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Cardiophrenic adenopathy in Hodgkin disease.

Hodgkin disease can involve diaphragmatic lymph nodes adjacent to the heart. When nodes at the cardiac apex are involved, the roentgenographic appearance simulates that of the normal pericardial fat pad. Though, to our knowledge, no cases have been reported in which this was the only intrathoracic manifestation of Hodgkin disease at the time the patient was first seen and the condition was diagnosed, it may be the only manifestation of relapse, as was true in the case presented.

Adolescent

Bronchial involvement by Hodgkin disease.

Two cases of Hodgkin disease, lymphocyte depletion type, with bronchial involvement are described. One patient revealed involvement of the right upper lobar bronchus, the other patient of the left main bronchus. The correct diagnosis was made by fiberbronchoscope examination with cytologic brushing and biopsy. That bronchial involvement by Hodgkin disease is very rare is confirmed by the literature, where only 20 cases have been so far reported.

Bronchial Neoplasms

Idiopathic thrombocytopenic purpura occurring in Hodgkin disease after splenectomy: report of two cases and review of the literature.

The occurrence of idiopathic thrombocytopenic purpura (ITP) in Hodgkin disease is uncommon. Even more unusual is the development of ITP in Hodgkin disease following splenectomy. This report describes two patients with Hodgkin disease who developed severe ITP with negative platelet antibody studies very soon after splenectomy (20 days in one and three months in the other). A review of the literature of 21 other patients with well-documented ITP and Hodgkin disease indicated that ITP occurring in Hodgkin disease may be more severe and refractory to therapy than ITP unassociated with Hodgkin. Nodular sclerosis or mixed cellularity histologic types of Hodgkin disease were present in all but one of the patients with ITP and Hodgkin disease, and males constituted 65% of cases. There appeared to be no correlation between the onset of ITP and activity of Hodgkin disease. Of five splenectomized patients with Hodgkin disease who developed ITP and were treated with immunosuppressive drugs for thrombocytopenia, three had an excellent response and two had a good response, suggesting that the combination of corticosteroids and immunosuppressive drugs may be indicated at the outset in patients with Hodgkin disease who develop ITP following splenectomy.

Autoantibodies

Immunodeficiency in Hodgkin disease.

The immunodeficiency in Hodgkin disease is characterized by a defect in cell-mediated immunity. Recent studies have reported that delayed hypersensitivity responses (DHR) are normal in untreated patients with early disease. The present study evaluated the DHR of 218 untreated patients using 2,4-dinitrochlorobenzene and a battery of intradermal antigens. The results confirm an immune deficiency in early stages which is not an all-or-none phenomenon and may be unmasked by using lower concentrations of antigens.

Adolescent

Immune thrombocytopenic purpura in Hodgkin disease.

Immune thrombocytopenic purpura is rarely seen in Hodgkin disease and the presence of platelet-associated antibody has not been previously reported in these patients. A patient with Hodgkin disease is described who developed a destructive thrombocytopenia demonstrated by shortened platelet survival. In conjunction with his thrombocytopenia, he had marked elevation of platelet-associated immunoglobulin G levels (nanograms IgG per 10(9) platelets: 15,187 prior to splenectomy and 71,130 and 81,900 after surgery). Mean values (+/-SD) of control subjects averaged 1,975 + 381 and four patients with Hodgkin disease and normal platelet counts had levels ranging from 1,581 to 4,011. We suggest that this patient had immune-mediated thrombocytopenia; whether the increase in platelet-associated immunoglobulin G was due to antiplatelet antibody or to adsorbed or phagocytosed immune complexes cannot be demonstrated by these studies. The platelet-associated immunoglobulin G test may be useful in evaluating these patients.

Antibodies

Use and efficacy of pneumococcal vaccine in patients with Hodgkin disease.

