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)

Immunohistochemical determination of p63 protein is frequently used in the pathologic diagnosis of nonhematological solid tumors. In malignant hematological disease, p63 expression has been reported in 22% of follicular lymphoma, about 35% of diffuse large B-cell lymphoma, 23% of chronic lymphocytic leukemia, and in some cases of blast crisis of chronic myelogenous leukemia. Anaplastic large cell lymphoma is a rare disease that accounts for less than 5% of all cases of non-Hodgkin's lymphoma. There is little information concerning p63 expression in this specific type of lymphoma. In some cases, the morphological and phenotypic features between anaplastic large cell lymphoma and classical Hodgkin's lymphoma are similar, making this differential diagnosis challenging. We studied p63 expression using a tissue microarray approach in 154 cases of anaplastic large cell lymphoma, including 38% anaplastic large cell kinase positive and 62% anaplastic large cell kinase negative, and 58 Hodgkin's lymphoma cases. Sixty-eight cases of anaplastic large cell lymphoma (44%) showed p63 nuclear positivity (41% of anaplastic large cell kinase positive and 47% of anaplastic large cell kinase negative). Of 130 cases of systemic-anaplastic large cell lymphoma, 42% showed p63 positivity. The neoplastic cells expressed p63 in 38% of the cases of CD45-negative/anaplastic large cell kinase-negative null cell-type anaplastic large cell lymphoma, a subgroup that offers the most difficulties in the differential diagnosis with classical Hodgkin's lymphoma. In contrast, none of the cases of classical Hodgkin's lymphoma demonstrated any p63 expression. These results demonstrate that p63 protein expression is frequently expressed in a subset of anaplastic large cell lymphoma cases and may be used as a potential tool in the differential diagnosis between anaplastic large cell lymphoma and classical Hodgkin's lymphoma.
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PMID:Expression of p63 in anaplastic large cell lymphoma but not in classical Hodgkin's lymphoma. 1862 Jul 33

T-cell lymphoblastic lymphoma is a rare form of non-Hodgkin lymphoma, which shows preponderance for young men. Most common symptoms are painless swelling of lymph nodes, accompanied by B symptoms and large mediastinal masses. Most often, an involvement of the nervous system is due to paraneoplastic symptoms or side effects of treatment. In a literature research, we could not find a case with affection of a cervical nerve root as the first symptom for T-cell lymphoblastic lymphoma. A 39-year-old man presented with right-sided C8 radiculopathy, including pareses and paresthesia. Since the magnetic resonance image disclosed a right-sided mass lesion in the region of the neuroforamen C8, compressing the corresponding nerve root, a schwannoma was suspected. The tumor was removed using a dorsal approach. Neuropathological examination revealed the diagnosis of T-cell lymphoblastic lymphoma. The patient underwent diagnostic staging and received further treatment. He experienced a very grim course and succumbed to his disease 12 months after surgery. T-cell lymphoblastic lymphoma is a rare disease, and tropism of lymphoma cells to neural structures is seldom encountered. However, the presence of radiculopathy, together with signs, referring to B symptoms, should prompt the physician to consider this coincidence in the differential diagnosis of schwannoma.
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PMID:T-cell lymphoma mimicking schwannoma of a cervical nerve root. 1881 May 14

Extranodal adult T-cell leukemia/lymphoma (ATLL) of the head and neck is a rare disease. We studied the clinicopathological features of nine patients with ATLL involving extranodal head and neck sites and conducted a literature review. Six patients presented with extranodal mass of the head and neck, whereas three had disseminated diseases. Tumors involved the parotid gland, sinonasal tract, masseter muscle, mandible and skull. Histopathology included diffuse pleomorphic-type (with angiocentric features), Hodgkin-like and anaplastic large cell-type. Five patients with localised disease showed prolonged survival regardless of unfavourable histology and/or aberrant provirus status, including integration of multiple copies or defective provirus. Patients with localised disease documented in the literature, including our study series, had a reduced frequency of elevated lactate dehydrogenase, no hypercalcemia and longer survival. ATLL should be included in the differential diagnosis of extranodal head and neck lymphoma. Localised extranodal ATLL of the head and neck may exhibit indolent clinical behaviours.
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PMID:Extranodal adult T-cell leukemia/lymphoma of the head and neck: a clinicopathological study of nine cases and a review of the literature. 1923 10

Adrenal "incidentalomas" are commonly found on body imaging, and treatment of these lesions 4 cm-6 cm in size is controversial. Most of these lesions are benign adrenal cortical adenomas. Lymphoma is a rare disease manifestation in the adrenal gland, and the overwhelming majority are metastatic lesions. Hodgkin lymphoma has never been reported as a primary adrenal lesion. We present a very unique case report of a 5 cm adrenal "incidentaloma" that represents the first reported case of primary Hodgkin lymphoma in the adrenal gland.
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PMID:Primary Hodgkin lymphoma of the adrenal gland: a unique case presentation. 1949 84

