Use of the World Health Organization (WHO) classification of non-Hodgkin's lymphoma in Mumbai, India: a review of 200 consecutive cases by a panel of five expert hematopathologists

This study aims to answer the question whether the World Health Organization (WHO) classification of non-Hodgkin's lymphoma (NHL) can be practised to international standards at the Lymphoma Registry (LR) established at the Tata Memorial Hospital, Mumbai, India. Furthermore, the study aims to identify differences in the distribution of NHL subtypes at this LR (likely to be representative of India) as compared to the rest of the world. A panel of 5 expert hematopathologists from the NHL Classification Project reviewed 200 consecutive NHL cases at the LR in January of 2001. These cases were accrued during August and September, 2000. On all cases, hematoxylin and eosin stains and appropriate immunostains were available for review. The diagnosis made by the host pathologist at the LR (KNN) and the initial diagnosis made by each of the expert hematopathologists was compared with the consensus diagnosis. A consensus diagnosis was made by the 5 experts in 197 cases. The agreement of the host pathologist with the consensus diagnosis was 82% and the agreement of the individual experts with the consensus diagnosis varied from 76-88% (mean 82%). According to the consensus diagnosis, 80% of NHLs were of B-cell type, 18% were of T-cell type, and the immunophenotype could not be determined in the remaining 2% of cases. In conclusion, the WHO classification of NHL was properly utilized at the Lymphoma Registry, Mumbai, India, and geographic differences were noted in the distribution of NHL subtypes at the LR as compared to the rest of the world. Precursor T lymphoblastic leukemia/lymphoma was more common in India (7%) than the rest of the world (1-4%), and indolent B-cell NHLs (29%) were less common than in the West. As compared to China and Japan, peripheral T-cell lymphoma (4.6%), extranodal NK/T cell lymphoma, nasal type (0.5%) and extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT-lymphoma) (2.6%) were less common, but follicular lymphoma (15%) and chronic lymphocytic leukemia/small lymphocytic lymphoma (5%) were more common. This suggests that the distribution of the B-cell and T-cell lymphomas in the Indian population, except for lymphoblastic lymphoma, lies in between the Western world (mainly Caucasian) and the Orientals.     

Practical Utility of the Revised European-American Classification of Lymphoid Neoplasms for Japanese Non-Hodgkin's Lymphomas

A clinicopathological study of 515 non-Hodgkin's lymphoma (NHL) cases was performed using the revised European-American classification of lymphoid neoplasms (REAL classification) in an HTLV1-nonendemic area of Japan. The following characteristics were revealed: 1) frequency of extranodal lymphomas was high (59%) with 79% B-cell lymphomas in this series, while the overall ratio of B:T/NK lineage was 3.7:1; 2) the most common type was the diffuse large B-cell lymphoma (46%), follicle center lymphomas occurred at an incidence lower (15%) than that in European and American populations, and marginal zone B-cell lymphomas accounted for as much as 12%; 3) peripheral T-cell lymphomas were common (19%), with the unspecified type predominant (11%), while adult T-cell lymphomas were present at a level equivalent to that among European and American patients (1%). Clear segregation of survival curves was rated according to cell lineage and B-cell lymphomas had a better prognosis than T/NK-cell lymphomas. Furthermore, new subtypes in the REAL classification, such as marginal zone B-cell and mantle cell lymphomas, exhibited distinct curves. Taken altogether, the REAL classification demonstrated advantages for assessment of Japanese NHL cases.     

Practical utility of the revised European-American classification of lymphoid neoplasms for Japanese non-Hodgkin's lymphomas.

