Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0019829 (Hodgkin's disease)
 30,247 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Pulmonary function tests (standard vital capacity, SVC; total lung capacity, TLC; forced expiratory volume in 1 second-forced vital capacity ratio, FEV1/FVC; carbon monoxide transfer factor, DLCO) were prospectively evaluated in patients (median age 25 years, 13-52 years; median follow-up 20 months, 6-51 months) with Hodgkin's disease (15 patients), non-Hodgkin's lymphoma (9 patients), and inflammatory breast cancer (3 patients) treated with sequential high-dose therapy comprising the following phases over approximately 2 months: a) cyclophosphamide (7 g/m2); b) vincristine (1.4 mg/m2), methotrexate (8 g/m2), and cisplatinum (120 mg/m2) or etoposide (2 g/m2); c) total body irradiation (TBI; 12.5 gy, 5 fractions over 48 hours), intravenous melphalan (120-180 mg/m2), and transplantation of autologous peripheral blood and/or bone marrow hematopoietic stem cells. Within 2 months after transplantation, 12 patients also received 25 Gy radiotherapy boost to mediastinum and clavicular regions. In vivo dosimetry evaluations of fractionated TBI treatments showed that mean radiation dose absorbed by lungs was 12.18 Gy (97.4% of TBI dose). Despite such a high radiation dose, we observed only transient and subclinical decrease of SVC, TLC, and DLCO. The decrease of SVC, TLC, and DLCO was more evident and prolonged in patients receiving radiotherapy boost. All parameters progressively recovered to normal values within 2 years after transplantation. In contrast, FEV1/FVC remained within normal limits in all patients, thus demonstrating the absence of obstructive ventilatory changes. In addition, no interstitial pneumonia was observed.
 ...
PMID:Prospective evaluation of pulmonary function in cancer patients treated with total body irradiation, high-dose melphalan, and autologous hematopoietic stem cell transplantation. 197 14

In clinical practice SVC syndrome is an important problem, given both the nature of the disease and its fast lethal evolution. Therapy must be instituted as soon as possible because the chances of a positive result are directly related to the staging of the primary illness. Surgery, chemotherapy and high energy therapy can be used. From the literature, although controversial, the superiority of surgical therapy is clear; particularly if up-to-date vascular reconstruction techniques are employed. From March 1980 to March 1988 8 cases of SVC syndrome were observed in which the aetiology was as follows: Hodgkin's disease (2 cases); secondary catheter thrombosis (1 cases); lung carcinoma (5 cases). The 2 cases of Hodgkin's disease were treated by chemotherapy; the secondary thrombosis by open thrombectomy. In the other 5 cases an innominate vein right appendage by-pass was used (3 PTFE, 2 pericardial grafts). The results were encouraging: complete, long-term remission was observed in the Hodgkin and thrombectomy patients. A PTFE graft thrombosis occurred in 2 cases but in the other cases the by-pass is functioning well at a mean 13 months follow-up.
 ...
PMID:[Our experience in the treatment of superior vena cava syndrome]. 234 46

Twenty-eight patients with malignant tumors of the thymus were surgically treated during the 15-year period from 1969 to 1984. Twenty-six patients had far advanced disease when treatment was began. Of fifteen patients with malignant thymoma, the tumors were successfully removed in five patients by using the technique of combined resection of SVC. Of two patients with carcinoid, one is alive and another died two years after extended operation. Of two patients with malignant lymphoma, one with non Hodgkin's disease died three years after operation, and the other with Hodgkin's disease is alive with tumor bearing. It is important to distinguish malignant lymphoma from other types of thymic tumors, because chemo-radiotherapy is superior to surgical therapy. Of nine patients with germ cell tumor, two patients with seminoma have been well for 11 and 5 years. CDDP and radiotherapy were effective on their long survival. Three patients with embryonal carcinoma responded well to combined surgical and chemo-radiotherapy. But only one of them is alive with tumor free one year after operation. Prosthetic grafts were employed in 7 cases for replacement of the innominate and superior caval vein. Angiogram taken one month after operation disclosed the overall patency rate with 92% and the longest patent period confirmed was 4 years. Extended operation by using the prosthetic graft should be performed in the advanced cases to achieve a complete removal and to aim better prognosis for this kind of disease. In conclusion, aggressive surgical removal followed by radio-chemotherapy offers best cure of the malignant thymic tumor.
 ...
PMID:[Management of malignant tumors of the thymus]. 299 45