Intravascular large B-cell lymphoma (IVLBCL) is a rare disease entity of non-Hodgkin lymphoma according to the current WHO classification. This rare form of B-cell lymphoma is characterised by selective growth of tumour cells in the lumina of small vessels of various organs. Strange characteristics of IVLBCL, including the absence of marked lymphoadenopathy and the usually aggressive clinical behaviour, result in the delay of timely and accurate diagnosis and fatal complications. Thus, the prognosis of IVLBCL is extremely poor. The success achieved with the anti-CD20 chimeric monoclonal antibody, rituximab, represents an important milestone in the clinical practice of B-cell lymphoma. An advantage of adding rituximab to conventional chemotherapies has been shown, in the process of increasing our understanding of the clinical and pathological manifestations for IVLBCL. This Review describes the cutting edge of research on IVLBCL, and discusses the unsolved issues from biological and clinical perspectives to provide a better understanding of this rare lymphoma.
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PMID:Presentation and management of intravascular large B-cell lymphoma. 1971 91

Carcinomas of the head and neck occur in both children and adults, but notable differences exist in their relative frequency, pathologic subtypes, etiologies, presenting symptoms, and late effects. In contrast, treatment strategies are similar depending on the disease type and distribution at the time of diagnosis. Thus, in adult patients, squamous cell carcinomas or one of its variants are the most common malignancies in the head and neck. However, in children, cancers of the head/neck are most commonly rhabdomyosarcomas, lymphomas, including Hodgkin's lymphoma, lymphoblastic lymphomas, and Burkitt's lymphoma or neuroblastoma. Epithelial cancers are unusual in the pediatric population, with the exception of nasopharyngeal carcinoma. Although nasopharyngeal carcinoma is a rare disease in children, representing less than 1% of childhood cancers, it does constitute 20%-50% of pediatric malignancies of the nasopharynx. This is one of the few malignant tumors in children that arise from the epithelium. Despite the differences between the diseases in children from that in adults, the management strategy has been based largely on the experience in adults. This review will describe the epidemiology, etiology, management, and late effects in children and adults, and offer explanations for both the similarities and differences.
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PMID:Head and neck carcinomas across the age spectrum: epidemiology, therapy, and late effects. 1995 31

Non-Hodgkins lymphoma of the ovary is a rare disease and there is only one previously documented case arising in a patient with human immunodeficiency virus (HIV). In this report, the authors discuss the management of a case of non-Hodgkins Lymphoma of the ovary occurring in a patient with HIV and demonstrate that treatment regimens may be successfully implemented in this immunocompromised population without an increase in adverse effects.
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PMID:Primary non-Hodgkins lymphoma of the ovary in the background of human immunodeficiency virus (HIV): a bold and curative approach to treatment. 2034 92

Patients with 47, XXY karyotype (Klinefelter syndrome) appear to have increased risk of developing cancer, especially male breast cancer, germ cell tumours and non Hodgkin lymphomas, but rarely acute myeloid leukaemia. We report a patient with acute basophilic leukaemia with 47, XXY karyotype in both the tumour and constitutional cells. Acute basophilic leukaemia is very rare disease comprising less than 1% of all acute myeloid leukaemias. Morphological characteristic of leukaemic blast cells is moderately basophilic cytoplasm containing a variable number of coarse basophilic granules. The most characteristic cytochemical reaction is metachromatic positivity with toluidine blue. Blast are myeloperoxidase negative. Also leukemic blasts express myeloid and monocyte markers. There is no consistent chromosomal abnormality identified in this leukaemia. This is the first reported case of acute basophilic leukaemia in patient with Klinefelter syndrome. In this article the medical history of the patient is given and the possible connection between Klinefelter syndrome and acute myeloid leukaemia is discussed.
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PMID:Klinefelter syndrome and acute basophilic leukaemia--case report. 2069 48

The case-cohort design is an economical solution to studying the association between an exposure and a rare disease. When the disease of interest has a delayed occurrence, then other types of event may preclude observation of the disease of interest giving rise to a competing risk situation. In this paper, we introduce a modification of the pseudolikelihood proposed by Prentice (Biometrika 1986; 73:1-11) for the analysis of case-cohort design, to accommodate the existence of competing risks. The modification is based on the Fine and Gray (J. Amer. Statist. Assoc. 1999; 94:496-509) approach to enable the modeling of the hazard of subdistribution. We show through simulations that the estimate that maximizes this modified pseudolikelihood is almost unbiased. The predictive probabilities based on the model are close to the theoretical probabilities. The variance for the estimates can be calculated using the jackknife approach. An application of this method on the analysis of late cardiac morbidity among Hodgkin Lymphoma survivors is presented.
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PMID:The analysis of case cohort design in the presence of competing risks with application to estimate the risk of delayed cardiac toxicity among Hodgkin lymphoma survivors. 2082 27

Autoimmune lymphoproliferative syndrome (ALPS) is a rare disease caused by defective lymphocyte apoptosis and is characterized by non-malignant lymphoproliferation, hepatosplenomegaly, autoimmune manifestations and increased risk of both Hodgkin's and non-Hodgkin's lymphoma. Most forms of the disease are due to germ line mutations of the FAS gene and manifest during the first years of life with fluctuating lymphadenopathies, hemolysis, immune thrombocytopenia. During the second decade of life disease manifestations improve spontaneously but autoimmune problems still occur and there is an increased risk of lymphoproliferative malignancy. We describe a typical case of ALPS in a now 44 year old man, followed since the age of 2 for disease manifestations that were unclear at the beginning.
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PMID:Autoimmune lymphoproliferative syndrome (ALPS). Case report and family history. 2088 45


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