Hodgkin's disease: a clinicopathologic study

One hundred and four cases of Hodgkin's disease diagnosed between July 1981 and June 1991 have been analysed. There was a definite male preponderance. Majority of the patients (82.7%) were below the age of 50 years. Mixed cellularity was the most common type (57.7%). It was followed by both nodular sclerosis and lymphocyte predominant types (16.3% each). Lymphocyte depletion Hodgkin's disease, the most aggressive variant, was the least common (9.7%). The detailed observations, as compared to the previous studies in this region as well as in other parts of the world have been presented and discussed.     

Hodgkin's disease presenting with a bronchoesophageal fistula

Bronchoesophageal fistula is a rare complication of Hodgkin's disease. We report a case of Hodgkin's disease presenting with a bronchoesophageal fistula that was successfully treated with bipolar esophageal exclusion and substernal gastric bypass. Direct invasion from mediastinal lymph nodes was the probable cause. Although bronchoesophageal fistula can result as a complication during the course of treatment for Hodgkin's disease, it rarely occurs as the presenting feature. Early recognition and surgical treatment are key aspects of management.     

Hodgkin's disease: a cytokine-producing tumor--a review

Cytokines and hematopoietic growth factors are actively involved in regulation of proliferation, differentiation, and cellular functions of various cell lineages. Each cytokine exhibits pleiotropic biological functions on different target cells and subfamilies of cytokines often have redundant biological effects on the same target cell. Hodgkin's disease represents one of the most common human lymphoma entities, the molecular pathogenesis of which is not well understood. Hodgkin's disease is characterized by the presence of typical, presumed malignant Hodgkin and Reed-Sternberg cells in a hyperplastic background of normal reactive lymphocytes, plasma cells, histiocytes, neutrophils, eosinophils, and stromal cells. The histopathological presentation and characteristic clinical features of Hodgkin's disease correlate with an unbalanced production of multiple cytokines. Hodgkin and Reed-Sternberg cells express mRNA and proteins of various cytokines, growth factors, and cytokine receptors, implying a predominant role for cytokines in the pathophysiology of Hodgkin's disease as a tumor of cytokine-producing cells.     

Vinorelbine: a new promising drug in Hodgkin's disease

Vinorelbine is a new semisynthetic vinca alkaloid that differs chemically from vinblastine by a substitution of the catharanthine moiety. The powerful cytostatic activity of vinorelbine against murine tumors, human malignant cell lines and human tumor xenografts in nude mice has been demonstrated. Phase I-II studies of intravenous vinorelbine, administered weekly as single agent or in combination chemotherapy have been conducted since 1986. Results suggest that vinorelbine has high activity in non-small cell lung cancer, breast cancer and cisplatin-resistant ovarian cancer with mild toxicity, being neutropenia the major treatment related complication. In this paper we critically review the activity of vinorelbine in pretreated Hodgkin's patients. Available results strongly suggest the inclusion of this drug in first or second line chemotherapy regimens in Hodgkin's disease.     

Subcutaneous granuloma annulare with Hodgkin's disease

We report an 85-year-old woman with subcutaneous granuloma annulare. Three nodules developed on her scalp and sacral regions and gradually increased with tenderness. Histologically, a biopsy specimen of the nodule was interpreted as subcutaneous granuloma annulare because palisading granuloma was present in the dermis and subcutaneous fatty tissue. Laboratory tests, including RA, showed no remarkable changes except for hyperglycemia and glycosuria. These nodules spontaneously regressed without medication. One year later, she noted swelling of some lymph nodules in the left axillary region. These were diagnosed as Hodgkin's disease (mixed cellularity type). She was treated with chemotherapy, but died after two months. Subcutaneous granuloma annulare which occurs suddenly in the elderly may be a dermadrome of malignant blood disease.     