A clinicopathological study of 515 non-Hodgkin's lymphoma (NHL) cases was performed using the revised European-American classification of lymphoid neoplasms (REAL classification) in an HTLV1-nonendemic area of Japan. The following characteristics were revealed: 1) frequency of extranodal lymphomas was high (59%) with 79% B-cell lymphomas in this series, while the overall ratio of B:T/NK lineage was 3.7:1; 2) the most common type was the diffuse large B-cell lymphoma (46%), follicle center lymphomas occurred at an incidence lower (15%) than that in European and American populations, and marginal zone B-cell lymphomas accounted for as much as 12%; 3) peripheral T-cell lymphomas were common (19%), with the unspecified type predominant (11%), while adult T-cell lymphomas were present at a level equivalent to that among European and American patients (1%). Clear segregation of survival curves was rated according to cell lineage and B-cell lymphomas had a better prognosis than T / NK-cell lymphomas. Furthermore, new subtypes in the REAL classification, such as marginal zone B-cell and mantle cell lymphomas, exhibited distinct curves. Taken altogether, the REAL classification demonstrated advantages for assessment of Japanese NHL cases.     

Clinicopathological evaluation of the Revised European-American Classification of Lymphoid Neoplasms (REAL) in Japan.

After the publication of a Revised European-American Classification of Lymphoid Neoplasms (REAL classification) in 1994, there have been reports from Europe and America regarding its practical utility and clinical significance. However, no studies have been published from Eastern countries including Japan. It has been well recognized that the distribution of malignant lymphoma in Japan is quite different from that seen in Western countries. In addition, some new entities have also been described in the REAL classification. Therefore, it seems important to examine its practical utility and clinical significance in Japan. Of the 579 cases reviewed, approximately 68% were B-cell non-Hodgkin's lymphoma (NHL) followed by 27% T-cell lymphomas. Hodgkin's disease (HD) comprised only 5% of all cases, making the ratio of NHL to HD 20.6. The most common type was diffuse large B-cell lymphoma which represented about 37% of all cases. Peripheral T-cell lymphomas, unspecified (PTCL), occurred in 15% whereas marginal zone B-cell lymphoma followed (14.9%). However, follicle center lymphoma (FCL) was less common (4.4%) as has been previously reported. We evaluated the clinical significance of the new REAL classification in 244 cases. International Prognostic Index (IPI) was a powerful predictor of survival (p<0.0001), and the immunophenotype was significant (p<0.05). Furthermore, here, we also attempt to establish a prognostic scheme based on the histologic type. In conclusion, the REAL classification appears to be useful and clinically significant in Japan.     

Distribution and prognosis of WHO lymphoma subtypes in Taiwan reveals a low incidence of germinal-center derived tumors

To assess the distribution of lymphomas in Taiwan according to the WHO (World Health Organization) classification, 175 recently diagnosed cases of malignant lymphomas were studied and the clinicopathologic data were analyzed. B-cell lymphomas accounted for 57.1% of cases, T-cell lymphomas 38.9%, and Hodgkin's lymphoma 4%. Extranodal lymphomas predominated (55.4%). The most common subtype of B-cell lymphoma was diffuse large B-cell lymphoma (33.1%). All tumor types believed to be derived from germinal center (GC) B-cells including follicular lymphoma (4.6%), Burkitt lymphoma (1.7%), Hodgkin lymphoma (4.0%), and GC-like diffuse large B-cell lymphoma (as defined by combined expression of bcl-6 and CD10) were rather uncommon as compared to frequencies seen in series from Western countries. The common T-cell lymphomas included nasal and extranasal NK/T cell lymphoma (7.4%), mycosis fungoides (7.4%), and unspecified peripheral T-cell lymphoma (6.9%). Adult T-cell leukemia/lymphoma was very uncommon and accounts for only 0.6%. The proportional increase in T-cell lymphomas that were unrelated to type I human T-cell lymphotropic virus (HTLV-1) may be linked to differential Epstein-Barr virus (EBV) oncogenesis. The survival data revealed that mantle cell lymphoma, NK/T-cell lymphoma, unspecified peripheral T-cell lymphoma, and subcutaneous panniculitis-like T-cell lymphoma had an aggressive course. Our results confirm the utility of the WHO classification scheme for prognostic stratification and further highlight the distinctive distribution pattern of malignant lymphoma in Taiwan including the higher relative incidence of T cell lymphomas and the rarity of germinal center-derived B-cell tumors.     

