Lymphocyte-predominant Hodgkin disease in children

PURPOSE: To describe the clinicobiological features, treatment, treatment outcome, and sequelae of children with lymphocyte-predominant Hodgkin disease. PATIENTS AND METHODS: The authors performed a retrospective chart review of 754 patients with Hodgkin disease diagnoses at New York Medical College and St. Jude Children's Research Hospital from 1962 to 2000 to identify those with lymphocyte-predominant histology. Hematopathologists at the treating institutions reviewed stored tissue specimens and reconfirmed the histopathology of each case. RESULTS: Fifty-one children (44 boys, 7 girls) were identified. The median age was 10.5 years (range 3.2-18.5); five children were younger than age 60 months. The median duration of lymphadenopathy before diagnosis was 4 months (range 0.5-30). Thirty-six children had stage 1 disease, eight had stage 2 disease, four had stage 3 disease, and three had stage 4 disease. Fifteen children underwent staging laparotomy, and four of these were upstaged. Treatment comprised combined modality therapy (n = 27), radiation therapy alone (n = 17), and chemotherapy alone (n = 7). Four children had a Hodgkin disease recurrence. Forty-eight (94%) patients were alive and disease-free at a median follow-up of 8 years (range 0.4-32.6). Eleven patients had long-term, therapy-related adverse effects (cardiac, infertility, pulmonary, and second malignant neoplasms). Three patients died. Two died of complications of second malignant neoplasms and one died of infectious complications after Hodgkin disease recurrence. CONCLUSIONS: Children with lymphocyte-predominant Hodgkin disease respond favorably to a variety of treatment modalities and are ideal candidates for less toxic therapy.     

Hodgkin disease in Iranian children

The epidemiological pattern of Hodgkin disease (HD) was studied in 139 Iranian children with an age range of 2.5–15 years (mean age 8.3) over a 17-year period (1972–1989). The main features observed were: a high male to female ratio of 3.21, a high frequency of HD in toddlers and young school children (73%), the prevalence of mixed cellularity subtype (74.5%) and rare occurrence of lymphocyte depletion subtype (2%) the high occurrence of constitutional symptoms (57%) and the high rate of stage III and IV patients (73.5%). Response to the treatment consisting mainly of chemotherapy and/or radiotherapy was favourable and overall and disease-free survival rates of 91.4% and 74.2% were achieved over a follow up period of 6 months –17 years. According to these findings the epidemiological pattern of Iranian children with HD is similar to that occurring in rural areas of developed countries.     

The management of Hodgkin disease in the young child

Although childhood Hodgkin disease is sensitive to the treatment regimens devised for Hodgkin disease in adults, long-term toxicity is enhanced in the developing individual. As a result, there have been dual goals in the design of clinical trials for pediatric Hodgkin disease: 1) to reduce long-term organ injury; and 2) to increase efficacy. Radiation dose and field has been reduced by enhanced reliance on chemotherapy, thus limiting the risks of hypoplasia, hypothyroidism, secondary cancers, and valvular and atherosclerotic heart disease. Multiagent, chemotherapeutic regimens for children have been developed to avoid the risks of sterility, leukemia, and cardiopulmonary toxicity. Newer approaches advocate for early dose intensity to limit cumulative therapy using response-based paradigms. Targeting molecular mechanisms specific for the Reed-Sternberg cell may allow for less toxic and more efficacious treatments in the future.     

Coexistence of Hodgkin lymphoma and cyst hydatic disease of the liver

Although both Hodgkin lymphoma and cyst hydatic disease in children have been seen with an increased frequency, there is no previously reported case of Hodgkin lymphoma associated with cyst hydatic disease from Turkey. The authors report such a case of Hodgkin lymphoma. Intrahepatic cystic masses were diagnosed during ultrasound examination for clinical staging on admission. The diagnosis of cyst hydatic of the liver was confirmed by surgery. Although there was no residual and/or new cyst formation on radiologic follow-up, elevated antibody titers (indirect hemagglutination test) persisted following surgical excision at least for 2 years of follow-up.     

