epstein-Barr virus status on presenting features and outcome in age-defined subgroups of patients with classic Hodgkin lymphoma: a population-based study

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