Search

Follicular lymphoma (FL) is a heterogeneous disease with varying prognosis owing to differences in clinical, laboratory, and disease parameters. Although generally considered incurable, prognosis for early and advanced stage disease has improved because of therapeutic advances, several of which have resulted from elucidation of the biologic and molecular basis of the disease. The choice of treatment for FL is highly dependent on patient and disease characteristics. Several tools are available for risk stratification, although limitations in their routine clinical use exist. For limited disease, treatment options include radiotherapy, rituximab monotherapy or combination regimens, and surveillance. Treatment of advanced disease is often determined by tumor burden, with surveillance or rituximab considered for low tumor burden and chemoimmunotherapy for high tumor burden disease. Treatment for relapsed or refractory disease is influenced by initial first-line therapy and the duration and quality of the response. At present, there is no consensus for treatment of patients with early or multiply-relapsed disease; however, numerous agents, combination regimens, and transplant options have demonstrated efficacy. While the number of therapies available to treat FL has increased together with an improved understanding of the underlying biologic basis of disease, the best approach to select the most appropriate treatment strategy for an individual patient at a particular time continues to be elucidated. This chapter considers prognostic factors and the evolving treatment landscape of FL, including recent and emerging therapies, as well as remaining unmet needs. [ABSTRACT FROM AUTHOR]

Objectives: Lymphoma (Hodgkin and Non-Hodgkin) is one of the most common malignancies in Sudan. Contemporaneous studies from several countries including the United States, Japan and Germany suggested a possible etiological link between Merkle cell polyoma virus (MCPyV) and these lymphomas; however, there are no previous studies that have been reported on this topic in Sudan. This study was applied to investigate the role of this virus in lymphoma patients in Sudan. Objective is to detect and characterize Merkle Cell Polyomavirus in lymphoma patients by using molecular techniques Method: Adult Sudanese patients (both females and males) diagnosed with lymphoma were included in this study. Blood samples were used for DNA extraction which was then subjected to Real-time PCR to detect MCPyV DNA. Samples, proved positive by Real-time PCR amplification, were sequenced and phylogenetically analyzed. Results: MCPyV was detected in 16/225 from Sudanese (7.1%) of lymphoma patients of which five were Hodgkin and eleven were Non-Hodgkin. MCPyV sequences in Sudanese patients showed 100% similarity to each other and 100% identity with reference sequences from gene bank. Conclusion: This study is the first evidence that MCPyV exists in a subset of Sudanese lymphoma patients. MCPyV is unlikely to have direct contribution in the pathogenesis of lymphoma in the majority of Sudanese cases. MCPyV identified from the Sudanese lymphoma subjects may constitute an African lineage and its pathogenicity needs to be investigated in future studies. [ABSTRACT FROM AUTHOR]

Lymphoma arises from cells of the immune system and trace metals augment the immune system and their imbalance may promote immunological disorders including tumorigenesis. The primary aim of the present investigation was to evaluate the levels of essential/toxic trace metals in the nails of non-Hodgkin and Hodgkin lymphomas patients in comparison with controls. The samples collected from patients and controls were digested in the mixture of HNO3-HClO4 and selected trace metals were analysed using flame atomic absorption spectrometry. The results showed that mean concentrations of some elements (Pb, Ni, Cd, Cu and Cr) in nails of non-Hodgkin lymphoma patients were significantly elevated (p < 0.05) than that of the controls whereas mean contents of Pb, Cu, Cd and Cr were observed to be significantly higher in the nails of Hodgkin lymphoma patients compared with healthy donors. Additionally, correlation study pointed out significantly diverse mutual associations of the trace metals among the patients and controls. The present results revealed noticeable disparities in the metal concentrations based on gender, food habits, tobacco use and types/stages of the donor's groups. Overall, the pathogenesis of disease significantly affected the trace metal balance in both patients' groups. [ABSTRACT FROM AUTHOR]

Primary cardiac neoplasms are uncommon and often present with nonspecific cardiac symptoms. Proper treatment can only be initiated after histological analysis of a surgical specimen. Two cases of primary cardiac lymphoma with various clinical presentations and different growth patterns are presented here. [ABSTRACT FROM AUTHOR]

Objectives: To assess the prevalence of Hashimoto thyroiditis (HT) in primary thyroid lymphoma (PTL) and whether it differs between mucosa-associated lymphoid tissue (MALT) lymphoma and diffuse large B-cell lymphoma (DLBCL).Methods: Electronic databases were searched for studies assessing HT prevalence in PTL, based on antithyroid antibodies, clinical history, or pathology. Pooled prevalence of HT and its association with histotype (MALT or DLBCL) were calculated.Results: Thirty-eight studies with 1,346 PTLs were included. Pooled prevalence results were 78.9% (any HT evidence), 65.3% (antithyroid antibodies), 41.7% (clinical history), and 64% (pathology). HT prevalence was significantly higher in MALT lymphoma than in DLBCL (P = .007) and in mixed DLBCL/MALT than in pure DLBCL (P = .002).Conclusions: Overall, 78.9% of patients with PTL have any HT evidence, but only half of these had been clinically followed. The difference in HT prevalence suggests that a subset of DLBCL may not derive from MALT lymphoma. [ABSTRACT FROM AUTHOR]

