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)

Primary renal lymphoma is a controversial and rare disease. There is no agreement whether or not it is an initial manifestation of a rapidly systemic disease. Most reported cases are questionable because of incomplete staging and lack of autopsy studies. Herein, we present a 71-year-old woman, initially diagnosed with primary non-Hodgkin lymphoma of the kidney, but was lost 4 months after radical nephrectomy due to systemic disease despite aggressive chemotherapy, and suggest that, the kidney of the patient was the initial presenting site of a rapidly progressive systemic disease.
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PMID:Lymphoma of the kidney: primary or initial manifestation of rapidly progressive systemic disease? 1711 Oct 87

Peripheral T-cell lymphomas (PTLs) are uncommon, accounting for fewer than 10% of all non-Hodgkin lymphomas. Success in therapy of the PTLs has lagged behind that of aggressive B-cell lymphomas, and most PTLs have a poor prognosis. The molecular pathogenesis of most PTLs is also poorly understood. In the WHO classification, clinical features, in conjunction with morphological and immunophenotypic criteria, are relied on to define most disease entities. Functionally, T-cell lymphomas are related to the two major arms of the immune system, the innate and adaptive immune systems. NK cells and T cells of the innate immune system recognize antigen in the absence of MHC antigens and are involved in mucosal immunity. The lymphomas derived from these cells often involve cutaneous and mucosal sites. The expression of cytotoxic molecules in these lymphomas may predispose to apoptosis by tumor cells and normal bystander cells. Hepatosplenic T-cell lymphoma is a systemic disease derived from functionally immature innate effector cells, most often of gammadelta T-cell origin. In contrast, most nodal T-cell lymphomas belong to the adaptive immune system. Angioimmunoblastic T-cell lymphoma (AILT) is mostly likely derived from follicular helper T-cells (T(FH)), a finding that explains many of its pathological and clinical features. Studies of these neoplasms may assist in further unraveling the functional diversity of their normal counterparts.
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PMID:Pathobiology of peripheral T-cell lymphomas. 1712 78

Lymphomas rarely present as a localized mass within the dura. We report a case of a 72-year-old woman, normally fit and well, who presented with a sudden episode of grand mal seizure. Imaging studies showed an extra-axial mass arising from the dura, with radiologic features characteristic of a meningioma. The mass was surgically excised, and the histologic features were of a grade 1 follicular lymphoma. Analysis using fluorescent in situ hybridization (FISH) demonstrated t(14:18) translocation. A bone marrow aspirate showed focal infiltration of lumbar vertebra by low-grade follicular lymphoma. Intracranial meningeal involvement by non-Hodgkin lymphoma usually occurs in the form of diffuse leptomeningeal spread. It is seen in high-grade lymphomas, often in association with widespread systemic disease. The majority of cases of dural lymphomas reported in the literature have been primary mucosa-associated lymphoid tissue (MALT)-type lymphomas. Most of these presented as localized dural masses mimicking meningiomas. Low-grade follicular lymphoma, either primary or secondarily involving the dura, appears to be an exceedingly rare occurrence.
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PMID:Low-grade follicular lymphoma in the dura: rare mimic of meningioma. 1720 94

The most common low-grade non-Hodgkin lymphomas are of B-cell origin. This review will focus on follicular lymphomas and extranodal marginal zone lymphomas, also known as mucosa-associated lymphoid tissue (MALT) lymphomas. These are radiation-sensitive lymphomas. Moderate doses (30-35 Gy) for these stage I and II low-grade lymphomas result in long-term local control and possible cure. Involved-field radiation therapy is the standard approach and produces minimal morbidity. However, a significant proportion of patients relapse with systemic disease outside of radiation fields. For follicular lymphoma, this occurs in approximately 50% of patients after 15 years and for nongastric MALT lymphoma 30% to 40% after 10 years. Patients with relapsed disease are not curable with chemotherapy, but the disease often remains indolent and prolonged survival is observed. For gastric MALT lymphomas associated with Helicobacter pylori but which did not respond to antibiotic therapy, radiation treatment is indicated and almost always curative. For localized MALT lymphomas not related to microorganisms, radiation therapy is the initial standard therapy regardless of anatomic location. Patients with stage III and IV low-grade lymphoma and local symptoms are often successfully palliated with a low dose regimen of 2 x 2 Gy (total dose 4 Gy).
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PMID:Low-grade non-hodgkin lymphomas. 1759 67

Anaplastic large cell lymphoma (ALCL) is 1 of 17 mature T cell neoplasms described by the World Health Organization. Primary central nervous system (PCNS) ALCL represents a distinct rare form of this family of non-Hodgkin lymphoma and discussions of prognosis and management are limited to case reports and case series. Therapies for this disease largely parallel that of other PCNS lymphomas. We report the case of a 38-year-old male soldier who presented with a parieto-occipital mass lesion and neurological sequelae without evidence of systemic disease. Pathologic evaluation of tissue from brain biopsy confirmed ALCL. We elected treatment with an intensive combination of systemic and intrathecal chemotherapy with radiotherapy. Our patient is in complete remission 15 months following therapy. Tailored therapies for PCNS ALCL are unavailable and this regimen may be an option for patients who can tolerate intensive treatments.
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PMID:Central nervous system anaplastic large cell lymphoma in an adult: successful treatment with a combination of radiation and chemotherapy. 1761 57