Fulminant bacterial sepsis has been described in patients with Hodgkin disease who have undergone splenectomy for staging purposes. The organisms commonly associated with sepsis in this setting include Streptococcus pneumoniae and Haemophilus influenzae. Polyvalent pneumococcal vaccine (Merck) has recently been licensed and has been suggested for use in patients with Hodgkin disease who are at risk for postsplenectomy sepsis. We administered 14-valent pneumococcal vaccine to 24 patients with Hodgkin disease and 24 normal controls, and measured antibody response to 13 antigens at time of immunization and at 3 wk and 3 mo following immunization. Our results indicate that patients who have been previously treated for Hodgkin disease, with chemotherapy, radiotherapy, or both, have severe impairment of antibody response. Untreated patients, however, respond in a manner similar to normal controls.

Antibodies, Bacterial

Microorganism-like structures in Hodgkin disease. Electron microscopical demonstration.

Ultrastructural examination of lymph nodes from patients with untreated Hodgkin disease showed unique spherical structures that were found extracellularly and within vacuoles of reticulum cells, macrophages, mononuclear cells, and occasional tumor cells. These spheroids varied in size and internal composition possibly in relation to developmental stages, and resembled mycoplasma morphologically and in cellular distribution. The spheroids were found predominantly in the neoplastic areas in lymph nodes of four out of nine patients with Hodgkin disease of the mixed cell variety, but were not seen in other types of Hodgkin disease or reticulum cell sarcoma. The structures were much more numerous in the lymph nodes from young children with Hodgkin disease. A lymph node of one child, disclosed numerous extracellular structures resembling viral type C particles in addition to mycoplasma-like spheroids. The results indicate that subclinical infections may occur in Hodgkin disease and contribute to the histopathologic manifestations of the disease.

Adolescent

Hodgkin disease and red cell aplasia.

A teenage boy with mixed-cellularity Hodgkin disease presented with severe anemia secondary to pure red cell aplasia of marrow without evidence of lymphomatous infiltration or hemolysis. In vitro studies of the patient's serum demonstrated an inhibitor of erythropoietin activity which appeared to be an IgG but which did not directly bind erythropoietin. The patient's anemia resolved and the inhibitor disappeared following chemotherapy for Hodgkin disease. Presumably, the inhibitor was directed at a very early stage of red blood cell production. This phenomenon may be related to other autoimmune manifestations occasionally seen in patients with lymphomas. The case is presented to bring attention to the unusual occurrence of red cell aplasia in Hodgkin disease. Several hypotheses concerning significance and etiology of the anemia are detailed.

Adolescent

Depressed in vitro lymphocyte responses to PHA in patients with Hodgkin disease in continuous long remissions.

Twenty consecutive patients with Hodgkin disease in continuous complete remission and off treatment for at least 5 yr (range 5-25 yr, median 9 yr) were studied with a battery of immunologic parameters. Skin test reactivity to four common antigens, sensitization to 2,4-dinitrochlorobenzene, absolute lymphocyte count, relative percentage of T cells (as measured by spontaneous rosette formation with sheep erythrocytes) and B cells (as measured by immunofluorescence with polyvalent antiserum), and absolute number of T and B cells were normal when compared with controls. However, the mean value of lymphocyte response in vitro to the mitogen phytohemagglutinin for the study population was significantly decreased (p less than 0.001) when compared with the controls. This abnormality in response to mitogen could not be correlated with age, sex, stage, symptoms, histologic subclassification, or previous treatment. The data suggest the existence of a persisting cell-mediated immune defect in the circulating lymphocytes in patients with long-standing Hodgkin disease that might otherwise be considered "cured."

Antigens

An evaluation of intraabdominal involvement in Hodgkin disease.