WHO non-Hodgkin's lymphoma classification by criterion-based report review followed by targeted pathology review: an effective strategy for epidemiology studies.

In a previous criterion-based pathology report review of 717 cases of non-Hodgkin's lymphoma in an Australian population-based epidemiologic study, a WHO category could be assigned in 91% of cases, but confidence in this classification was high in only 57.5%. Given this lack of confidence, a pathology review was done in a subset of 315 cases, with the aims of assigning a WHO classification category and the corresponding International Classification of Diseases for Oncology, Third Edition code in all cases previously unclassified or classified with low confidence and testing the accuracy of report review in assigning a confident WHO classification. After pathology review, 10 cases were ineligible (not non-Hodgkin's lymphoma, 3.2%) and 99% (301 of 305) of the remainder were assigned a WHO classification, with high confidence in 87% (261 of 301). There was 78% overall agreement between the WHO classification assigned by report review and pathology review, with 92% agreement when there was high confidence in the report review classification and 69% agreement when there was low confidence. Eighteen percent of follicular lymphomas and 23% of diffuse large B-cell lymphomas were reclassified. The pathology review increased the accuracy of WHO classification by an estimated 12.5% in the 694 cases who were still eligible in the study. Although a potential error rate of 7.5% remained, reviewing more cases, or not reviewing any cases classified with high confidence, would have produced only a small change in accuracy. Criterion-based pathology report review of all cases followed by selective pathology review in cases classified with low confidence is recommended as a cost-saving and accurate strategy for pathology review in large epidemiologic studies.     

Clinicopathological features of extranodal lymphomas: Kuwait experience

A total of 935 patients with extranodal non-Hodgkin lymphoma (NHL) diagnosed in the period between January 1985 and December 2000 in Kuwait Cancer Center, serving the whole population of Kuwait, were used to describe the clinicopathological and epidemiological features of extranodal lymphomas in Kuwait. Extranodal lymphomas accounted for 45% of all NHL observed during this time. All NHL cases from Kuwait Cancer registry were analyzed and pathologically reclassified using the latest WHO (2000) classification. The most common lymphoma observed was diffuse large B-cell lymphoma (58.60%) followed by Burkitt s lymphoma (BL) (3.80%). In the pediatric group, BL comprises more than two thirds of all patients (77.20%). The most common extranodal sites were stomach (19.70%) and skin (17.80%) in the adult group, large intestine (29.80%) and small intestine (19.30%) in the pediatric age group. The majority (73.40%) of adult extranodal lymphomas was in stage IE-IIE and had a very good prognosis. On the contrary, the majority of pediatric extranodal lymphomas were found to be in stage III and IV. Variations in treatment policies (single agent or combined chemotherapy, radiotherapy, combined modality treatment) adopted and changed during the time period of 16 years of this retrospective study were documented.     

Use of the WHO lymphoma classification in a population-based epidemiological study.

BACKGROUND: Non-Hodgkin's lymphoma (NHL) is pathologically diverse. Epidemiological investigations into its increasing incidence and aetiology require accurate subtype classification. PATIENTS AND METHODS: Available pathology reports of 717 cases aged from 20 to 74 years in an Australian, population-based epidemiological study of NHL were reviewed by one anatomical pathologist to assign a World Health Organization (WHO) classification category. High or low confidence was assigned to the diagnosis of NHL, cell phenotype and WHO category and reasons given for low confidence. RESULTS: The most informative biopsy reports were from open tissue biopsy (79% of cases), tissue core biopsy (8%), cytology (4%) and bone marrow (9%); 8% of cases had inadequate biopsies for diagnostic purposes. Immunohistochemistry or flow cytometry reports were available for 96% of cases, gene rearrangement studies for 6% and cytogenetics for 3%. The reviewer assigned high confidence to the diagnosis of NHL in 93% of cases and also the phenotype in 88%. While a WHO classification could be assigned in 91% of cases, confidence was high in only 57.5%; insufficient immunophenotyping was the commonest reason for low confidence. CONCLUSIONS: Expert pathology review of a population-based sample of NHL can provide a WHO classification category for most cases. A high level of confidence in the classification, however, would require review of diagnostic material and additional phenotyping.     