Introduction. Even in the era of combined antiretroviral therapy, the mortality rate in patients with human immunodeficiency virus infection remains high, especially with a contributing diagnosis of a malignant disease, such as non-Hodgkin lymphoma. Given the previous, the goal of this research was to establish the incidence of non-Hodgkin lymphoma in human immunodeficiency virus positive patients, as well as to determine their clinical characteristics and mortality in regard to patients with human immunodeficiency virus only. Material and Methods. The retrospective study included 396 human immunodeficiency virus-positive patients. Medical records were reviewed to analyze the average age, duration of infection, average duration of therapy, nCD4+ T-cell count, human immunodeficiency virus viral load, as well as the number and types of malignant diseases. Results. The average age of the patients was 44.2 years; the average nCD4+ T-cell count was 296.94 cells/μL, while the mortality rate was 14.65%. The leading causes of death were non-Hodgkin lymphoma and acquired immunodeficiency syndrome. The most frequently diagnosed malignancy was non-Hodgkin lymphoma, where the average count of nCD4+ T-cells was 162.29 cells/μL. Patients with human immunodeficiency virus and non-Hodgkin lymphoma had significantly lower nCD4+ T-cell count, in regard to patients with human immunodeficiency virus only, and the mortality rate in this group of patients was 85%. Conclusion. The incidence of non-Hodgkin lymphoma in human immunodeficiency virus-positive patients represents a growing threat, given the exceptionally high mortality. The nCD4+ T-cell count may indicate acquired immunodeficiency syndrome and late diagnosis of human immunodeficiency virus together are predictors for non-Hodgkin lymphoma and its poor outcome. It points to the importance of increasing the scope of human immunodeficiency virus testing, as well as finding a better treatment approach. [ABSTRACT FROM AUTHOR]

Despite highly active antiretroviral therapy (HAART), AIDS related lymphoma (ARL) occurs at a significantly higher rate in patients infected with the Human Immunodeficiency Virus (HIV) than in the general population. HIV-infected macrophages are a known viral reservoir and have been shown to have lymphomagenic potential in SCID mice; therefore, there is an interest in determining if a viral component to lymphomagenesis also exists. We sequenced HIV-1 envelope gp120 clones obtained post mortem from several tumor and non-tumor tissues of two patients who died with AIDS-related Non-Hodgkin's lymphoma (ARL-NH). Similar results were found in both patients: 1) high-resolution phylogenetic analysis showed a significant degree of compartmentalization between lymphoma and non-lymphoma viral sub-populations while viral sub-populations from lymph nodes appeared to be intermixed within sequences from tumor and non-tumor tissues, 2) a 100-fold increase in the effective HIV population size in tumor versus non-tumor tissues was associated with the emergence of lymphadenopathy and aggressive metastatic ARL, and 3) HIV gene flow among lymph nodes, normal and metastatic tissues was non-random. The different population dynamics between the viruses found in tumors versus the non-tumor associated viruses suggest that there is a significant relationship between HIV evolution and lymphoma pathogenesis. Moreover, the study indicates that HIV could be used as an effective marker to study the origin and dissemination of lymphomas in vivo.

Background Summary statistics comparing the stage at diagnosis of geographically defined populations of cancer patients are increasingly used in public reporting to monitor geographical inequalities but may be confounded by patient case mix. We explore the impact of case-mix adjustment on a publicly reported measure of early stage at diagnosis in England. Methods We analyzed data used for publicly reported statistics about the stage of patients diagnosed with 1 of 11 solid tumours in 2015 in England, including information on cancer site (bladder, breast, colon, rectum, kidney, lung, melanoma, non-Hodgkin lymphoma, ovarian, prostate, endometrial), age, gender, income deprivation and population-based commissioning organization. We investigated how cancer site and other patient characteristics influence organizational comparisons and attainment of early-stage targets (≥60% of all cases diagnosed in TNM stages I–II). Results Adjusting for patient case mix reduced between-organization variance by more than 50%, resulting in appreciable discordance in organizational ranks (Kendall's tau = 0.53), with 18% (37/207) of organizations being reclassified as meeting/failing the early-stage target due to case mix. Conclusion Summary statistics on stage of cancer diagnosis for geographical populations currently used as public health surveillance tools to monitor organizational inequalities need to account for patient sociodemographic characteristics and cancer site case mix. [ABSTRACT FROM AUTHOR]

T-cell/histiocyte-rich large B-cell lymphoma (THRBCL) is an uncommon subtype of non-Hodgkin's lymphoma. It is a predominant nodal neoplasm; however, extranodal sites, such as the spleen, liver and bone marrow, can be involved at diagnosis. However, only one case of primary THRLBCL in the jaws have been reported. We herein describe a 29-year-old female patient who presented with a swelling of the right mandible that had grown rapidly over the previous 2 months. Periapical and panoramic radiographs showed a multilocular osteolytic lesion located in the mandibular periapical region of the canine and premolar teeth and molar region. Preoperative examination and incisional biopsy were performed. Immunohistochemistry was applied to confirm the diagnosis of THRBCL in the jaw. The treatment consisted of CHOP therapy and radiotherapy. After complete tumor remission following initial treatment, additional sites of the disease appeared in the lung, abdomen and long bones. The patient died within 2 months. THRLBCL is an uncommon and aggressive malignant neoplasm that can involve the jaws, mimicking a periapical disease. [ABSTRACT FROM AUTHOR]