An analysis of the staging procedure in 80 patients with histologically confirmed Hodgkin disease was performed at Tata Memorial Hospital, Bombay. There was a marked male preponderance. The mixed cellularity type of Hodgkin disease is the commonest histological variety (47.5%) and has the highest incidence of subsequently proven generalized disease (76%). The lymphocytic predominant variety was the next most common (27.5%) and was present as unsuspected disease in 45% of cases. Systemic symptoms were associated with advanced disease in 57% of clinical IB and 73% of clinical IIB cases. Splenic involvement was histologically confirmed in 41% of cases with stage I disease and 52% of cases with stage II disease and was associated with liver involvement in two cases. A lymphogram was performed, in addition, prior to the laparotomy in 40 cases and the lymphogram-laparotomy concordance was approximately 72% in our series. This stresses the importance of a staging laparotomy.

Abdominal Neoplasms

Monocyte-mediated inhibition of lymphocyte blastogenesis in Hodgkin disease.

Mononuclear leukocytes isloated from the blood of previously treated patients with advanced active Hodgkin disease contained high concentrations of monocytes and showed poor lymphocyte blastogenesis to mitogens. In five of eight patients with disseminated disease, blastogenesis became normal or improved markedly when the leukocyte suspensions were depleted of monocytes before culture. Addition of autologous macrophages to the monocyte-depleted lymphocytes resulted in a reappearance of the inhibition of blastogenesis. Monocyte inhibition was associated with the presence of active disease, lymphocytopenia, and low lymphocyte/monocyte ratios in the peripheral blood. The role of previous treatment is uncertain, since inhibition tended to disappear when the patients were retreated. Inhibitory monocyte-lymphocyte interactions may be one of the causes of impaired cell-mediated immunity in Hodgkin disease.

Hodgkin Disease

Achalasia of the esophagus associated with Hodgkin disease.

Esophageal achalasia has not been reported in the literature as a complication of Hodgkin disease. Involvement of the esophagus is rare but when present, consists mostly of bleeding, ulceration or diverticuli. A case is reported in a fifty-one year old female with achalasia of the distal third of the esophagus associated with Hodgkin disease, Stage II B.

Esophageal Achalasia

A new combination chemotherapy for resistant Hodgkin disease.

A new four-drug combination chemotherapeutic regimen (BVDS) was used in the treatment of advanced Hodgkin disease resistant to MOPP (mechlorethamine hydrochloride, vincristine sulfate, procarbazine hydrochloride, and prednisone). The BVDS regimen, consisting of 12 cycles of bleomycin sulfate, vinblastine sulfate, doxorubicin hydrochloride (Adriamycin), and streptozocin (streptozotocin), was administered to ten patients. Responses were seen in five (50%) of these patients. Complete remissions occurred in three (30%). These results suggest that BVDS is an effective alternative regimen to MOPP, and may be of benefit not only to patients resistant to MOPP, but also to newly-diagnosed patients with advanced hodgkin disease when combined sequentially with MOPP.

Adolescent

Peritoneoscopy in Hodgkin disease. Confirmation of results by laparotomy.

Peritoneoscopy was used to evaluate the liver in 35 previously untreated patients with Hodgkin disease. Four were found to have hepatic involvement. Of the 31 patients with normal peritoneoscopies, only one had liver disease demonstrated subsequently at confirmatory exploratory laparotomy. The diagnostic accuracy of peritoneoscopy was 93% for patients at high risk for hepatic disease and 97% for all patients studied. Morbidity from the procedure was minimal. There was no mortality. Peritoneoscopy is a highly accurate staging procedure that should be considered as an antecedent or as an alternative to laparotomy in patients with Hodgkin disease. Its accuracy and minimal morbidity should be considered in the critical selection process of determining which individuals should undergo laparotomy.

Biopsy, Needle

Multiple carcinomas following therapy for Hodgkin disease.

A 52-year-old man developed synchronous squamous-cell carcinoma of the larynx and middle one-third of the esophagus following treatment of Hodgkin disease. This patient fell into the subgroup of patients treated by intensive radiation therapy followed by intensive chemotherapy. The link between the therapy and subsequent carcinoma is suggestive. Long-term follow-up care of patients with Hodgkin disease demands continuing evaluation of this possible complication.

Antineoplastic Agents