Characteristics of patients with systemic lupus erythematosus (SLE) and non-Hodgkin’s lymphoma (NHL)

Patients with systemic lupus erythematosus (SLE) are at increased risk of developing non-Hodgkin’s lymphoma (NHL), but features of SLE associated with NHL are not well described. The objective of this study was to describe SLE characteristics, laboratory serologies, and medication histories in patients who subsequently develop NHL. Two thousand twenty patients with SLE were identified using the online Partners’ patient database research tool between October 1992 and June 2005. We confirmed the diagnoses of SLE and NHL and sought details of medical history and treatment by medical record review. Eleven patients with NHL without coexisting rheumatoid arthritis, Sjögren’s, or HIV were identified; seven of these (64%) had a diffuse large B cell lymphoma subtype, and 83% of those stained were Epstein–Barr virus (EBV) negative. The mean duration of SLE at NHL diagnosis was 17.8 years (range 1.6–41.8), and the mean Systemic Lupus International Collaborative Clinics/American College of Rheumatology damage index was 1.9. Seven patients (64%) had SLE hematologic involvement, four had anti-dsDNA antibodies, and four had anti-phospholipid antibodies. One patient had significant renal disease. All patients had arthritis and had received antimalarial therapy. Five of 11 patients had received other treatments for SLE, including cyclophosphamide, imuran, methotrexate, and/or sulfasalazine. Diffuse large B cell lymphoma was the most common subtype of NHL, and most were EBV negative. Although disease duration was fairly long and end organ damage moderately severe in this group of patients, renal disease and the use of immunosuppressive chemotherapeutic agents were rare and did not appear to confer an increased risk of NHL development.     

Primary mantle-cell non-Hodgkin’s lymphoma of the tongue

The evaluation of tongue swellings often represents a diagnostic challenge, because of the wide spectrum of benign and malignant possible lesions. We report a case of a patient presenting a tongue mass. An incisional biopsy was performed. Diagnosis of primary Mantle Cell non-Hodgkin's Lymphoma of the tongue was made by histological, immunohistochemical and cytogenetic studies. Our patient was treated with Rituximab-Cyclophosphamide, Epirubicine, Vincristine, Prednisone polychemotherapy plus Rituximab as single agent maintenance. Complete remission was achieved and no relapse has occurred during a follow-up of 53 months. We emphasize the importance of including also NHL in differential diagnosis of a tongue mass.     

Primary non-Hodgkin’s lymphoma of the breast: eight-year follow-up experience

The objective of this study is to analyze the clinical characteristics and treatment of patients with primary non-Hodgkin's lymphoma of the breast (PNHLB). Forty-five patients with PNHLB treated in our hospital during a 15-year period were retrospectively analyzed. Forty-four were females and one male, with a median age of 47 years. Forty-two patients were at stage I or II and 82.2% had diffuse large B cell lymphoma (DLBCL). Local control rate was 95.2 and 66.7% for patients with and without radiotherapy, respectively (P = 0.020). Median overall survival and progression-free survival (PFS) of all patients was 6.8 and 4.3 years, respectively. For patients with DLBCL or T cell lymphoma, median PFS was 6.5 years with chemoradiation and 3.9 years with chemotherapy or radiation only (P = 0.029). Patients who used rituximab had not reached median PFS, while those treated without rituximab had a median PFS of 5.1 years (P = 0.301). International prognostic index (IPI) score and bilateral breast involvement were two independent prognostic factors for survival. Chinese patients with PNHLB have early occurrence in lifespan. Radiation confers a better local control. Patients with intermediate or high-grade PNHLB might be treated with chemotherapy, radiotherapy, and for CD-20 positive disease, rituximab. Bilateral disease and IPI are two prognostic factors.     