Malignant lymphomas originating primarily in the mediastinum consist predominantly of Hodgkin's disease of the nodular sclerosis type, lymphoblastic lymphomas, and large cell non-Hodgkin's lymphomas of diffuse growth pattern (DHL). This analysis of 20 cases of primary mediastinal DHL presents the clinical and pathologic findings in nine patients with T-immunoblastic sarcoma (T-IBS), six with sclerosing variants of follicular center cell lymphoma (FCCL), and five with B-immunoblastic sarcoma (B-IBS). T-IBS patients were predominantly young adult women (mean age 31 years) presenting with relatively well confined mediastinal tumors; four of nine manifested the SVC syndrome. The immunomorphologic findings in T-IBS were similar to those of node-based peripheral T-cell lymphomas. Patients with FCCL and B-IBS were predominantly men, exhibited a broad age range, and presented with larger tumors with a high incidence of contiguous involvement of intrathoracic structures (83% in FCCL, 60% in B-IBS). Chemotherapeutic intervention attained CR in 19 of 20 patients, with 14 of 20 remaining alive in relapse-free CR a median of 26 months after completion of therapy. Durable CR was attained in eight of nine T-IBS patients, in four of six patients with FCCL, and in three of five patients with B-IBS. The morphologic features of these lymphoma subtypes are presented in detail and discussed in relation to the complex differential diagnosis of mediastinal neoplasms.
 ...
PMID:Primary large cell lymphomas of the mediastinum: an analysis of 20 cases. 387 48

A 47-year-old man was referred to our hospital because of dyspnea, cough and weight loss. On physical examination, marked dilatation of thoraco-superficial epigastric venous anastomosis was found. The chest wall collateral vessels revealed enlarged head-to-toe flow, suggesting complete obstruction of the SVC and one or more of the major caval tributaries, including the azygos system. Thoracic CT demonstrated that a huge anterior mediastinal tumor completely obstructed the superior vena cava. He was diagnosed with Hodgkin lymphoma of the nodular sclerosis type, Stage III(X)B based on the biopsy specimen from the right subcutaneous lumbodorsal mass.
 ...
PMID:Huge mediastinal mass with SVC syndrome accompanying numerous chest wall collateral vessels. 1882 23

Up to 40% of patients with advanced Hodgkin lymphoma (HL) become refractory or relapsed after current standard chemotherapy, among which primary refractory HL confers a particularly poor outcome. With intensive salvage chemotherapy and autologous stem cell transplantation, the long-term remission rate for these patients was only 30%, but more selective treatments with higher therapeutic index are needed. We report the experience of using a new anti-CD30 immunotoxin, brentuximab vedotin, in salvage treatment of a 30-year-old woman with primary refractory Hodgkin lymphoma. The patient presented with SVC syndrome due to the bulky mediastinal tumor and was confirmed to have classical Hodgkin lymphoma, nodular sclerosis type, stage IIIA. The tumor responded to induction chemotherapy transiently, but local progression was noted during subsequent cycles of treatment. Salvage radiotherapy to the mediastinal tumor, obtained no remission but was followed by rapid in-field progression and then lung metastasis. She declined stem cell transplantation and received salvage brentuximab vedotin (BV) therapy, which induced dramatic shrinkage of tumor without significant side effects. Serial followup of PET/CT imaging confirmed a rapid and continuous complete remission for 12 months. Although durability of the remission needs further observation, this case illustrates the excellent efficacy of brentuximab vedotin in primary refractory Hodgkin lymphoma.
...
PMID:Brentuximab vedotin treatment for primary refractory hodgkin lymphoma. 2419 83