Burkitt's lymphoma in Australia

An Australian case of Burkitt's lymphoma is reported. The clinical features of large ovarian masses and subsequent bone marrow invasion, as well as the results of investigations related to the Epstein-Barr virus, were more consistent with the American than the African type of Burkitt's lymphoma. After a good initial response to cyclophosphamide and vincristine, the tumour rapidly became resistant to these and other therapeutic measures and the patient died 10 months after diagnosis.     

The so-called white Burkitt's lymphoma

A case of histopathologically proven Burkitt's lymphoma is described with special reference to clinical, serological and immunological features. This case report is followed by a review of the literature on the problem of American and African Burkitt's lymphoma. We can state that there is good correspondence between the white and black Burkitt's lymphomas with regard to epidemiology, histopathology, therapy and some immunological aspects. The two groups differ from each other in age, primary tumor manifestation, involvement of the bone marrow at time of diagnosis and quantitatively in the positive EBNA-test. It is therefore suggested that black and white Burkitt's lymphoma are not different diseases but different patterns of one disease.     

Insight into Burkitt's lymphoma from immunoglobulin variable region gene analysis

Analysis of usage of V(H) and V(L) genes, and the degree and pattern of somatic mutation, has been used to investigate the cell of origin and clonal history in cases of Burkitt's lymphoma (BL). Tumor cell lines and biopsy material from patients with endemic, sporadic and AIDS-associated BL have been compared. V(H) genes were most commonly derived from the V(H)3 (52%) and V(H)4 (39%) families. This shows a similar gene usage of the V(H)3 family to that seen in the normal peripheral blood repertoire (55%), but a biased usage of the V(H)4 family (22% in normal). There was no restriction in V(L) gene usage. This overall distribution was similar in all subsets of BL. In all categories, there was significant somatic mutation in both V(H) and V(L) sequences. There was no evidence for accumulation of mutations in cell lines cultured in vitro indicating that all mutations in BL-derived cell lines have accumulated in vivo. The mean percentage level of mutation +/- standard deviation was greater in endemic BL (V(H) = 7.7 +/- 4.0, V(L) = 5.3 +/- 2.2) and AIDS-associated BL (V(H) = 7.5 +/- 3.6, V(L) = 3.9 +/- 1.9) than in sporadic BL (V(H) = 4.0 +/- 2.5, V(L) = 2.2 +/- 1.2). The pattern of somatic hypermutation was similar in V(H) and V(L) sequences of the different types of BL although the light chain genes were less mutated. Mutational patterns in the V(H) genes did not reveal a conventional role for antigen in selection of tumor cell sequences in 23/25 V(H) genes analysed. In contrast, patterns in V(L) sequences were consistent with a role for antigen in 8/13 sBL +/- eBL cases and 8/17 cases overall. The presence of EBV did not seem to influence the quantity or pattern of somatic mutations. Evidence for intraclonal variation was seen in uncloned cell lines from cases of eBL and AIDS-associated BL and confirmed in biopsy material in some, but not all cases of eBL, sBL and AIDS-associated BL examined. These common features indicate that the B-cells involved in all types of BL are derived from cells that have traversed the germinal centre, and that the somatic mutation mechanism may still be operative following neoplastic transformation. Overall, in 10/30 cases, there was evidence of significant clustering of replacement amino acids, in CDRs, particularly in V(L), indicating that the B-cell of origin is likely to have been selected by antigen.     

The imaging of Burkitt's and Burkitt-like lymphoma

We describe the radiological findings at presentation and follow up in 24 adult patients with Burkitt's and Burkitt-like lymphoma, age range 17-67 years. This is an older age group than previously described in North American series, but the clinical and imaging characteristics appear similar. Disease confined to the abdomen was seen in 12 (50%) at presentation, of whom 11 had bowel or mesenteric tumours. Of those with bowel involvement, five patients had disease that had arisen in the ileocaecal region. Intra-abdominal disease was large volume (greater than 5 cm) in the majority of patients. Four patients (17%) had small isolated masses in the head and neck region, two (8%) had isolated unilateral axillary lymphadenopathy. Six (25%) patients presented with disseminated disease, all with hepatic and/or splenic involvement, intra-abdominal and peripheral lymphadenopathy. Three of these patients with disseminated disease were human immunodeficiency virus (HIV) positive. Neurological symptoms were present in a total of five patients at presentation or relapse but positive imaging findings were present in only two patients who had MRI. Computed tomography (CT) head scans performed in four patients were normal. Disease recurrence most commonly occurred within the abdomen.     