Clonal relationship in multifocal non-Hodgkin’s lymphoma of mucosa-associated lymphoid tissue (MALT)

To elucidate the progression of gastric marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT) type, we analyzed a case presenting simultaneously with MALT lymphoma of the stomach and lung, and a gastric high-grade diffuse large lymphoma. The rearranged immunoglobulin heavy chain (IgH) variable regions were analyzed using a polymerase chain reaction (PCR)-based assay. Clonal relationship was shown between the gastric high-grade and the pulmonary low-grade lymphoma. The gastric MALT lymphoma was not related to the other manifestations. Translocation t(11;18) was not detected in the gastric high-grade lymphoma. MALT lymphomas at various locations and with different histologies may derive from a common precursor cell. Lymphomas at identical sites may have different stem cells.Supported in part by Deutsche Krebshilfe (70–2251-Ne1) and the Kempkes Stiftung     

Phase II study of ifosfamide, etoposide, and oxaliplatin (IFETOx) chemotherapy for relapsed or refractory non-Hodgkin’s lymphoma

As part of an effort to develop a more effective and safe treatment for relapsed or refractory non-Hodgkin’s lymphoma (NHL), we conducted a phase II study of the oxaliplatin, etoposide, and ifosfamide (IFETOx) regimen. Patients with relapsed or refractory NHL and a performance status of 0–2 were eligible. The IFETOx consisted of etoposide at 100 mg/m² on days 1–3, oxaliplatin at 130 mg/m² on day 2, and ifosfamide 5,000 mg/m² on day 2, every 21 days. The primary endpoint was the overall response rate (ORR) for IFETOx regimen. A total of 23 eligible patients were enrolled. The median age was 58 years (range 19–76 years), and the male-to-female ratio was 15:8. The disease status was as follows: 15 patients had relapsed and 8 patients were refractory to treatment. The ORR for IFETOx chemotherapy was 65.2 %. In the 15 patients who responded to the protocol treatment, five underwent hematopoietic stem cell transplantation. The 2-year probability of progression-free survival and overall survival rates were 51.4 and 56.1 %, respectively. Grade 3/4 neutropenia was observed in 73.9 % of the patients. No significant renal impairment was observed. In conclusion, IFETOx chemotherapy shows a tolerable toxicity profile and efficacy as a salvage treatment regimen for relapsed or refractory NHL.     

Long-term results of autologous stem cell transplantation for Hodgkin’s disease (HD) and low-/intermediate-grade B non-Hodgkin’s lymphoma (NHL): a report from the Austrian Stem Cell Transplantation Registry (ASCTR)

Between 1990 and 2001, 68 patients with advanced Hodgkin's disease (HD) and 86 patients classified as low-/intermediate-grade B non-Hodgkin's lymphoma (NHL) were reported to the Austrian Stem Cell Transplantation Registry (ASCTR). Following autologous stem cell transplantation (SCT) for HD, overall survival was 56% [95% confidence interval (CI): 40-72%] with a disease-/progression-free survival of 49%, reaching a plateau at 5 years. Using multivariate Cox regression analysis BEAM conditioning (carmustine, cytarabine, etoposide and melphalan) was predictive for favourable outcome, better disease-/progression-free survival and a significantly lower risk for relapse. The cumulative incidence of relapse was 30%, even for patients in complete remission at time of SCT. The cumulative risk for developing a secondary malignancy increased continuously over time, achieving 20% at 7 years and 46% at 10 years with previous radiotherapy as the only risk factor in the multivariate analysis. Overall survival for NHL patients was 45% (95% CI: 26-64%) with a disease-/progression-free survival of 26% at 7 years. In the multivariate Cox regression analysis stage of disease at time of SCT was the most powerful parameter for overall survival, disease-/progression-free survival and relapse. Mantle cell lymphoma, greater than or equal to three lines of previous therapy, and a conditioning regimen other than BEAM were also predictive for death. The main reason for treatment failure was relapse (cumulative incidence 54-75%). Because of the high risk of relapse/progression in both disease categories and the additional high rate of second malignancies in HD patients, allogeneic stem cells should be considered a valuable alternative for selected patients. The efficacy of allotransplantation following reduced-intensity conditioning should be tested in randomised trials.     