Hodgkin's disease: impact on childbearing

Although Hodgkin's disease is considered one of the "curable" cancers, the high cure rates associated with this disease are not accomplished without risk of potentially severe, immediate, and late side effects. Because the predominant histologic subtype of Hodgkin's disease found in the United States generally affects the 15- to 25-year-old age group, the disease, its treatment, and the side effects of treatment will affect many women of childbearing age. The treatment and its effects on the patient and offspring, including posttreatment fertility, are key issues for perinatal nurses.     

Phototherapy for patients with Alzheimer disease with disturbed sleep patterns: results of a community-based pilot study

Twenty of 67 children registered on the International Registry of Childhood Adrenocortical Tumors between May 1988 and December 1994 had small adrenocortical tumors (defined for this study as measuring < or = 200 cm3 and/or weighing < or = 100 g). We reviewed the records of these 20 patients to characterize the clinical and pathologic findings and outcomes of children with small adrenocortical tumors. Median patient age was 2 years (range, 4 months to 5 years). There was only one boy. All had clinical signs of virilization, and seven had signs or symptoms of Cushing syndrome. A median 5.5 months (range, 1-40 months) had elapsed between the first signs of endocrine dysfunction and diagnosis. All tumors were surgically resected. Tumor volume was 3.3-195 cm3 (median, -8.7 cm3), and weight was 3.7-100 g (median, 36 gm Tumor samples were histologically reviewed in 18 cases. Eight were adenomas, and 10 were carcinomas (6 low grade and 4 high grade). Pathology records described tumor with diagnostic features of adrenocortical carcinoma in two patients. One patient received mitotane for 8 months after surgery. Only one patient had recurrent disease, which was detected 6 months after diagnosis and proved rapidly fatal. Another has been lost to follow-up. The remaining 18 patients are alive with no evidence of disease at a median 2.3 years (range, 6 months to 6.1 years) after diagnosis. Our data suggest that children with small adrenocortical tumors have an excellent prognosis with surgery as the sole therapy, regardless of tumor histiotype.     

Deficient major histocompatibility complex class II antigen presentation in a subset of Hodgkin's disease tumor cells

Hodgkin's disease is a common malignancy of the lymphoid system. Although the scarce Hodgkin and Reed-Sternberg (HRS) tumor cells in involved tissue synthesize major histocompatibility complex (MHC) class II and costimulatory molecules such as CD40 or CD86, it is unclear whether these tumor cells are operational antigen-presenting cells (APC). We developed an immunofluorescence-based assay to determine the number of MHC class II molecules present on the surface of single living HRS cells. We found that in fresh Hodgkin's disease lymph node biopsies, a subset of HRS cells express a substantial number of surface MHC class II molecules that are occupied by MHC class II-associated invariant chain peptides (CLIP), indicating deficient loading of MHC class II molecules with antigenic peptides. Cultured Hodgkin's disease-derived (HD) cell lines, however, were found to express few MHC class II molecules carrying CLIP peptides on the cell surface and were shown to generate sodium dodecyl sulphate (SDS)-stable MHC class II alphabeta dimers. In addition to showing deficient MHC class II antigen presentation in a subset of HRS cells, our results show that the widely used HD-cell lines are not ideal in vitro models for the disease. The disruption of MHC class II-restricted antigen presentation in HRS cells could represent a key mechanism by which these tumor cells escape immune surveillance.     

Tuberculosis in Hodgkin's disease in a child

During its evolution, Hodgkin disease is accompanied by changes in the cell immunity, mainly of delayed type. Tuberculosis can precede Hodgkin disease or complicate its evolution during a simultaneous development of both diseases, or the lymph node tuberculosis can hurry hemopathy onset. Tb lesions in such patients can have atypical features.     