BackgroundNon-Hodgkin lymphoma (NHL) is the fifth most common cancer in the U.S. and few causes have been identified. Genetic association studies may help identify environmental risk factors and enhance our understanding of disease mechanisms.Methodology/principal findings768 coding and haplotype tagging SNPs in 146 genes were examined using Illumina GoldenGate technology in a large population-based case-control study of NHL in the San Francisco Bay Area (1,292 cases 1,375 controls are included here). Statistical analyses were restricted to HIV- participants of white non-Hispanic origin. Genes involved in steroidogenesis, immune function, cell signaling, sunlight exposure, xenobiotic metabolism/oxidative stress, energy balance, and uptake and metabolism of cholesterol, folate and vitamin C were investigated. Sixteen SNPs in eight pathways and nine haplotypes were associated with NHL after correction for multiple testing at the adjusted q

ObjectiveWe sought to determine the accuracy of cancer registry data regarding the human immunodeficiency virus (HIV)/acquired immunodeficiency syndrome (AIDS) status of patients with non-Hodgkin's lymphoma (NHL).MethodsWe used the population-based San Diego/Orange County cancer registry to identify 392 patients with HIV-related NHL diagnosed 1994-1999. After matching for age, sex, race, period of NHL diagnosis, and hospital type, we were able to find 324 corresponding patients among the remaining 4,863 NHL patients diagnosed 1994-1999 (who did not have HIV infection according to cancer registry records). We sought to review these patients' charts at 41 hospitals with 15 separate institutional review boards to determine if the HIV serostatus from the cancer registry was correct. We performed a forward conditional multivariate logistic regression to determine characteristics associated with a false positive HIV status.ResultsThe false positive rate was 8% while the false negative rate was 3%. The positive predictive value was 93% while the negative predictive value was 97%. Compared to correctly identified patients, false positives were more likely to be > or =50 years old, female, and treated with chemotherapy and less likely to be single with high grade or extranodal disease.ConclusionUsing cancer registry data to identify AIDS-related NHL is a valid research practice.

Primary thyroid lymphoma is incredibly rare with an annual incidence of approximately 2.1 per million persons, accounting for 2% of thyroid malignancies. One of the only known risk factors is autoimmune thyroid disease, specifically Hashimoto’s, although there have been a few cases of thyroid lymphoma associated with Graves’ disease. Here, we present one such case of a patient with pre-existing hyperthyroidism who developed non-Hodgkin’s lymphoma of the thyroid. The patient initially presented with rapidly progressing right-sided neck swelling. He was clinically and biochemically euthyroid with negative thyroid stimulating immunoglobulin, maintained on methimazole. Biopsy of the thyroid nodule with flow cytometry revealed non-Hodgkin lymphoma. Intervention included radiation therapy.

We used the San Diego/Orange County cancer registry to identify 64 cases of systemic non-Hodgkin's lymphoma (NHL) with AIDS who received highly active antiretroviral therapy (HAART) at the time of NHL diagnosis or thereafter and 64 NHL controls without AIDS, matched on age, sex, race, time of NHL diagnosis (1994-1995 and 1996-1999), and hospital type (academic, large community, and small community). We compared cases and controls by chi-squared tests and Kaplan-Meier methods. Thirty-three percent of cases had high grade histology versus 11% of controls (p < 0.01); 69% had baseline hemoglobin

A population-based case-control study of lymphomas in England collected height and weight details from 699 non-Hodgkins lymphoma (NHL) cases and 914 controls. Obesity, defined as a body mass index (BMI) over 30 kg m(-2) at five years before diagnosis, was associated with an increased risk of NHL (OR = 1.5, 95% CI 1.1-2.1). The excess was most pronounced for diffuse large B-cell lymphoma (OR = 1.9, 95% CI 1.3-2.8). Genetic variants in the leptin (LEP 19G > A, LEP -2548G > A) and leptin receptor genes (LEPR 223Q > R), previously shown to modulate NHL risk, as well as a polymorphism in the energy regulatory gene adiponectin (APM1 276G>T), were investigated. Findings varied with leptin genotype, the risks being decreased with LEP 19AA (OR = 0.7, 95% CI 0.5-1.0) and increased with LEP -2548GA (OR = 1.3, 95% CI 1.0-1.7) and -2548AA (OR = 1.4, 95% CI 1.0-1.9), particularly for follicular lymphoma. These genetic findings, which were independent of BMI, were stronger for men than women.