Primary non-Hodgkin’s lymphoma presenting as radicular syndrome: report of two cases

We report two cases of primary Non-Hodgkin’s Lymphoma in the spine leading to radicular compression secondary to infiltration of lumbar body vertebras. The two patients were free of either nodular or other extra-nodular disease. Treatment consisted of chemotherapy alone, one patient have had a cauda equina syndrome and surgical decompression was performed in emergency. The patients were in remission for 20 months after diagnosis. A review is given for the incidence of primary vertebral localization of lymphoma, its diagnosis, treatment and prognosis.     

Bendamustine, vincristine and prednisone (BOP) versus cyclophosphamide, vincristine and prednisone (COP) in advanced indolent non-Hodgkin’s lymphoma and mantle cell lymphoma: results of a randomised phase III trial (OSHO# 19)

Purpose: The purpose of this study was to compare the efficacy and toxicity of bendamustine, vincristine + prednisone (BOP) with a standard regimen of cyclophosphamide, vincristine + prednisone (COP) in patients with previously untreated advanced indolent non-Hodgkin’s lymphoma (NHL) and mantle cell lymphoma. Methods: A total of 164 patients with follicular lymphoma (grade 1/2), mantle cell lymphoma or lymphoplasmacytic lymphoma (immunocytoma) was randomised to treatment with vincristine 2 mg (day 1) and prednisone 100 mg/m² (days 1–5) + bendamustine 60 mg/m² (days 1–5) or + cyclophosphamide 400 mg/m² (days 1–5) for a total of eight 21-day cycles. Results: The rate of complete remission was 22% with BOP and 20% with COP. The projected 5-year survival rate was 61% with BOP and 46% with COP. The BOP-associated 5-year survival advantage almost reached significance in the subgroup of patients who responded to therapy (74% vs. 56%; P=0.05), and did reach significance in responders who did not receive interferon maintenance therapy (70% vs. 47%; P=0.03). Toxicity was acceptable in both treatment groups, although alopecia and leucopenia were more severe with COP. Conclusions: Bendamustine can efficaciously and safely replace cyclophosphamide, as used in standard COP therapy, for the treatment of patients with indolent NHL and mantle cell lymphoma. Long-term survival data suggest a clinically significant benefit for patients treated with BOP Funding support: Supported by a grant from ribosepharm GmbH, Clinical Research, Munich.     

The impact of treatment,socio-demographic and clinical characteristics on health-related quality of life among Hodgkin’s and non-Hodgkin’s lymphoma survivors: a systematic review

Cancer survivors are at risk of experiencing adverse physical and psychosocial effects of their cancer and its treatment. Both Hodgkin’s lymphoma (HL) and non-Hodgkin’s lymphoma (NHL) survivors face problems that can affect their health-related quality of life (HRQoL). The authors systematically reviewed the literature on HRQoL among HL and NHL survivors. A PubMed and PsychINFO literature search for original articles published until May 2011 was performed. Twenty-four articles, which met the predefined inclusion criteria, were subjected to a quality checklist. HL survivors showed the most problems in (role) physical, social and cognitive functioning, general health, fatigue and financial problems. In addition, HL survivors treated with a combination of therapies, with older age and female sex reported worse HRQoL. NHL survivors showed the most problems in physical functioning, appetite loss, vitality and financial problems. Having had chemotherapy was negatively associated with HRQoL, but no differences in chemotherapy regimens were found. Furthermore, in NHL survivors not meeting public exercise guidelines, HRQoL is low but can be improved with more exercise. More research on the longitudinal comparison between HL and NHL survivors and healthy controls should be performed in order to better understand the long-term (side) effects of treatment on HRQoL and possibilities to alleviate these.     