恶性淋巴瘤1126例临床特点分析

 目的　分析恶性淋巴瘤患者住院的临床特点。方法　从该院病案数据库提取2005年1月至2009年12月住院恶性淋巴瘤患者的资料,剔除未能明确病理分型及重复入院的病例,从年龄、性别、病理类型、肿瘤起病部位及分期等方面进行分析、总结。结果　住院的恶性淋巴瘤患者1126例,男女比例为1.94∶1。霍奇金淋巴瘤（HL）患者年龄集中在20～40岁,以混合细胞型（64.16 %）、结节硬化型（29.48 %）为主。非霍奇金淋巴瘤（NHL）患者年龄以50~70岁为多,发病率位于前10位的为弥漫大B细胞淋巴瘤（53.31 %）、结外NK／T细胞淋巴瘤（7.35 %）、套细胞淋巴瘤（6.40 %）、B细胞慢性淋巴细胞白血病／小淋巴细胞淋巴瘤（4.30 %）、间变性大细胞淋巴瘤（4.09 %）、前T细胞淋巴母细胞白血病／淋巴瘤（3.88 %）、外周T细胞淋巴瘤（非特指）（3.46 %）、血管免疫母细胞型淋巴瘤（3.04 %）、滤泡性淋巴瘤（2.94 %）、伯基特淋巴瘤（2.52 %）。两者起病部位均以颈部淋巴结常见。结论　HL和NHL发病存在性别、年龄、病理类型、起病部位等差异。     

Simian virus 40 sequences in malignant lymphomas in Japan

Recent studies showed that SV40 is detected in >40% of non-Hodgkin's lymphoma (NHL) in United States, suggesting SV40-contaminated poliovaccines widely used during the period 1955-1963 to be a major source of SV40 in NHL. We examined the presence of SV40 sequences in 122 cases with NHL and 3 with Hodgkin's lymphoma from Japan. The detection rate of SV40 sequences in diffuse large B-cell lymphoma (19%) was higher than that in peripheral blood cells of normal healthy volunteers in Japan (4.7%; P < 0.05) reported previously as controls for comparison with the study results from cancer patients, suggesting a role for SV40 in the development of diffuse large B-cell lymphoma. In contrast, the frequency of SV40 sequences in NHL cases born between 1951 and 1963 (12%), during which SV40-contaminated poliovaccines might have been inoculated, is not significantly different from that in cases born before 1950 (11%) or after 1964 (15%). SV40 is a new candidate etiologic factor for malignant lymphoma not only in the United States but also in Japan.     

Mature B-cell lymphoma/leukemia in children and adolescents: intergroup pathologist consensus with the revised European-American Lymphoma Classification.