Relapse of Hodgkin disease after extended-field radiotherapy

Thirteen relapses occurred among 51 consecutive surgically staged patients with I, IIA, and IIIA Hodgkin disease who achieved complete remission following extended-field radiotherapy. The median disease-free interval was 14 months, but three disseminated relapses occurred at 36, 36, and 39 months. In 6 of the 13 cases, relapses occurred within treated fields. Relapse frequency was greater with advancing stage, but was not related to histology. In 9 of 12 evaluable cases the patients achieved complete remission with MOPP combination chemotherapy. All responders continue in complete remission a median time of 23 months after completing MOPP. This report emphasizes the need for sustained, close follow-up after radical radiotherapy and the excellent response rate of relapsed patients to MOPP combination chemotherapy.     

Hodgkin disease as the terminal malignancy in richter syndrome

A case of Richter syndrome in which autopsy revealed typical infiltrates of Hodgkin disease, including Reed—Sternberg cells, is reported. Previous reports of this association have met with skepticism. Clinical findings in patients with Hodgkin infiltrates do not differ from those with histiocytic lymphoma, except for the more frequent occurrence of a declining lymphocyte count. The present case is further evidence that Hodgkin disease does occur as a terminal malignancy in Richter syndrome.     

A role for surgery in the treatment of relapsed Hodgkin lymphoma

Treatment of Hodgkin lymphoma (HL) has advanced over time, rendering a fatal disease now largely curable. Multiagent chemotherapy regimens, hematopoietic stem cell transplantation, and radiotherapy are the mainstays of care. Surgical intervention is rarely indicated other than for biopsy at diagnosis. However, for patients with recurrent relapsed HL isolated to one anatomical location, refractory to all other therapy, there may be a beneficial role for surgical excision. Herein, we report the surgical management of three relapsed patients with stage IVB HL who were refractory to multiple other therapeutic approaches, who all achieved good event‐free survival after operative management.     

伴有纵隔淋巴结巨大病变的霍奇金病诊治分析

目的加强对伴有纵隔淋巴结巨大病变霍奇金病 (HD)的认识 ,改进治疗方法 ,提高缓解率 (CR)和长期无病生存率 (EFS)。方法将43例HD分为伴纵隔淋巴结巨块病变组 (巨块组 )、伴纵隔淋巴结非巨块病变组 (非巨块组 )、无纵隔淋巴结病变组 (无块组 ) ,回顾分析其临床特点、治疗和预后。结果巨块组Ⅲ、Ⅳ期占85.7 % ,与非巨块组、无块组比较P<0.05;B组 (有全身症状者 )占64.3 % ,较非巨块组高 (P<0.05)、与无块组比P<0.01 ;诊断时巨块组(64.3 %)出现上腔静脉压迫综合征 (SVCS)或上纵隔压迫综合征 (SMCS)高于非巨块组 (9.1 % ) ,P<0.01。均给予MOPP ,COMP或与ABVD交替治疗 ,部分加放疗 ,巨块组的CR率 (62.5%)低于非巨块组(88.9% ) ,P>0.05,与无块组 (91.7 % )比较P<0.05。结论巨块组CR率较其他两组低 ,复发率高 ;在COMP/ABVD交替正规化疗后 ,加用放疗可能会提高疗效 ;出现SVCS和/或SMCS患儿应按急症处理。     

Endometrial carcinoma after Hodgkin disease in childhood

A 34-year-old patient developed metastatic endometrial carcinoma after Hodgkin disease in childhood. She had ovarian failure after abdominal irradiation and chemotherapy for Hodgkin disease, and received exogenous estrogens, a treatment implicated in the development of endometrial cancer in menopausal women. Young women on replacement estrogens for ovarian failure after cancer therapy may also have increased risk of endometrial carcinoma and should be examined periodically.     