Prognostic significance of CD95, P53, and BCL2 expression in extranodal non-Hodgkin’s lymphoma

Apoptosis-related proteins play an important role in lymphoma cell death during chemotherapy. In our study, we investigated the prognostic significance of CD95, BCL2, and P53 expression in extranodal non-Hodgkin’s lymphoma (NHL). We examined 71 patients with extranodal NHL [45 diffuse large B-cell lymphomas (DLBCLs) and 26 mucosa-associated lymphoid tissue lymphomas (MALTLs)], 35 male and 36 female, with a median age of 65.8 years. The most common site of origin was the stomach (N = 31). Paraffin-embedded specimens were analyzed immunohistochemically for CD95, BCL2, and P53 expression. Multivariate analysis revealed that in DLBCLs, positive CD95 and negative BCL2 expression were independent prognostic factors for overall survival. We reached the same conclusion for MALTLs, with positive CD95 and negative P53 expression. In DLBCLs, the 5-year overall survival rate was 71.5% for the CD95-positive cases and 35% for the CD95-negative cases (p = 0.004) and the 5-year overall survival was significantly better in BCL2-negative cases (70.8%) when compared to BCL2-positive cases (37%; p = 0.009). In MALTLs, the 5-year overall survival rate for the CD95-positive and CD95-negative groups was 89.5% and 42.9%, respectively (p = 0.004) and the 5-year overall survival rate was 50% for the P53-positive cases and 88.9% for the P53-negative cases (p = 0.016). In conclusion, positive CD95 expression proved to be a good prognostic factor of overall survival in both extranodal DLBCLs and MALTLs. In contrast, positive expression of BCL2 and P53 was found to be unfavorably associated with survival in extranodal DLBCLs and MALTLs, respectively.     

Association of HLA alleles with non-Hodgkin’s lymphoma in Korean population

Several studies have been performed on the association between non-Hodgkin’s lymphoma (NHL) and the presence of certain human leukocyte antigens (HLA) class II alleles in Asian countries, and these studies have shown different results, according to the ethnicity, for the frequencies of the HLA class II alleles, and especially for HLA-DRB1. Therefore, the distribution of the HLA-A, B, C, DRB1, and DQB1 alleles in 89 Korean patients with NHL and also in 200 healthy Korean controls was investigated in this study. For the class I alleles, the frequencies of HLA-B51 was increased in patients with NHL and diffuse large B cell (DLBC) lymphoma compared with the normal control. For the class II alleles, the frequencies of the HLA-DRB1*09 and DQB1*03 alleles were increased in patients with NHL and DLBC lymphoma compared with the normal controls. Also, the B51-DRB1*09-DQB1*03 haplotype was significantly increased in the patients with NHL. These results suggest that some genes in HLA-B*51-DRB1*09-DQB1*03 haplotype may contribute to NHL susceptibility in the Korean population.     

Frequencies of non-Hodgkin’s lymphoma subtypes in Kuwait: comparisons between different ethnic groups

There is a wide variation in the prevalence of various subtypes of non-Hodgkin’s lymphoma worldwide. The aim of this study was to determine the relative frequency of different subtypes of non-Hodgkin’s lymphoma in Kuwait based on the Revised European–American Lymphoma (REAL) classification. From 1998 to 2006, 738 subjects were included that were registered with non-Hodgkin’s lymphoma in the population-based cancer registry at the Kuwait Cancer Control Center. Expert pathologists reviewed histological slides from all subjects. We performed detailed immunohistochemical studies and classified subjects based on the REAL classification. The prevalence of different types of non-Hodgkin's lymphoma was determined based on age, sex, site of disease, and ethnicity. Ethnicity groups comprised Kuwaiti Arabs, non Kuwaiti Arabs, Asians, and others. The prevalence of B- and T-cell lymphomas was 81.8% and 14.2%, respectively. The most common age group was 41–60 years old. The three most common subtypes in Kuwaiti Arabs were diffuse large B-cell lymphoma (46.5%), follicular lymphoma (15.5%), and mycosis fungoides (9.3%). In non-Kuwaiti Arabs, the most common subtypes were diffuse large B-cell lymphoma (48%), B-cell small lymphocytic lymphoma/chronic lymphocytic leukemia (15.8%), and follicular lymphoma (12.7%). Overall, non-Kuwaiti Arabs exhibited the highest prevalence (59%), and 54% of all cases had extranodal presentation. Compared to the Western world, Kuwait had a lower prevalence of follicular lymphoma, a higher prevalence of diffuse large B-cell lymphoma and extranodal presentation, and a high frequency of mycosis fungoides. Compared to other parts of Asia, Kuwait had a lower frequency of peripheral T-cell lymphomas.     