Cutaneous Hodgkin's disease is a rare condition that usually occurs late in the course of Hodgkin's lymphoma. This rare condition is thought to have decreased in incidence in recent decades, likely owing to improved treatment of patients with Hodgkin's disease, who are receiving improved chemotherapy and radiation therapy, and the advent of peripheral blood stem cell transplantation. We present the case of a man who developed specific cutaneous Hodgkin's lymphoma 6 months after nonmyeloablative allogenic stem cell transplantation for his recurrent systemic disease. The patient's manifestation of relapse was cutaneous dissemination of the tumor, manifested by erythematous papules and ulcerated nodules. At the time of the cutaneous relapse he had minimal systemic disease. This case illustrates an example of this complication of Hodgkin's disease and stresses the importance of a timely diagnosis to direct appropriate therapy. A review of the literature demonstrates that the patient's lesion morphology and distribution are typical of specific manifestations of cutaneous Hodgkin's disease.
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PMID:Cutaneous Hodgkin's disease. 1822 26

Non-Hodgkin lymphomas of the breast are rare, encompassing approximately 0.04-0.5% of all malignant breast tumors, and the vast majority are B-cell lymphomas. In contrast, lymphomas of T-cell phenotype have been rarely reported and some of these have been in close proximity to a breast implant. In our consultation practice, we have identified four patients with primary T-cell anaplastic large-cell lymphoma presenting adjacent to silicone or saline breast implants. All patients presented with seroma and neoplastic cells were identified in suspension in the serous fluid without solid tissue invasion. Three patients had no evidence of systemic disease (stage 1E), and one patient was not staged. The mean age of the patients was 46 years (range, 34-59 years). In all patients, the neoplastic cells had a T-cell phenotype, expressed CD30, cytotoxic granule-associated proteins, EMA and clusterin, and were anaplastic lymphoma kinase-1-negative. Clonal T-cell receptor gamma-chain gene rearrangements were identified in three patients. All patients underwent capsulectomy with removal of the implant. One patient subsequently received chemotherapy and radiation therapy, and another was treated with radiation alone. The third patient received no further therapy and the fourth patient has been recently diagnosed. After a mean time of 13 months (range, 9-20 months), all three patients with follow-up were alive and well without any recurrence or systemic disease. Although the follow-up time was relatively short, our series and other reported cases suggest that primary anaplastic large-cell lymphoma adjacent to breast implants is an indolent T-cell lymphoproliferative disorder.
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PMID:Seroma-associated primary anaplastic large-cell lymphoma adjacent to breast implants: an indolent T-cell lymphoproliferative disorder. 1822 53

The gastrointestinal (GI) tract contains the largest collection of lymphocytes anywhere in the body. GI lymphoma may arise at any site in the GI tract but typically involves the stomach and small bowel in cases of systemic disease. Most cases are non-Hodgkin B-cell type. Enteropathy-associated T cell lymphoma can complicate celiac disease. Less commonly, lymphoma may originate in the GI tract without systemic involvement. This sometimes occurs in response to chronic infections. This article discusses the role of imaging in detecting and staging GI tract lymphomas, using fluoroscopy and cross-sectional imaging, primarily CT.
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PMID:Imaging of gastrointestinal lymphoma. 1861 82

Primary central nervous system lymphoma (PCNSL) is a rare variant of extranodal non-Hodgkin lymphoma that is restricted in distribution to the brain, leptomeninges, spinal cord, and intraocular compartments. Although PCNSL shares overlapping features with systemic lymphoma, recent studies also reveal a unique pattern of gene and protein expression in PCNSL. These findings have yielded new insights into the pathophysiology of the disease, as well as the identification of novel prognostic biomarkers. Immune system compromise, such as is seen in acquired immune deficiency syndrome (AIDS), is the best established known risk factor for PCNSL. Like other lesions of the brain, meninges, and eye, the presenting symptoms associated with PCNSL typically include focal neurological deficits related to the site of disease or more global consequences of increased intracranial pressure. Diagnosis of PCNSL typically includes gadolinium-enhanced MRI and pathologic tissue analysis, as well as additional studies aimed at excluding concurrent systemic disease. PCNSL typically has a worse overall prognosis than systemic lymphoma. High-dose chemotherapy, particularly with methotrexate-based regimens, is the backbone of therapy for most patients, and chemotherapy is associated with much lower rates of treatment-related morbidity and mortality than whole-brain irradiation. Autologous stem cell transplantation is an emerging treatment modality, particularly in younger patients with relapsed disease, but high rates of treatment-related mortality are observed in older patients. Immunotherapy, including treatment with intrathecal rituximab, is another area of active research that may have promise in refractory or relapsed disease. Treatment options for intraocular lymphoma parallel those for PCNSL elsewhere in the brain: systemic chemotherapy, radiation, and local delivery of cytotoxic and immunologically active agents such as anti-CD20 antibody.
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PMID:Biology and treatment of primary central nervous system lymphoma. 1956 Jul 47

Primary central nervous system lymphoma (PCNSL) is an extranodal non-Hodgkin lymphoma involving brain, intraocular structures and spinal cord, without evidence of systemic disease. The majority of PCNSLs are diffuse large B-cell type. We encountered a rare case of primary dural marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT) with extension into the brain in a 59-year-old man. A magnetic resonance imaging scan showed a 22-mm tumor located in the left posterior temporal lobe extending from the dura. Histopathology revealed a lymphoplasmacytic infiltration of the dura and the brain parenchyma in a perivascular pattern. Immunohistochemical and in situ hybridization studies showed a B-cell phenotype with kappa light chain restriction. Fluorescent in situ hybridization study showed a t(14;18)(q32;q21) with immunoglobulin heavy-chain/MALT1 fusion. The molecular study for immunoglobulin heavy-chain gene rearrangement by polymerase chain reaction showed a clonal gene rearrangement.
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PMID:Extranodal marginal zone lymphoma of the dura mater with IgH/MALT1 translocation and review of literature. 1966 12


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