Background:The Revised European–American Lymphoma(R.E.A.L.) Classification criteria were evaluated in the internationalprotocol FAB LMB 96 Treatment of Mature B-Cell Lymphoma/Leukemia: A SFOP LMB96/CCG-5961/UKCCSG NHL 9600 Cooperative Study. This includes B-lineagelymphomas: Burkitt's lymphoma (including ALL-L3); high-grade B-cell lymphoma,Burkitt-like; diffuse large B-cell lymphoma (excluding anaplastic large cellKi-1 lymphoma). Patients and methods:Cases were independently reviewed by eighthematopathologists from the three cooperative national groups (two SFOP, twoCCG, four UKCCSG), without prior discussion of classification criteria orguidelines for case rejection. Consensus diagnosis was determined by eachnational cooperative group, and final consensus diagnosis established when atleast two national consensus diagnoses were in agreement, or following groupagreement at a multiheaded microscope. Results:Two hundred eight cases were reviewed, with finalconsensus diagnosis established in two hundred three. The percent agreementof each group's national consensus diagnosis with final consensus diagnosiswas 86%, 86% and 71%. The percent agreement of thegroup's national consensus diagnosis with final consensus diagnosis forBurkitt's and diffuse large B-cell lymphoma were 88% and 80%,respectively, but only 42% for Burkitt-like lymphoma. Conclusions:International panel review of mature B-celllymphoma/leukemia in children and adolescents highlighted difficulties insubclassification, particularly with Burkitt-like, which is a 'provisionalentity' in the R.E.A.L. Classification. The absence of previous discussion ofclassification and guidelines for case rejection may in part explain thediscrepancy between pathologists. These results underline that morphology mayneed to be complemented by other studies, such as molecular genetics andcytogenetics, to discriminate between the mature B-cell lymphomas.     

Non-Hodgkin lymphoma in Chile: a review of 207 consecutive adult cases by a panel of five expert hematopathologists

The distribution of subtypes of non-Hodgkin lymphoma (NHL) in Latin America is not well known. This Chilean study included 207 consecutive cases of NHL diagnosed at five cancer centers in the capital, Santiago, and one center in Vi?a del Mar. All cases were reviewed and classified independently by five expert hematopathologists according to the 2001 World Health Organization classification of NHL. A consensus diagnosis of NHL was reached in 195 of the 207 cases (94%). B-cell lymphomas constituted 88% of NHL, and diffuse large B-cell lymphoma (DLBCL, 38.5%) and follicular lymphoma (25.1%) were the most common subtypes. There was a high frequency of marginal zone B-cell lymphoma (10.3%), as well as of extranodal natural killer (NK)/T-cell lymphoma, nasal type (2.6%) and adult T-cell leukemia/lymphoma (0.5%). Extranodal presentation was seen in 74 of the 195 cases (38%) and the most common extranodal presentation was in the stomach (37.6%). The most common gastric lymphoma was DLBCL (54.5%) followed by mucosa-associated lymphoid tissue (MALT) lymphoma (41%). Overall, the frequency of NHL subtypes in Chile is between that reported in Western and Eastern countries, which is probably a reflection of the admixture of ethnicities as well as the environment and socioeconomic status of its population.     

AIDS-Related Non-Hodgkin Lymphoma: Imaging Feature Analysis of 27 Cases and Correlation with Pathologic Findings

Background: Some tumor types are related to HIV, including non-Hodgkin lymphoma (NHL). The morbidityand mortality of NHL has remained high, even after highly active antiretroviral therapy (HAART) was introduced.We collected cases of AIDS with NHL, and evaluated the imaging features and strategies for diagnosis. Materialsand Methods: There were 27 patients with AIDS and tumors confirmed by pathology. There were 9 patients withBurkitt lymphoma, 16 with diffuse large B cell lymphomas (DLBCLs), and 2 with primary central nervous system(PCNS) lymphomas. All of the patients underwent a series of imaging studies. Three radiologists analyzed theimages, and any disagreement was discussed until consensus was reached. Results: The radiologic manifestationsof AIDS with NHL were mainly masses and lymphadenopathy, 3 patients having one mass and 12 two or moremasses. 7 patients had lymphadenopathy in one site and 3patients had lymphadenopathy in two or more sites.Coarse mucosal folds, thickening of the gastrointestinal wall, and lumen narrowing were typical manifestationsof NHL within the gastrointestinal tract. There were 4 patients with masses and 5 with lymphadenopathyinthe 9 with Burkitt lymphoma, and 11 patients with masses 5 with lymphadenopathy in the 16 with DLBCLs.Conclusion: NHL is a malignancy that usually occurs in patients with AIDS. Imaging is an important method bywhich to evaluate lesions, masses, and lymphadenopathy. Fine needle aspiration biopsy and stereotaxis biopsyare useful methods by which to diagnose NHL.     