Dexa-BEAM in patients with Hodgkin's disease refractory to multidrug chemotherapy regimens: a trial of the German Hodgkin's Disease Study Group

PURPOSE: A prospective phase II study was conducted to evaluate the efficacy of dexamethasone, carmustine, etoposide, cytarabine, and melphalan (Dexa-BEAM) as salvage chemotherapy for patients with Hodgkin's disease. PATIENTS AND METHODS: Fifty-five patients progressing on or relapsing after eight- or 10-drug chemotherapy (cyclophosphamide, vincristine, procarbazine, and prednisone plus doxorubicin, bleomycin, vinblastine, and dacarbazine [COPP+ABVD] or COPP+ABV+ifosfamide, methotrexate, etoposide, and prednisone [IMEP]) were treated with Dexa-BEAM. Patients who responded after two cycles of Dexa-BEAM either continued treatment for another two to three cycles or received high-dose chemotherapy/autologous bone marrow transplantation (HDCT/ABMT) with cyclophosphamide, etoposide, and carmustine (BCNU) (CVB) as conditioning regimen. RESULTS: Seventeen patients (31%) achieved a complete remission and 16 (29%) a partial remission, resulting in a response rate of 60% (95% confidence interval, 46% to 73%). Progressive disease developed in 18 patients. Toxicity of Dexa-BEAM was acceptable with pronounced, but temporary World Health Organization (WHO) grade III/IV granulocytopenia and thrombocytopenia occurring in more than 90% of all courses. Two patients died of sepsis during granulocytopenia. Three prognostic subgroups could be distinguished: (1) patients progressing on initial chemotherapy, (2) patients relapsing within 12 months, and (3) patients with late relapses. The response rates for these groups were 52%, 60%, and 83%, and the median survival duration 12, 29, and 40+ months, respectively. In a nonrandomized comparison, the survival of patients who responded to two cycles of Dexa-BEAM and had additional cycles of Dexa-BEAM (n = 14) was not different from those responding patients who underwent HDCT/ABMT (n = 19). However, the power to detect a 20% survival difference was only 33% in this comparison. CONCLUSION: Dexa-BEAM is an effective salvage treatment for patients with Hodgkin's disease who fail to respond to multidrug chemotherapy. Efficacy and toxicity are comparable to HDCT/ABMT and underline the need for prospective randomized trials to define better the role of HDCT with and without ABMT in these patients.     

Cytogenetics of Hodgkin's disease

Cytogenetic studies over the past 35 years have made a major contribution towards the understanding of the nature of Hodgkin's disease by demonstrating unequivocally the consistent presence of a clonal population of cells that have the cardinal features of malignancy e.g. more or less gross aneuploidy, frequently with complex chromosomal changes and showing considerable variation from case to case, thus comparable to the findings in carcinomas and other solid cancers. The mode is frequently in the triploid-tetraploid region, as we found in 17 of 27 cases studied in this laboratory by Feulgen microspectrophotometry, compared to only 10 cases with neardiploid modes. It is disappointing that no specific change, such as a translocation that could give a clue to the chromosomal location of a gene or genes involved in the etiology of Hodgkin's disease, has yet been found. Nevertheless it is clear that a number of nonrandom changes, including several that are also common in other malignancies including the non-Hodgkin's lymphomas, are frequently present, e.g., deletions of 1p, 6q, and 7q. Interestingly, deletions of 4q, with loss of 4q25 --> q27, that have also been reported may show some specificity for Hodgkin's disease.     

Hodgkin's disease in siblings

An incidence survey of Hodgkin's disease in Greater Boston during 1959-1973 detected five sibling pairs under the age of 45. The expected number is 0.7; thus, siblings of young adults with Hodgkin's disease have about a sevenfold excess risk of the disease (P = 0.0008). Eight sibling pairs, not in the incidence series, were also identified. Among all 13 pairs, 12 were sex concordant; the number expected is 6.8 (P = 0.01). The literature includes 46 sibling pairs under 45 of which 30 are sex concordant. The expected number is 23.9 (P = 0.05). Combining the present and the literature series suggests that siblings of the same sex as an affected person have a risk of Hodgkin's disease double that of siblings of the opposite sex. The sex concordance suggests that the excess Hodgkin's disease among siblings of affected persons is due either to inter-personal transmission of an etiologic agent by prolonged or intimate contact or to common-source exposures.