DP-1 is a G1 cell cycle-related protein that forms heterodimers with E2F, a family of transcriptional factors regulating the expression of genes important for G1 to S progression. Although the exact role of DP-1 is not well understood, it has been shown to stabilize DNA binding of E2F proteins. By immunohistochemistry, the authors examined the expression of DP-1 in lymphoid tissues, including 8 cases of reactive follicular hyperplasia and 69 cases of B-cell non-Hodgkin lymphoma. The expression of the cell cycle-related proteins E2F-1 and Ki-67 was also assessed. Scoring was based on the proportion of labeled nuclei (1-10%, 11-25%, 26-50%, and > 50%). In reactive follicular hyperplasia, staining for DP-1, E2F-1, and Ki-67 was largely confined to the germinal centers. All 25 cases of follicular lymphoma, regardless of grade, had a high proportion (> 50%) of DP-1-positive cells but a lower proportion of cells marking for E2F-1 and Ki-67 (P < 0.001). The diffuse large B-cell lymphomas (n = 24) had high DP-1 and Ki-67 scores but low E2F-1 scores (P < 0.001). Small lymphocytic (n = 10), marginal zone (n = 3), and mantle cell lymphomas (n = 5) contained relatively low proportions of cells labeled for all three markers. Precursor B-cell lymphoblastic lymphoma (n = 2) displayed high proportions of cells positive for DP-1, Ki-67, and E2F-1 (> 50% in both cases). Except in follicular center cell lesions, DP-1 expression generally correlated with that of Ki-67. However, the expression of DP-1 was discordant with that of E2F-1 in benign and malignant follicular center cells, suggesting that DP-1 may have functions other than facilitating E2F-1-dependent gene regulation and cell cycle progression in these neoplasms.

Lymphomas are solid malignant tumors having a wide spectrum of clinical and pathological features. Non-Hodgkin’s lymphoma (NHL) is a subtype of lymphoma with two-thirds of the cases presenting as lymph node enlargement. The remaining one third of NHL cases has been reported in the extranodal sites, including the gastro intestinal tract, Waldeyer’s ring, bone, skin, and brain. Intraoral non-Hodgkin’s lymphoma is uncommon and may affect either the jaw bones or occur within the soft tissues of the oral cavity. Here we report a case of non-Hodgkin’s lymphoma in a 65-year-old male patient who presented with a growth from the extraction socket. Non-Hodgkin’s lymphoma presenting as a growth from the extraction socket is unusual. An orthopantamograph (OPG) was taken which revealed a diffuse radiolucent defect in relation to the extraction socket of the left lower molar region. Routine hemogram, urine analysis, and chest radiograph were normal. Incisional biopsy was performed and the tissue was subjected to histopathological examination. Histopathological and immunohistochemical analysis confirmed the diagnosis of B-cell lymphoma. The patient was referred to a regional cancer institute for further management, where chemotherapy was planned. However, prior to chemotherapy, the patient was diagnosed with brain metastasis and he expired in hospital within one month.

Burkitt lymphoma (BL) is the most frequent non-Hodgkin lymphoma in pediatric patients, accounting for approximately 34% of the cases of lymphoma in children. This subtype of non-Hodgkin lymphoma was first described in 1958 as a monoclonal proliferation of B cell lymphocytes. Cardiac involvement of BL in association with osseous compromise and lymphadenopathy is rare and poorly documented. We report a case of femur primary BL in an 8-year-old boy with metastatic cardiac involvement, retroperitoneal and iliofemoral lymphadenopathy, and hepatosplenomegaly. We highlight the diagnostic challenge in a patient with clinical nonspecific findings and systemic disease.

Intravascular large B-cell lymphoma (IVLBCL) is a rare subtype of diffuse large B-cell lymphoma with an estimated incidence of less than one per million. Unlike other hematopoietic malignancies, lymphadenopathy and hepatosplenomegaly are uncommon, and patients typically present with nonspecific symptoms. IVLBCL presents a diagnostic challenge and patients are usually diagnosed late in the disease course, if at all, and the prognosis is poor. The differential diagnosis is broad, and physicians often pursue multiple diagnostic possibilities during patient workup. We present a case of IVLBCL discovered at autopsy in an 80-year-old male who presented with history and symptoms pointing to the tick-borne illness ehrlichiosis.

Richter transformation (RT), or Richter syndrome, is defined as the transformation of chronic lymphocytic leukemia (CLL) to an aggressive B-cell lymphoma. The vast majority, up to 99%, transform into diffuse large B-cell lymphoma (DLBCL), with a small subset (

Lymphomas are malignant diseases that originate in the immune system cells and are predominantly manifested by lymphadenopathy; sometimes the presentation is in extranodal places. The eye and its annexes may be affected by a heterogeneous group of primary lymphomas or, secondarily, by the extension of lymphomas originated in lymph nodes or other extranodal sites. The case of a patient who went to the consultation due to left palpebral ptosis is reported and physical examination showed bilateral involvement, with tumors in the lower right eye bulbar conjunctiva and in the left eye upper bulbar conjunctiva. A small B cell lymphocytic non-Hodgkin lymphoma was diagnosed by conjunctiva biopsy. The patient was considered a stage IIIAE of Ann Arbor and treated with six cycles of cyclophosphamide, vincristine, doxorubicin and prednisone, plus the Cuban monoclonal antibody anti-CD20 CIMABior®. The complete response was reached, state in which the disease remains so far. [ABSTRACT FROM AUTHOR]