Soft tissue Burkitt's lymphoma: radiological findings

An unusual case is reported of a soft tissue mass in the lower extremity, without bone involvement, in an 85-year-old woman; the histopathological diagnosis was Burkitt's lymphoma. Pertinent clinical history, histological examination, and imaging procedures allowed early diagnosis. To our knowledge, the radiological findings in Burkitt's lymphoma with this unusual clinical presentation have not been described previously.     

Non-African Burkitt's lymphoma in a young woman

Non-African Burkitt's lymphoma is presented in a 29-year-old, unmarried woman, who developed tumors in both breats and ovaries, ascites and pleural effusion. Assessment of B cells in the tumor cells, derived from ascites, pleural effusion and tumor tissue is 90%, surface IgM being consisted of 86%, in an average. Histologically, the tumor tissue demonstrates prominent, socalled starry-sky effect, and cytologically, tumor cells are poorly-differentiated lymphocytoid cells in their feautures.     

Burkitt's lymphoma mimicking an acute dentoalveolar abscess

Burkitt's lymphoma is a monoclonal proliferation of B lymphocytes classified histologically as a poorly differentiated lymphocytic lymphoma. The jaw and retroperitoneal structures are the most commonly involved sites. Prognosis is highly dependent on the stage of the disease. In some cases, the first manifestation of Burkitt's lymphoma is in the jaws, and symptoms may be misdiagnosed as infection. Dental radiographs can play an important role in the diagnosis. A case of a peculiar Burkitt's lymphoma involving the mandible that was misdiagnosed as an acute dentoalveolar abscess is presented.     

Radiation therapy in Burkitt's lymphoma. Long term results

In 40 of 61 irradiated patients with Burkitt's lymphoma the long term results were evaluated. Five patients were primarily irradiated while the remaining patients had late recurrences or were chemotherapy failures. Seven patients were alive and free of recurrence after 6 months and 4 after 24 months.     

Mandibular soft tissue mass as the initial presentation of Burkitt's lymphoma

A six-year-old girl was evaluated for an intraoral mass of 5 months duration, which had been treated with antibiotics in a district hospital. Extraoral examination showed a firm, non-tender swelling at the angular region of the left mandible with regional lymphadenopathy. Intraoral examination revealed a mass extending from the left mandibular primary first molar to the retromolar region, interfering normal occlusion. The mass was painful and firm, peduculated and reddish in appearance. The remainder of the oral examination was unremarkable. Panoramic radiograph and CT scan showed a large multilocular radiolucency beginning from the bud of second premolar, extending to the mandibular ramus that displaced the bud of second molar. Incisional biopsy of the intraoral mass was performed and the result was reported as Burkitt's lymphoma. The child was treated with chemotherapy and radiation, with recurrence.     

Integration of human herpesvirus 6 in a Burkitt's lymphoma cell line

Human herpesvirus 6 (HHV-6) genome has been found in several human lymphoid malignancies, but configuration of the HHV-6 genome has not been well delineated. We established the HHV-6-positive, Epstein-Barr virus-negative Burkitt's lymphoma cell line Katata. In this study we investigated the status of the HHV-6 genome in Katata cells. Neither linear nor circular HHV-6 DNA was detected by Gardella gel analysis. The fluorescence in situ hybridization technique enabled us to directly visualize the integrated HHV-6 DNA at the single-cell level. Only one integrated site of viral DNA was detected in metaphase chromosomes and it was preferentially located at the long arm of chromosome 22 (22q13). Treatment of the cells with 12-O-tetradecanoyl-phorbol-13-acetate (TPA) or with calcium ionophore A23187 led to induction of the HHV-6 immediate-early gene as well as the late gene. Sodium n-butyrate also gave rise to expression of the HHV-6 genes. The TPA inducibility was synergistically enhanced when combined with A23187 or n-butyrate. Our study provides, for the first time, an in vitro model system of latent HHV-6 infection whose genome is integrated into host DNA of lymphoma cells.     