A review of childhood cases of malignant lymphoma in Osaka, Japan

Fifty-one cases of malignant lymphomas in patients under 20 years of age have been reviewed in Osaka, Japan. The breakdown of these cases revealed 6 cases (11.8%) of Hodgkin's disease (HD) and 45 cases (88.2%) of a non-Hodgkin's lymphoma (NHL). Both the HD and NHL cases were determined by using the Rye classification, as well as the Rappaport, Kiel, and LSG classifications, respectively. The results have shown that (1) the incidence of HD in childhood is the same as that seen in the adult in Japan; (2) that a NHL of the nodular type is rare in Japan as it is in Western countries; (3) that any significant differences were not present in the distribution of each histologic subtype in the cases of a NHL among Japan and Western countries, and that the lymphoblastic type was the most common. From this study it is concluded that geographical differences were not a factor in comparing cases of childhood malignant lymphomas in Japan and Western countries.     

Head-and-neck stages I and II extranodal non-Hodgkin's lymphomas: real classification and selection for treatment modality

PURPOSE: We re-evaluated histopathological specimens of head and neck early-stage extranodal non-Hodgkin's lymphoma (NHL) using the revised European and American lymphoma (REAL) classification, and also investigated the relationship between the clinical characteristics and histopathological classification in an attempt to evaluate the usefulness of this new classification system in selecting treatment modalities. MATERIALS AND METHODS: Between 1979 and 1995, 117 patients with histologically confirmed stages I and II NHL of head-and-neck extranodal regions were treated. Of these patients, 110 specimens were available for re-evaluation. Sixty-four patients had Stage I, and 46 had Stage II diseases. All but 3 had received radiation therapy, and 59 patients were also treated with intensive combination chemotherapy. RESULTS: There were 32 extranodal marginal-zone B-cell lymphomas, 57 diffuse large B-cell lymphomas, 11 peripheral T/NK-cell lymphomas, and 10 others. The 5- and 10-year cause-specific survival rates for all patients were 72% and 62%, respectively. Patients with extranodal marginal-zone B-cell lymphoma or other low-grade B-cell lymphomas demonstrated higher survival rates than patients with other lymphomas. Patients with peripheral T/NK lymphomas showed the lowest survival rate. CONCLUSION: The REAL classification accurately indicated the prognosis of patients with NHL. These results suggest that appropriate treatment modalities can be selected using this classification.     

Changes in cancer registry coding for lymphoma subtypes: reliability over time and relevance for surveillance and study.

Because lymphoma comprises numerous histologic subtypes, understanding the reasons for ongoing increases in its incidence requires surveillance and etiologic study of these subtypes. However, this research has been hindered by many coexisting classification schemes. The Revised European American classification of Lymphoid Neoplasms (REAL)/WHO system developed in 1994 and now used in clinical settings was not incorporated into the International Classification of Diseases-Oncology (ICD-O), used by cancer registries, until the release of the third edition (ICD-O-3) in 2001. Studies including patients diagnosed before 2001 may have codes from earlier ICD-O versions that must be converted to ICD-O-3 and have higher proportions of unclassified (e.g., lymphoma and not otherwise specified) cases. To better understand (a) the agreement of computer-converted ICD-O-3 codes to ICD-O-3 codes generated directly from diagnostic pathology reports and (b) the reproducibility of unclassified status, we reviewed a population-based series of diagnostic pathology reports for lymphoma patients diagnosed before (1988-1994; n = 1,493) and after (1998-2000; n = 1,527) the REAL/WHO scheme was introduced. Overall, computer- and coder-assigned ICD-O-3 codes agreed for 77% of patients in both groups and improved slightly (82%) when codes were grouped. The most common lymphoma subtypes, diffuse large B cell and follicular, had relatively good reliability (84-89%) throughout the study period. T-cell and natural killer cell lymphomas had worse agreement than B-cell lymphomas, even when grouped. Many (42-43%) lymphomas reported as unclassifiable could be assigned a subtype upon pathology report review. These findings suggest that the study of lymphoma subtypes could be improved by (a) use of more standardized terminology in pathology reports, (b) grouping individual ICD-O-3 codes to reduce misclassification bias, and (c) routine secondary editing of unclassified lymphomas by central cancer registries.     