We analyzed the clinicopathologic characteristics of 136 intestinal diffuse large B-cell lymphomas (DLBCLs) among 126 patients. The DLBCL sites were categorized as: duodenum (n = 23), ileocecal region (n = 63), other small intestine (n = 29), rectum (n = 7), and other large intestine (n = 14). Patients with DLBCLs of the ileocecal region or other small intestine frequently underwent surgery for ileus or perforations (P < 0.001), were predominantly male (P = 0.042), and had a higher incidence of limited-stage disease (P = 0.001), lower International Prognostic Index (P = 0.015), and lower incidence of lactate dehydrogenase elevation (P = 0.007) than those with DLBCLs of other regions. Half of the intestinal DLBCLs exhibited the germinal center B-cell phenotype. A low-grade B-cell lymphoma background was found in 21% of the cases; the prevalence was significantly lower in the ileocecal region (13%, P = 0.025), suggesting a higher incidence of de novo DLBCLs. Intestinal follicular lymphoma (FL) and mucosa-associated lymphoid tissue (MALT) lymphoma backgrounds were observed in 10% and 0% of the cases, respectively. Five percent (5/107) of intestinal DLBCL cases were Epstein-Barr virus-encoded RNA-1 positive. The clinicopathologic characteristics of the DLBCLs differed by region. Histologic transformation of intestinal FL was observed in around 10% of the intestinal DLBCL cases. [ABSTRACT FROM AUTHOR]

The article discusses a population-based research which investigated link between systemic lupus erythematosus (SLE) and non-Hodgkin's lymphoma (NHL). Topics explored include the use of the National Health Insurance Research Database (NHIRD) of Taiwan in identifying SLE and NHL patients for the study, the cancer development follow-up performed, and the prevalence of SLE and NHL incident patients in Taiwan.

We describe the clinical course of a patient who developed high-grade lymphoma during immunosuppression treatment with cyclosporine A, following liver transplantation. After anti-neoplastic polychemotherapy treatment, the remission of lymphoma was confirmed and maintained for over four years. The patient, a 27 year old female had liver transplantation at the age of 17, due to acute liver failure, caused by non-diagnosed Wilson disease. Nearly seven years post-transplantation, the patient was diagnosed with non-Hodgkin B-cell lymphoma (NHBCL), potentially induced by Cephalosporin A therapy. After the treatment with rituximab and CHOP therapy (r-CHOP protocol), remission was determined using computer tomography. Remission is maintained to date. A review of reported cases of post-transplant lymphoproliferative disorders (PTLDs) in liver transplanted (LT) patients showed that the onset of PTLDs is the highest in the first year after transplantation. In addition, remission rates of NHBCL in LT patients are not much elaborated in the literature. It is our opinion that the presented case is rare, both from the aspect of timeline of occurrence of the PTLD and the achieved remission, using r-CHOP protocol. [ABSTRACT FROM AUTHOR]

Primary mediastinal large B-cell lymphoma (PMBCL) is a rare type of non-Hodgkin lymphoma that typically has a good response rate to first line chemotherapy regimens. There have been reports of successful chemotherapy, but with a residual mass from fibrosis. Here, we report the case of a 19-year-old primigravida presenting with cardiogenic shock and superior vena cava (SVC) syndrome at full term who was found to have a PMBCL. Following delivery via urgent cesarean section, she was put on veno-arterial extra corporeal membrane oxygenation (VA-ECMO) and once hemodynamically stable was started on chemotherapy. In view of limited change in tumor size on consecutive CT scans and questionable response to chemotherapy, there were multidisciplinary meetings wherein withdrawing support was discussed and put forward to the family. At that point, surgical debulking was offered on compassionate grounds to be able to wean her off the VA-ECMO. This case report highlights the role of salvage resection when there are no other options. [ABSTRACT FROM AUTHOR]

Background: Reducing diagnostic delays in cancer has been a major interest worldwide; however, the literature on diagnostic delays in lymphoma remains scarce. Diffuse large B‐cell lymphoma (DLBCL) is the most common non‐Hodgkin's lymphoma. We aimed to determine whether certain structural factors predicted diagnostic delays in DLBCL and whether diagnostic delays impacted overall survival (OS). Methods: Data were extracted via a retrospective cohort design from a single academic tertiary care referral center. A total of 104 patients were included. Time from first symptoms to diagnosis of <3 months was defined as "early diagnosis" and ≥3 months as "delayed diagnosis". Analysis was performed with student's t‐test, chi‐square testing, binomial logistic regression, and Kaplan‐Meier log‐rank testing. Results: "Delayed diagnosis" was more likely with lower stage, lower international prognostic index (IPI), and further distance from referral center (OR 0.66, CI 0.46‐0.95; OR 0.69, CI 0.51‐0.94; OR 1.008, CI 1.001‐1.015). Patients of "other" ethnicity and without medical insurance were more likely to have significant diagnostic delays and worse overall survival (P = 0.002 and P = 0.007, respectively). Diagnostic delays of ≥3 months did not predict worse OS. However, delays of >6 months did predict worse OS. Conclusion: Our data suggest that excessive diagnostic delays of more than 6 months, ethnic minority status, and uninsured status in DLBCL may lead to worse outcomes. Efforts should be undertaken to reduce excessive diagnostic delays. More investigation needs to be done on the impacts of diagnostic delays in both DLBCL and other aggressive lymphomas. [ABSTRACT FROM AUTHOR]

