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

A rare presentation of intraorbital Hodgkin's lymphoma in a patient without prior history of systemic disease who complained of episodic monocular visual loss with minimal proptosis is reviewed. The case is further distinguished by intracranial extension of the tumor. A literature review highlights such an unusual event against the background of prior experience. Despite the fact Hodgkin's disease accounts for about 30% of systemic lymphomas, it rarely occurs in the orbit, where non-Hodgkin's lymphomas account for practically all lymphomas studied. Hodgkin's disease has occurred in patients with known systemic disease in its terminal stages and usually with proptosis as the presenting symptom. We present the case of a young man with intraorbital Hodgkin's disease whose presenting symptom was recurrent transient episodes of complete monocular visual loss. His condition was further distinguished by seeding of the skull base and dura by tumor.
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PMID:Hodgkin's disease of the orbit with intracranial extension. 883 30

We have retrospectively examined the outcome of 41 patients with high grade non-Hodgkin's lymphomas (NHL) and central nervous system (CNS) involvement who were treated with and without radiation at a single institution. Group I consisted of 25 patients with CNS involvement at presentation and Group II, of 16 with CNS involvement at first relapse. All 41 had systemic disease at diagnosis and received systemic and intrathecal chemotherapy. Response to therapy did not differ whether patients received concomitant radiation or no CNS radiation. Thirteen of 16 non-irradiated (81%) and 8 of 9 irradiated Group I patients (89%) achieved complete responses. Three of 4 non-irradiated (75%) and 7 of 12 irradiated (58%) Group II patients achieved complete responses. CNS relapse patterns were similar whether or not patients were irradiated, and regardless of radiation dose. Most patients (18) failed systemically; there were few (6) isolated CNS relapses. Survival was not improved by the addition of radiation. Of the 15 patients who achieved long term survival, 13 remained disease-free throughout their clinical course: 7 of these 13 patients (all Group I) did not receive CNS radiation and 6 (4 Group I, 2 Group II) did. In this series, in which 44% of patients who presented with CNS disease and 13% of those who relapsed with CNS became long term disease-free survivors, there was no discernable benefit from radiation, but increased toxicity was observed.
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PMID:Treatment of patients with high grade non-Hodgkin's lymphomas and central nervous system involvement: is radiation an essential component of therapy? 890 76

Therapy of the lymphomas is predominantly nonoperative, relying on chemotherapy and radiation therapy. In Hodgkin's lymphoma, the role of the surgeon was once large, involving staging laparotomy. At present, chemotherapy is the predominant mode of therapy in patients of all ages, and the therapeutic distinctions centered on the histology of laparotomy specimens no longer exist. The surgeon most often will encounter the patient with lymphoma for biopsy for tissue diagnosis and for institution of long-term venous access. Non-Hodgkin's lymphoma presents with three cellular subtypes in children, and frequently is first seen in the abdomen and mediastinum as a fast-growing diffuse tumor. As a systemic disease, these lymphomas also are nearly always treated systemically by chemotherapy, yet a small number of patients with localized disease may benefit from attempts at tumor extirpation.
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PMID:The lymphomas: an update for surgeons. 911 75

Mature or peripheral T-cell lymphomas are uncommon, accounting for only 10%-15% of all non-Hodgkin's lymphomas. The classification of these neoplasms has been controversial. In contrast to B-cell lymphomas, cytologic grade has not been very useful in predicting the clinical course. This finding may result from the generally aggressive clinical course associated with T-cell lymphomas. Prior studies have suggested that stage of disease may be more important than cytologic subtype. Clinical presentation is very important in the classification of T-cell malignancies. For T-cell lymphomas, cytologic features alone are not sufficient to distinguish among disease entities. For example, adult T-cell leukemia/lymphoma (ATLL) often cannot be distinguished morphologically from HTLV-1-negative T-cell lymphomas. Most extranodal T-cell lymphomas appear to be derived from cytotoxic T cells, which express perforin, TIA-1, and granzyme B. Three broad groups of T-cell malignancies can be identified: (1) leukemic or systemic disease; (2) nodal disease; (3) extranodal disease. Anaplastic large-cell lymphoma (ALCL) is probably the single most common subtype of T-cell lymphoma. Classical ALCL should be distinguished from primary cutaneous ALCL (CD30+ lymphoproliferative disease of the skin), which is a distinct disease entity.
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PMID:Classification of T-cell and NK-cell neoplasms based on the REAL classification. 920 34