Lymphome bei Kindern und Jugendlichen

Background

Lymphomas represent more than 15?% of malignancies in children and adolescents.

Objective

This review article provides an overview on the state of the art diagnostic evaluation and treatment of malignant lymphomas in children and adolescents.

Material and methods

Data obtained from peer reviewed publications of the involved study consortia and by a PubMed search are presented.

Results

According to the World Health Organization (WHO) classification of Hodgkin’s lymphoma a differentation is made between classical (90?%) and nodular, lymphocyte-predominant Hodgkin’s lymphomas. Non-Hodgkin’s lymphomas (NHL) in children and adolescents are classified according to the WHO classification based on the immunophenotype and developmental stage of the lymphoid cells. The mature B?cell NHL (Burkitt lymphoma 46?% and diffuse large cell B?NHL 8?%) represent more than half of pediatric NHL, the other main subtypes are the precursor B and T?cell NHL (lymphoblastic lymphoma 21?%) and anaplastic large cell lymphoma (ALCL 13?%). With increasing age the distribution of subtypes changes towards lymphomas more typical for an adult population. The grey zone lymphomas are entities that cannot be clearly assigned to either HL or NHL and are so defined in the WHO classification. The exact diagnosis of NHL and HL is essential for the subtype-specific treatment. Currently, survival rates of more than 80?% (NHL) and 97?% (HL) can be achieved.

Discussion

Especially in the treatment for HL, the principles are quite different from those used in adult patients. Avoidance of late effects while maintaining high cure rates are the main objectives; therefore, children and adolescents up to the age of 18 years should exclusively be treated within trials provided at pediatric oncology centers.

     

Characteristics of indolent non-Hodgkin lymphoma in patients with type 1 human immunodeficiency virus infection

BACKGROUND: There is recent evidence that the incidence of indolent non-Hodgkin lymphoma (NHL) appears to be increased in persons with the acquired immunodeficiency syndrome (AIDS). The current study was conducted to describe the clinical, immunologic, and pathologic characteristics of indolent B-cell lymphoma in patients infected with the human immunodeficiency virus (HIV). METHODS: The current report was a retrospective study of 10 cases of indolent NHL identified from the AIDS-Lymphoma Registry at the University of Southern California School of Medicine. These patients were compared with 336 consecutive patients with systemic intermediate/high-grade AIDS-related NHL who were diagnosed and treated at a single institution. RESULTS: The pathology of the indolent cases included follicular lymphoma (five patients), small lymphocytic lymphoma (two patients), and one case each of mucosa-associated lymphoid tissue (MALT), monocytoid B-cell, and marginal zone lymphoma. When comparing the indolent lymphomas with the intermediate/high-grade AIDS-NHL cases, no differences were observed with regard to demographic characteristics or history of prior opportunistic infection. HIV patients with indolent lymphomas were found to have a significantly higher median CD4+ lymphocyte count compared with patients with intermediate/high-grade NHL (531 /mm3 vs. 90 /mm3) (P < 0.0001). Bone marrow involvement was significantly more common in indolent NHL cases (50%) versus intermediate/high-grade NHL cases (17%) (P = 0.02). The median survival for patients with indolent NHL was significantly longer compared with patients with intermediate/high-grade NHL (66.8 months vs. 7.1 months) (P = 0.007). CONCLUSIONS: Indolent lymphomas occurring in patients infected with HIV appear to differ from intermediate/high-grade lymphomas with regard to immune status and propensity for bone marrow involvement and prolonged survival. The median survival in the group of HIV-seropositive patients with indolent NHL examined in the current study was found to be comparable to that reported in HIV-negative individuals.     