Copyright of Acta Médica Peruana is the property of Colegio Medico del Peru and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Background Primary lymphoma of the skull base (PLSB) is an extremely rare neoplasm and not much is known regarding the clinical features of, treatment strategies for, and prognoses of these lesions. We investigated the manifestations and clinical outcomes of Chinese patients with PLSB. Methods We retrospectively reviewed the data from 8 consecutive patients with PLSB who had been treated at our center from October 2008 to December 2016 and analyzed their clinical features, treatment strategies, and prognoses. Results Of the 8 patients, 3 were men and 5 were women, with a male/female ratio of 0.6. The median age was 53.5 years. In most instances, the onset was characterized by headache, followed by dysfunction of the cranial nerves. The most frequently affected region was the cavernous sinus. On magnetic resonance imaging, the main characteristic was that the internal carotid artery was encased by the tumor without an obvious tendency to be deformed, dislocated, or narrowed. All patients underwent surgical biopsy via a transsphenoidal or sublabial–transmaxillary approach. The pathological diagnosis was non-Hodgkin lymphoma for all 8 patients, with diffuse large B-cell lymphoma in 7 patients. The patients underwent chemotherapy and involved-field radiotherapy. The median overall survival was 52 months, and the 1-year progression-free survival rate was 87.5%. Conclusions PLSB is a rare disease and a diagnostic challenge, with most cases in the Chinese due to diffuse large B-cell lymphoma. We found that the incidence peaked in middle and old age, and the cavernous sinus was usually affected. Multidisciplinary treatment involving surgical biopsy, chemotherapy, and radiotherapy was an effective therapeutic strategy. [ABSTRACT FROM AUTHOR]

The article offers a study to demonstrate contemporary data on outcomes of rare forms of paediatric Non-Hodgkin lymphomas (pNHL), a group of blood cancers that includes all types of lymphoma except Hodgkin's lymphomas. Topics discussed paediatric patients with rare forms of T cell NHL which had more guarded prognoses; and performed large and contemporary registry-based analysis which confirms that rare subtypes of paediatric B cell NHL and cutaneous T cell lymphomas have excellent outcomes.

Mantle cell lymphoma (MCL) is a rare, aggressive subtype of non-Hodgkin lymphoma with a poor prognosis and high recurrence rate. It seldom affects the Waldeyer's ring let alone the nasopharynx. Patients usually present at late stages of the disease leading to poor failure-free and overall survival rates. Intensive chemotherapy regimes and autologous stem cell transplantation have reported increased survival rates. We report a relapsed case of nasopharyngeal MCL, which previously occurred in the gastrointestinal tract. The patient had undergone a hemicolectomy for colon intussusception secondary to the intraluminal lymphoma mass. He was unable to complete the treatment regime for MCL due to the adverse side effects. Oropharyngeal mass was discovered during routine outpatient follow-up, which was confirmed as nasopharyngeal MCL. We discuss the prognosis, disease progression, and possible treatments. [ABSTRACT FROM AUTHOR]

Introduction. The research aimed to evaluate the impact of ageadjusted international prognostic index and time to the first relapse on overall survival and progression-free survival from the beginning of the second line of treatment in patients with relapsed/ refractory diffuse large B-cell lymphoma. Material and Methods. The research included 36 patients with relapsed/refractory diffuse large B-cell lymphoma treated at the Oncology Institute of Vojvodina, Serbia, from January 2013 to December 2015. Patients were stratified according to age-adjusted international prognostic index score at the time of relapse into patients with low risk (score 0 - 1) and patients with high risk (score 2 - 3), as well as according to the time of the first relapse: early relapse (≤ 12 months) and late relapse (> 12 months). Results. In the group of patients with a score of 0 - 1, the median overall survival was 44 months compared with 6 months in patients with score of 2 - 3, hazard ratio 0,4 (confidence interval 0,16 - 0,99), p = 0,03. In patients with early relapse, the median overall survival was 7 months compared with 25 months in patients with late relapse, hazard ratio 0,55 (confidence interval 0,25 - 1,19), p = 0,12. In patients with early relapse, median progression-free survival was 0 months compared with 10 months in patients with late relapse, hazard ratio 0,34 (confidence interval 0,12 - 1,00), p = 0,0017. Conclusion. The impact of age-adjusted international prognostic index score significantly affects overall survival in patients with relapsed diffuse large B-cell lymphoma. The time to the first relapse impacts progression-free survival calculated from the time of the second-line treatment initiation. [ABSTRACT FROM AUTHOR]