Association of VEGF genetic polymorphisms with the clinical characteristics of non-Hodgkin’s lymphoma

Purpose  

Vascular endothelial growth factor (VEGF) plays an important role in tumor angiogenesis and cancer progression. The VEGF genetic polymorphisms were shown to be independently associated with an adverse outcome in various malignancies. We investigated the possible associations of two polymorphisms (−2578C/A and +936C/T) in the VEGF gene with the clinicopathologic parameters for patients with non-Hodgkin’s lymphoma (NHL).     

Clinical significance of jejunoileal involvement of non-Hodgkin’s lymphoma detected by double-balloon enteroscopy

Jejunoileal involvement of non-Hodgkin’s lymphoma (NHL) is an important diagnostic factor in determining optimal treatment strategies. Here, we used double-balloon enteroscopy (DBE) to detect jejunoileal involvement of NHL and studied its clinical significance in a series of patients with NHL. Adults aged between 18 and 85 years with infiltration of the stomach, duodenum, or colon confirmed by gastrointestinal endoscopy or colonoscopy, suspected jejunoileal involvement determined by CT or FDG-PET, or any other gastrointestinal symptoms, were eligible for inclusion in the study. Among 428 patients with histologically confirmed NHL between 2004 and 2011, 83 were eligible for DBE, but 20 patients were excluded due to rejection or poor clinical status. Thus, 63 underwent DBE. The 3-year overall survival rate was significantly lower in patients with (n = 33), than without (n = 30) jejunoileal involvement of NHL confirmed by DBE (49 vs. 92 %, p < 0.005). Four participants developed aspiration pneumonia, but recovered after treatment with antibiotics.     

Dose-dense therapy improves survival in aggressive non-Hodgkin’s lymphoma

This study aimed to determine whether dose-dense therapy improves 3-year survival over the standard therapy for untreated aggressive lymphoma. One hundred and fifteen patients with untreated aggressive lymphoma were stratified by center, age, and international prognostic index and randomized to one of two treatment arms. One hundred and three were eligible. The experimental dose-dense arm consisted of weekly therapy with cyclophosphamide, epirubicine, vincristine, prednisolone, ifosfamide, etoposide, methotrexate, dexamethasone, and filgrastim (CEOP/IMVP-Dexa). The standard arm consisted of three-weekly cyclophosphamide, doxorubicin, vincristine, and prednisolone (CHOP). The primary endpoint was overall survival after 3 years. Overall survival at 3 years was 0.766 (95% CI 0.6247, 0.8598) in the dose-dense arm and 0.462 (95% CI 0.3200, 0.5925) in the CHOP arm. Overall 5-year survival was 0.746 (95% CI 0.603, 0.843) in the dose dense and 0.406 (95% CI 0.265, 0.543) in the CHOP arm (P?=?0.0062). Grade 3 and 4 infections occurred four times more frequently in the dose-dense arm. However, two patients died from toxicity in the dose-dense arm and three in the CHOP arm. Dose-dense therapy with CEOP/IMVP-Dexa is feasible and resulted in an absolute increase of 34% in the survival probability compared to CHOP in untreated patients with aggressive lymphoma.