Atherosclerosis and the immune system

The immune system is involved at all stages of the atherosclerotic disease process. Innate immunity, represented by macrophages and other cells, is directly activated by microbial components and possibly also by autologous lipids and proteins. It elicits inflammatory activity, which is a key component of the atherosclerotic lesion. Adaptive immunity is initiated by recognition of disease-related antigens, which include oxidatively modified lipoproteins, heat shock proteins and microbial macromolecules. In the artery wall, adaptive immune recognition mainly leads to Thl effector responses, which are characterized by secretion of proinflammatory cytokines and by activation of macrophages and vascular cells. Therefore, both the innate and adaptive arms of the immune system lead to inflammation in the developing atherosclerotic lesion. Interestingly, several effector pathways of cellular as well as humoral immunity tend to counteract proatherogenic, proinflammatory immunity. The notion that immunity plays an important role in the development of atherosclerosis has focused attention on a number of potential novel targets for intervention based on modulation of such immune responses.     

Atherosclerosis and the immune system

The immune system is involved at all stages of the atherosclerotic disease process. Innate immunity, represented by macrophages and other cells, is directly activated by microbial components and possibly also by autologous lipids and proteins. It elicits inflammatory activity, which is a key component of the atherosclerotic lesion. Adaptive immunity is initiated by recognition of disease-related antigens, which include oxidatively modified lipoproteins, heat shock proteins and microbial macromolecules. In the artery wall, adaptive immune recognition mainly leads to Th1 effector responses, which are characterized by secretion of proinflammatory cytokines and by activation of macrophages and vascular cells. Therefore, both the innate and adaptive arms of the immune system lead to inflammation in the developing atherosclerotic lesion. Interestingly, several effector pathways of cellular as well as humoral immunity tend to counteract proatherogenic, proinflammatory immunity. The notion that immunity plays an important role in the development of atherosclerosis has focused attention on a number of potential novel targets for intervention based on modulation of such immune responses.     

Prognosis of Hodgkin’s disease in india

In a study of 388 patients with proved Hodgkin’s disease, peak age incidence was found to be between 10 to 30 years. Mixed cellular pattern was the most common pattern on histopathology which is more common in India than in Western countries where nodular sclerosis is seen in greater number of patients. Of 388 patients 149 were subjected for exploratory laparatomy and splenectomy for proper staging. Patients were treated with extended radiotherapy for stage I and II disease, and 84% and 73% of patients respectively had 5years survival period. In stage III and IV disease combined chemotherapy regime led to cure in 19–40% of cases. The newer developments have changed the total outcome of the disease which was considered as fatal illness only two decades ago.     

Prognostic factors and treatment outcome in childhood Hodgkin disease

BACKGROUND: The goals of this study included: (1) Identification of factors prognostic for event-free survival (EFS) and overall survival (OS), and (2) Definition of risk groups for risk adapted therapy in children with Hodgkin disease (HD). PROCEDURE: From 1991 to 2003, 69 children with newly diagnosed, untreated biopsy-proven stage I-IV HD were treated with chemotherapy (CT) and low-dose involved field radiotherapy (LD-IFRT). The relationship of pretreatment factors to EFS and OS was analyzed by univariate and multivariate analysis. RESULTS: The 5-year EFS and OS for all patients were 90.77% and 96.22%, respectively with a median follow-up of 73 months (3-137 months). Male to female ratio was 3:1 and 21 children (32.3%) were less than 7 years of age. Mixed cellularity was the predominant histologic subtype (38.5%). Factors associated with inferior EFS by univariate analysis were extranodal disease, hemoglobin level <11 g/dl, number of involved lymph node regions and stage. By multivariate analysis only stage IV disease was significant. CONCLUSION: Our study confirms that excellent results are achievable with combined modality therapy in childhood HD. In order to use risk-adapted therapy in children with HD, clinical prognostic factors should be validated with large, multicentered prospective clinical studies.     

Second malignancy after treatment of pediatric Hodgkin disease

Although treatment of pediatric Hodgkin disease has become highly effective over the past 40 years, a number of patients have developed concerning late effects, such as secondary malignancies. These cancers may occur years to decades after remission and arise in the breast, thyroid, gastrointestinal tract, lung, skin, urogenital tract, and brain. There is also an increased risk of leukemia and non-Hodgkin lymphoma. Etiology and risk factors for each cancer type vary but often include certain chemotherapy agents and radiation dosages. Survivorship also varies but is often poor. The authors examined retrospective analyses of these secondary malignancies and present a summary of these findings. The information may allow clinicians to better monitor childhood Hodgkin disease survivors and reduce mortality.