A 56-year-old man with no previous medical history collapsed and was not able to be resuscitated. The major findings at autopsy were enlargement of the heart (weight = 527 g) which contained an infiltrating firm, homogeneous tumor in an epicardial location adjacent to the left anterior descending and right coronary arteries, with further extension into the right ventricular outflow tract, the interventricular septum and left ventricular free wall. Sections showed sheets of small lymphoid cells with scattered large lymphocytes amounting to a low-grade follicular non-Hodgkin lymphoma, as tumor cells were CD20, CD10, Bcl 2 and LMO 2 positive. This case demonstrates a very rare cause of sudden and unexpected death that resulted from a low-grade follicular lymphoma in an asymptomatic, immunocompetent individual. [ABSTRACT FROM AUTHOR]

Follicular lymphoma (FL) is the most common subtype of indolent non-Hodgkin lymphoproliferative disorders. Treatment strategies for FL may include several therapeutic choices, ranging from a "watchful waiting" approach to stem cell transplantation, mostly depending on staging, age, risk factors, and disease burden at diagnosis. The high radiosensitivity of FL compared with other solid tumors has been known since the beginning of radiotherapy treatment in lymphoma patients. Doses of 24 to 40 Gy were considered appropriate in first line curative treatment for localized disease (stages I-II), but several publications investigating low-dose radiotherapy (LDRT) of 4Gy (2 × 2Gy) reported an overall response rate surprisingly high. Due to its high local efficacy and negligible toxicity, LDRT might be offered to both early and advanced stage FL patients in combination with new agents, at diagnosis or after several lines of systemic therapy. The aim of this review is to summarize and discuss the current knowledge on LDRT for FL and its potential application in a curative setting in combination with new drugs for both early and advanced disease. [ABSTRACT FROM AUTHOR]

T cell lymphoma (TCL) is a group of rare and aggressive diseases. TCL primary to head and neck organs often present as extranodal NK/T cell lymphoma, nasal type. Systemic TCL with initial head and neck presentation is extremely rare. Here we report our institutional experience. Clinicopathologic data was collected from patients diagnosed with TCL and treated at the University of Alabama at Birmingham between 2002 and 2012. Eleven cases of systemic TCL initially presented at head and neck region were identified. The median age was 54 years and male:female ratio was 1.8. The most common sites involved were sinonasal tissue, tonsil, tongue and larynx. Most patients presented with a mass lesion without systemic symptoms. The presentation of TCL primary to the head and neck region is often non-specific. A misdiagnosis of undifferentiated tumor or chronic inflammation due to ambiguous morphology is not uncommon. TCL should be considered in differential diagnosis and a thorough evaluation is warranted for accurate diagnosis. [ABSTRACT FROM AUTHOR]

Lymphoma is a malignant disease of lymphatic cells. Despite numerous sub-types, the disease is generally divided into two main groups: Hodgkin and non-Hodgkin lymphoma. Primary laryngeal non-Hodgkin lymphoma accounts for less than 1% of all laryngeal tumors. We herein present a 43-year-old male patient with difficulty swallowing due to a laryngeal mass. Morphological and immunohistochemical examination revealed T cell lymphoma according to the World Health Organization classification. The patient was referred for chemo-radiotherapy. Primary lymphoma of the larynx is a rare, and primary Tcell laryngeal lymphoma is an even rarer tumor. [ABSTRACT FROM AUTHOR]

Unilamellar liposomes that retain their contents in the systemic circulation can alter the pharmacokinetics of anticancer agents in favorable ways. It has long been recognized that certain liposome compositions may increase the local drug concentration substantially above that achievable with a free drug. We report here that liposomes can alter the in vivo disposition of an entrapped drug not only on a macroscopic but also on a microscopic scale. We show through in vitro studies that intact liposomes composed of distearoylphosphatidylcholine and cholesterol and containing daunorubicin (DaunoXome) are taken up into P1798 tumor cells. These liposomes produce an enhanced cytotoxicity relative to the free drug for incubation times longer than about 8 h. For in vivo studies, we developed and used a noninvasive fluorescence imaging technique to follow the accumulation of liposomal daunorubicin within murine tumors. With this method, we show that the maximum concentration of the available liposomal drug in tumors exceeds that of the free drug, and additionally, liposomal daunorubicin persists at high levels for several days. Total liposome-delivered drug fluorescence from whole tumor extracts peaks at about 8 h. In comparison, the fluorescence intensity of daunorubicin demonstrate persistent high levels of daunorubicin fluorescence within cells and throughout the tumor masses. Free daunorubicin, in contrast, transiently achieves modest levels of fluorescence and rapidly drops to background within a few h. These results indicate distinct mechanisms for the localization of free and liposomal daunorubicin, suggesting that liposmal daunorubicin can provide sustained intracellular levels of the drug within the tumor.