Anaplastic large cell lymphoma (ALCL) is a heterogeneous group of diseases by morphology, phenotype, genotype, and clinical presentation. Using a new monoclonal antibody (ALK1) that recognizes the native anaplastic lymphoma kinase (ALK) protein as well as the fusion product of the t(2;5)(p23;q35), nucleophosmin (NPM)/ALK, we investigated for ALK expression cases diagnosed as ALCL as well as lympho-proliferative disorders possessing overlapping features with ALCL. Thirteen cases showed cytoplasmic staining of the neoplastic cells. These cases were characterized by a fairly uniform morphology and occurred in children and young adults as a systemic disease. All other cases comprising T or null ALCL (17 cases), B ALCL (8 cases), Hodgkin's disease (HD) (15 cases), HD-like ALCL (23 cases), and lymphomatoid papulosis (9 cases), were negative for ALK expression. Translocation t(2;5)(p23;q35) was found by classical cytogenetics or interphase fluorescence in situ hybridization in 8 of the ALK1-positive cases and by reverse transcription-polymerase chain reaction in 1 other case. Two additional ALK1-positive cases with an abnormal karyotype, but without t(2;5)(p23;q35), showed by fluorescence in situ hybridization analysis a cryptic NPM/ALK gene fusion caused by an insertion of ALK near NPM in one case and a translocation of ALK to 2q35 as a result of an indiscernible inv(2)(p23q35) in the other. The latter variant translocation points to a localization of an unknown gene at 2q35 that, like NPM, might deregulate ALK and be involved in the pathogenesis of ALCL. In summary, immunohistochemistry with ALK1 antibody allows the identification of a distinct subgroup within the ALCL of T or null phenotype that is associated with 2p23 abnormalities and lacks the marked histological pleomorphism described in ALCL in general. Whereas immunostaining is the most sensitive method to identify this group, it does not help to additionally clarify the relationship among ALCL, HD, and HD-like ALCL.
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PMID:The monoclonal antibody ALK1 identifies a distinct morphological subtype of anaplastic large cell lymphoma associated with 2p23/ALK rearrangements. 925 Jan 48

We report an elderly patient who presented with a single cutaneous nodule of primary cutaneous Hodgkin's disease (PCHD). Reed-Sternberg cells, expressing CD30 and CD15, but not leukocyte common antigen (CD45RB), were identified in the neoplastic cells. Southern blot analysis failed to detect T-cell receptor or immunoglobulin gene rearrangement. A work-up for systemic disease at the time of presentation was negative, supporting the diagnosis of PCHD. Nevertheless, 1 year later, the disease course was complicated by the development of nodal HD and nonHodgkin's lymphoma (NHL). This case report supports the existence of this rare, but distinct, cutaneous tumor.
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PMID:Skin as the primary site of Hodgkin's disease: a case report of primary cutaneous Hodgkin's disease and review of its relationship with non-Hodgkin's lymphoma. 955 96

Discussion of the total costs and cost-effectiveness ratios of patients receiving high-dose chemotherapy (HDC) and peripheral blood stem cell support (PBSCS) is controversial. In Germany, no reliable data are available, whereas in other countries this issue has been extensively studied. We performed a pharmacoeconomic evaluation on all patients (n = 37) treated with HDC and PBSCS at our institution between July 1994 and June 1997. Patients suffered from high-risk or poor-prognosis breast cancer (n = 24), Hodgkin's disease (n = 3), high-grade non-Hodgkin's lymphoma (n = 4), multiple myeloma (n = 2), small-cell cervical cancer (n = 1), malignant hystiocytosis (n = 1) and testicular cancer (n = 2). For pharmacoeconomic evaluation, the period from initiation of induction chemotherapy (IC) until reconstitution after the last course of HDC and PBSCS was considered. A total of 18 patients received IC/HDC/PBSCS for locally advanced or systemic disease, and 19 patients received adjuvant or consolidation IC/HDC/PBSCS. Treatment protocols were heterogeneous. Patients were treated with two to five courses (median two) respectively of IC and sequential mono-HDC (n = 26), tandem-HDC (n = 10) or triple-HDC (n = 1). All patients received granulocyte/macrophage-colony-stimulating factor (G-CSF) for stem cell mobilisation and for amelioration of neutropenia after HDC. The relative costs (based on supplier prices) for the total amount of drugs prescribed during the in-patient period was 29.8% for G-CSF, 35.8% for blood products 18.5% for chemotherapy, 2.4% for antiemetics, 5.9% for antimicrobial drugs and 7.6% for other drugs. Contrary to expectations, antimicrobial drugs had only a minor pharmacoeconomic impact during IC/HDC/PBSCS in patients with high-risk or poor-prognosis malignancies, indicating that prolonged septic complications were uncommon in our institution. We conclude that pharmacoeconomic evaluations in IC/ HDC/PBSCS might be integrated into the effort to ensure quality control and monitoring.
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PMID:Pharmacoeconomic evaluation of high-dose chemotherapy and peripheral blood stem cell support in high-risk or poor-prognosis malignancies. 964 62