Differential regulation of glycosphingolipid biosynthesis in phenotypically distinct Burkitt's lymphoma cell lines

Earlier studies have shown that Burkitt's lymphoma (BL) cell lines can be divided into 2 major groups: group I, which retain the original BL biopsy phenotype with expression of CD10 and CD77 antigens and lack of B-cell activation markers, and group III, which, after several in vitro passages, progress toward an "LCL-Like" phenotype with loss of CD10 and C77 expression and up-regulation of B-cell activation antigens. In previous studies we have shown that several glycolipid molecules constitute stage-specific antigens for B cells and that sequential shifts in the 3 major glycolipid series are observed during B-cell differentiation, these changes being mostly due to sequential activations of the corresponding glycosyltransferases. In the present work, 10 BL cell lines with group I or group III phenotype have been examined for cell surface expression of 5 glycolipid antigens (LacCer, GM3, Gb3/CD77, Gb4 and GM2), total glycolipid content and enzymatic activities of 4 glycosyltransferases (GM3, Gb3, Gb4 and GM2 synthetases). We now report that group I and group III BL cells differ in their glycolipid metabolism and express either mostly globoseries or ganglioseries compounds. Indeed, Gb3 is the major glycolipid of group I cells, whereas GM3 and GM2 are the 2 major components of group III cells, and these phenotypic differences are mainly due to differential activities of the corresponding glycosyltransferases: group I cells have high Gb3 synthetase activities and low or no GM3 and GM2 synthetase activities, whereas group III cells have high GM3 and GM2 synthetase activities and low Gb3 synthetase activities. Finally, we also show that, unlike LCL, group III BL cells do not synthesize Gb4.     

A B-cell-homing chemokine made in lymphoid follicles activates Burkitt's lymphoma receptor-1

Secondary lymphoid organs (spleen, lymph nodes and Peyer's patches) are divided into compartments, such as B-cell zones (follicles) and T-cell zones, which provide specialized environments for specific steps of the immune response. Migration of lymphocyte subsets into these compartments is essential for normal immune function, yet the molecular cues guiding this cellular traffic are poorly defined. Chemokines constitute a family of chemotactic cytokines that have been shown to direct the migration of leukocytes during inflammation and which may be involved in the constitutive homing of lymphocytes into follicles and T-cell zones. Here we describe a novel chemokine, B-lymphocyte chemoattractant (BLC), that is strongly expressed in the follicles of Peyer's patches, the spleen and lymph nodes. BLC strongly attracts B lymphocytes while promoting migration of only small numbers of T cells and macrophages, and therefore is the first chemokine to be identified that is selective towards B cells. An orphan chemokine receptor, Burkitt's lymphoma receptor 1 (BLR-1), has been found to be required for B-cell migration into lymphoid follicles. We show that BLC stimulates calcium influx into, and chemotaxis of, cells transfected with BLR-1. Our results indicate that BLC functions as a BLR-1 ligand and may guide B lymphocytes to follicles in secondary lymphoid organs.     

Age and sex as factors influencing remission duration in African Burkitt's lymphoma

The frequency of Burkitt's lymphoma peaks in children six to nine years of age and is twice as high in males as in females. To determine if age or sex had any relationship to outcome of therapy, the authors examined the experience of 387 patients treated for Burkitt's lymphoma at the Burkitt Tumor Project, Accra, Ghana, between 1966 and 1978. No age or sex relationship could be demonstrated with overall survival. However, among children without central nervous system disease who achieved an initial remission, the relapse rates were higher for those diagnosed initially in the age interval 6-9 years than for those diagnosed at younger or older ages. This pattern was seen for each stage of the disease as well as overall. Sex did not influence relapse rate. The relationship of these findings to the Epstein-Barr virus and malaria, both suspected factors in the etiology of this disease, is discussed, but neither appears to explain our results.     

Chromosome 8-14 translocation in a non-African Burkitt's lymphoma with leukemic conversion

A specific chromosome translocation, t(8q-; 14q+), was observed in a 43-year-old female with non-African Burkitt's lymphoma in which leukemic conversion had occurred. The chromosome studies used cells from ascites. The ascites was apparently the result of a primary tumor involving the ovaries and contained 68% of lymphoma cells. The frequent occurrence of abnormalities related to chromosomes 1, 8 and 14 in African and non-African Burkitt's lymphomas was emphasized.