The results of two consecutive multicenter clinical trials enrolled 241 patient with childhood mature B-cells non-Hodgkin lymphomas/leukemia are presented. Patients received treatment according B-NHL 2004mab protocol (n = 83) and B-NHL 2010M (n = 158) with combined immunochemotherapy (ICT) in Russian and Belarus pediatric clinics from 2004 to 2015 years. Primary patients with different mature B-NHL (Burkitt lymphoma/leukemia, diffuse large B-cell lymphoma and primary mediastinal B-cell lymphoma (DLBCL and PMBCL)) aged from 2 to 18 years are included in the studies.Protocol B-NHL 2004mab for treatment of children and adolescents with B-NHL/B-AL, stage III and IV, includes a combination of chemotherapy (PCT) and rituximab – an antibody against the B-cells receptor CD20. PCT courses similar to those in the B-NHL BFM90 protocol (group III) with the exception of methotrexate dose in induction courses, reduced to 1 g/m2 /24 h in order to reduce toxicity. Rituximab (Mabthera, 375 mg/m2 /h) used for the first time in the treatment of children and adolescents with B-NHL. Of the 83 patients included, clinical remission was achieved in 77 (92.8 %). With a median follow time of 51.6 months, remission continued in 23 (85.2 %) patients with B-AL, in 32 (88.9 %) patients with LB and 19 (95.0 %) patients – with DLBCL. With median follow time of 65.2 months, event-free and overall survival was 84 ± 6 and 82 ± 8 %, respectively.Based on previous experience in order to further optimize B-NHL treatment, new protocol B-NHL 2010M with effect-adapted therapy and improvement of stratification risk group criteria was proposed. Overall survival in patients of 1st and 2nd risk groups with full implementation of diagnosis and treatment is approaching 100 %. In interim analysis of 3rd risk group patients, pOS was 88 ± 3 %. The incidence of induction death (infections, metabolic complications) remains within 2.7 % (n = 4); refractory cases (n = 2; 1.3 %) and relapses (n = 4; 2.7 %) developed after 2–4 months of remission, were observed only in patients with Burkitt lymphoma/leukemia. In this cases 2nd line therapy and auto-HSCT is not allowed to achieve remission. All PMBCL and DLBCL patients were achieved remission, but in 50 % of cases only after second line, radio- and cell therapy.The authors conclude that a combined immunochemotherapy of B-NHL in children and adolescents, including the target drug (rituximab) and 5-day courses of cytostatic therapy, highly effective, despite a reduce induction intensity. Therapy for the analyzed protocol requires qualitative dynamic efficacy monitoring and timely correction of therapy. In order to solve a refractory problem and further reduce the toxicity, necessary to continue research using fundamental discoveries in recent years.

Lymphomas of the orbit and orbital adnexae are rare tumors, comprising only 1% of all non-Hodgkin’s lymphoma. Lymphomas are the most common primary orbital tumor in adults of age 60 years and older. Here we present a case of 49 year male with left upper lid swelling since 1 year who was diagnosed histopathologically as non-Hodgkin’s lymphoma of orbital region.

Natural killer (NK) cells are phenotypically and functionally diverse lymphocytes that recognize and kill cancer cells. The susceptibility of target cancer cells to NK cell-mediated cytotoxicity depends on the strength and balance of regulatory (activating/inhibitory) ligands expressed on target cell surface. We performed gene expression arrays to determine patterns of NK cell ligands associated with B-cell non-Hodgkin lymphoma (b-NHL). Microarray analyses revealed significant upregulation of a multitude of NK-activating and costimulatory ligands across varied b-NHL cell lines and primary lymphoma cells, including ULBP1, CD72, CD48, and SLAMF6. To correlate genetic signatures with functional anti-lymphoma activity, we developed a dynamic and quantitative cytotoxicity assay in an integrated microfluidic droplet generation and docking array. Individual NK cells and target lymphoma cells were co-encapsulated in picoliter-volume droplets to facilitate monitoring of transient cellular interactions and NK cell effector outcomes at single-cell level. We identified significant variability in NK-lymphoma cell contact duration, frequency, and subsequent cytolysis. Death of lymphoma cells undergoing single contact with NK cells occurred faster than cells that made multiple short contacts. NK cells also killed target cells in droplets via contact-independent mechanisms that partially relied on calcium-dependent processes and perforin secretion, but not on cytokines (interferon-γ or tumor necrosis factor-α). We extended this technique to characterize functional heterogeneity in cytolysis of primary cells from b-NHL patients. Tumor cells from two diffuse large B-cell lymphoma patients showed similar contact durations with NK cells; primary Burkitt lymphoma cells made longer contacts and were lysed at later times. We also tested the cytotoxic efficacy of NK-92, a continuously growing NK cell line being investigated as an antitumor therapy, using our droplet-based bioassay. NK-92 cells were found to be more efficient in killing b-NHL cells compared with primary NK cells, requiring shorter contacts for faster killing activity. Taken together, our combined genetic and microfluidic analysis demonstrate b-NHL cell sensitivity to NK cell-based cytotoxicity, which was associated with significant heterogeneity in the dynamic interaction at single-cell level.

Richter syndrome or Richter transformation comprises the conversion of chronic lymphocytic leukemia into an aggressive type of large cell lymphoma. Classically, patients have diffuse and abrupt lymphadenopathy and organomegaly, in addition to fever, weight loss, and fatigue. Cutaneous involvement is rare and often nonspecific. We report a patient with chronic lymphocytic leukemia who presented with a large and rapidly evolving ulcer, revealed to be a high-grade cutaneous lymphoma. [ABSTRACT FROM AUTHOR]