Mature or peripheral T-cell lymphomas are uncommon, accounting for only 10% to 15% of all non-Hodgkin lymphomas, and their classification has been controversial. In contrast to B-cell lymphomas, cytologic features have not been useful in defining disease entities, and cytologic grade has not been useful in predicting the clinical course. Similarly, many entities of T-cell or NK-cell derivation do not have a specific immunophenotype. Clinical features are of major importance, sometimes more important than the precise cell of orgin, in defining T-cell and NK-cell neoplasms. Most extranodal T-cell/NK-cell lymphomas have a cytotoxic phenotpye. The expression of cytotoxic molecules may predispose to apoptosis by tumor cells and normal bystander cells. Three major categories of extranodal T/NK cell tumors are nasal, intestinal, and subcutaneous panniculitis-like. Hepatosplenic gamma delta T-cell lymphoma is a more systemic disease derived from functionally immature cytotoxic cells. Many extranodal T-cell and NK-cell neoplasms are associated with Epstein-Barr virus (EBV); the association seems site dependent and shows some geographic variation. Tumors resembling any of the 3 prototypes may occur in a variety of extranodal sites. Extranodal T/NK cell lymphomas occur with increased frequency in the setting of immune suppression, especially after organ transplantation.
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PMID:Extranodal peripheral T-cell and NK-cell neoplasms. 989 69

A case of primary intracerebral Hodgkin's disease (HD) without dural attachment in a 54-year-old immunocompetent patient is described. The infiltrate was located superficially in the occipital lobe and corresponded to the histologic type of nodular sclerosis. A typical immunohistochemical profile (membrane and cytoplasmic staining with dotlike Golgi enhancement of CD30, moderate cytoplasmic staining of CD15 in the Golgi area, membrane staining of CD20 of <10% of blastic cells, CD45RB negative) and in addition Epstein-Barr virus (EBV) latent membrane protein was detectable in Reed-Sternberg cells. Staging revealed no other organ sites of involvement. After combined surgery, postoperative radiotherapy, and chemotherapy, there are no signs of recurrence or systemic disease on follow-up for >1 year. To the authors' best knowledge, an association of EBV with primary central nervous system HD has not been demonstrated before.
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PMID:Primary intracerebral Hodgkin's disease: report of a case with Epstein-Barr virus association and review of the literature. 1019 79

The mediastinum is a frequent site of involvement for malignant lymphoma. The most common types encountered in this location include Hodgkin's and non-Hodgkin's lymphoma and lymphoblastic lymphoma. With the exception of lymphoblastic lymphoma in children and adolescents, most cases of mediastinal involvement by lymphoma represent part of systemic disease and do not generally pose difficulties for diagnosis. However, a relatively small group of tumors have become increasingly recognized in recent years that are primarily localized in the anterior mediastinum and show features of nonlymphoblastic/non-Hodgkin's large-cell lymphoma. Because such tumors are capable of adopting unusual morphological appearances, they can often pose difficulties for diagnosis and be frequently mistaken for other conditions. This review discusses this group of neoplasms collectively known as diffuse large-cell lymphoma of the mediastinum. The clinicopathologic, immunohistochemical, and molecular genetic aspects of these tumors appear to indicate that a significant proportion of them may correspond to a distinctive type of lymphoproliferative process most likely arising from a native B-cell population of the thymus, thus representing, in essence, a primary extranodal large-B-cell lymphoma of the thymus.
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PMID:Primary large-cell lymphomas of the mediastinum. 1035 54


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