Evaluation and management of the “New” lymphoma entities: Mantle cell lymphoma, lymphoma of mucosa-associated lymphoid tissue, anaplastic large-cell lymphoma, and primary mediastinal B-cell lymphoma

Non-Hodgkin's lymphomas (NHL) represent a major health problem worldwide, and incidence has been on the rise continuously for the last few decades. It is estimated that approximately 55,000 new cases of NHL will be diagnosed in the United States in 1998 and that slightly fewer than 25,000 patients will die of treatment failure or recurrent disease. The rising incidence of NHL is related not only to the acquired immunodeficiency syndrome epidemic but to also a steady increase in the number of cases diagnosed in older patients without immunosuppression. The new pathologic classification of NHL (revised European-American lymphoma classification, REAL) developed by the International Lymphoma Study Group (ILSG) is already resulting in more accurate disease-specific epidemiologic and clinical investigations. These studies have brought a new awareness of the existence and the relative prevalence of discrete NHL subtypes that appear to predominate among patients in different populations according to age, sex, geographic distribution, and predisposing conditions. This developing database has also the potential to result in the discovery of specific environmental causes, predisposing genetic factors, and therapeutic approaches. Some of the entities defined in the REAL classification, such as follicular lymphomas, diffuse B large-cell lymphomas, and T-cell lymphoblastic lymphomas, were already well described in the older classification systems (Kiel and Working Formulation). Others, such as mantle cell lymphoma, (MCL) anaplastic large-cell lymphoma (ALCL), lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma), and primary mediastinal B-cell lymphoma (PMBCL) are relatively new members of the family, and accurate data on their clinicopathologic features and natural histories have only recently begun to emerge.This review presents in detail the most recent data on the clinical presentation of, diagnostic evaluation of, and treatment options for the most common of the new NHL entities: MCL, MALT lymphoma, CD30⁺ (Ki-1⁺) ALCL, and PMBCL. These four entities combined represent approximately 20% of all cases of NHL and exemplify well the broad clinicopathologic spectrum of NHL and the diagnostic and therapeutic challenges facing those who care for patients affected by these conditions.     

Malignant lymphoma in the Miyazaki district: analysis of 237 biopsy cases

Pretreatment biopsy specimens of 237 consecutive patients with malignant lymphoma, who presented to us from 1979 to 1982, were reviewed and reclassified. According to the new classification proposed by the Lymphoma Study Group of Japan (LSG), there were 226 patients with non-Hodgkin's lymphoma (NHL) which was further classified as diffuse lymphoma (216 cases), follicular lymphoma (4 cases), mycosis fungoides (4 cases), and others (2 cases). The 216 cases of diffuse NHL were subdivided into small cell (2 cases), medium-sized cell (71 cases), mixed (7 cases), large cell (92 cases), pleomorphic (40 cases), lymphoblastic (3 cases), and Burkitt's type (1 case) lymphoma. Cell surface marker studies using conventional methods were performed on 65 NHL patients, of whom 45 showed T-cell marker and 11 B-cell marker, and 8 had neither marker. The average survival periods were 13.1 mo for 107 patients with NHL, 27.9 mo for those with mycosis fungoides and 70.0 mo for 10 patients with Hodgkin's disease. Patients with adult T-cell leukemia survived for an average of only 5.5 mo. Histologically diffuse pleomorphic type had the worst prognosis. T-cell lymphoma appeared to have a poorer prognosis than B-cell lymphoma.