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)

Diffuse nodular lymphoid hyperplasia (DNLH) of the intestine is an extremely rare lymphoproliferative disorder occasionally associated with non-Hodgkin lymphomas. We report an unusual case of DNLH of the entire colon, which resembled malignant lymphoma (particularly low-grade B-cell lymphoma) both on clinical and pathologic grounds. The patient was a 62-year-old Japanese woman who was found to have multiple polypoid lesions along the entire large intestine by colonoscopy. Abdominal computed tomography revealed hepatosplenomegaly and multiple mesenteric lymphadenopathy. Histologically, the lesion was characterized by numerous lymphoid follicles with active germinal centers and a diffuse infiltrate of lymphoid cells in the mucosa and submucosa. The present case appears to be an essentially benign condition bearing a resemblance, both clinically and histologically, to malignant lymphoma, and from which it can be distinguished by use of immunohistochemical or molecular analysis.
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PMID:Diffuse nodular lymphoid hyperplasia of the large bowel without hypogammaglobulinemia or malabsorption syndrome: a case report and literature review. 1249 Sep 83

It is now well recognized that hemophagocytic syndrome (HPS) is occasionally associated with malignant lymphomas. However, its association with Hodgkin's disease has been only rarely reported. We present here a 72-year-old woman manifesting with HPS as the primary and solitary clinical symptom of Hodgkin's disease. She had been suffering from high-grade fever and anemia for more than a month. Based on the findings in bone marrow aspirates, she was diagnosed as having HPS. In spite of extensive surveys including various cultures, serological tests for collagen disease, abdominal and cardiac sonography, chest computed tomography (CT), and renal biopsy, the origin of the fever was not determined. She was treated with steroid pulse therapy and then referred. Radiological studies revealed only mild hepatosplenomegaly and small lymph node swellings around celiac and common hepatic arteries. Reevaluation of the bone marrow specimen revealed the infiltration of small numbers of CD30-, CD15-, and EBER-1-positive large-sized lymphocytes with bizarre nucleus. Under the diagnosis of Hodgkin's disease, she was treated with combination chemotherapy containing pirarubicin, cyclophosphamide, vincristine, and prednisolone. However, it was not effective and she died of rapidly progressive hepatic failure on the 5th day of the chemotherapy. Autopsy was performed, which showed proliferation of lymphoma cells in para-aortic lymph nodes. We believe that diagnostic survey to rule out the underlying lymphoma should be vigorously performed for patients with hemophagocytic syndrome of unknown origin.
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PMID:Hemophagocytic syndrome as the primary clinical symptom of Hodgkin's disease. 1257 67

The haemophagocytic syndrome (HPS) is clinically characterized by fever, pancytopenia and hepatosplenomegaly. Usually it takes an acute course with a high mortality. The pathogenetic basis is inadequate activation of the immune system--in particular Th1-lymphocytes with subsequent overproduction of cytokines and extreme activation of macrophages with haemophagocytosis. The activated cells infiltrate organs, cause tissue damage and clinical manifestations of the syndrome. From the etiological aspect two forms exist: primary (familial) with autosomal recessive inheritance and the secondary form which forms a heterogeneous sub-group, caused as a rule by infection and/or a tumour. The prognosis seems somewhat more favourable in secondary forms. In treatment which is essentially the same for both forms, chemotherapy combined with immunosuppression proves useful, in more aggressive forms chemotherapy as used in the treatment of non-Hodgkin lymphomas. The only curative method is transplantation of haematopoietic stem cells which is also the treatment of first choice in the familial form of haemophagocytosis. In the submitted paper the authors present a review of contemporary knowledge on this treacherous and relatively rare entity. The haemopgagocytic syndrome should be always taken into account in the differential diagnosis of fever with an obscure etiology. The authors assume that the haemophagocytic syndrome is rarely considered in practice and therefore is usually inadequately diagnosed and thus not treated in time. In the conclusion the authors describe the case-records of a 26-year-old female patient with haemophagocytic syndrome which developed during pregnancy.
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PMID:[Hemophagocytic syndrome (case report and literature review)]. 1272 83

We report the case of a 64-year-old male in whom fever, cutaneous rash and hepatomegaly were the first manifestations of Hodgkin disease (HD). Histologically a dense lymphohistiocytic infiltrate with a granulomatous pattern was found in the skin infiltrate. A computed tomography scan revealed hepatosplenomegaly and a small retroperitoneal lymphadenopathy. An hepatic percutaneous biopsy showed a granulomatous infiltration with typical Reed-Sternberg cells. Cutaneous manifestations of HD are briefly reviewed. The authors underline that granulomatous infiltration of the skin as the first manifestation of lymphoma is a very rare feature. We also discuss the possible pathogenic mechanisms of skin granulomas.
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PMID:Cutaneous granulomas as the first manifestation of Hodgkin's disease. 1280 95

Relapse after anthracycline based combination chemotherapy is frequently seen in patients with aggressive non Hodgkin's Lymphomas (NHL), whereas complications such as secondary leukemia or solid tumor rarely occur. We report a patient with diffuse large cell (DLC) NHL and concurrent renal cancer, who developed acute myelofibrosis (AMF) later in the course of her disease. This 60-year-old female patient presented with pancytopenia and a right sided renal mass. Diagnostic work up revealed severe bone marrow infiltration by DLC NHL and renal cancer T1N0M0G2. Cytogenetic and molecular evaluation of bone marow cells showed three distinct clones, (a normal 46XX karyotype, a ringed chromosome 7 and a third clone with an enlarged chromosome 2 as well as several fragments). The patient underwent nephrectomy and eventually received 6 cycles of CHOP 14 chemotherapy. Anemia persisted followed by severe granulocytopenia and thrombocytopenia 6 weeks later. Repeated bone marrow biopsy showed absence of lymphoma and/or cancer metastasis, but massive myelofibrosis with an increased number of atypical megakaryocytes. Considering the short clinical course and the absence of hepatosplenomegaly AMF was diagnosed. The concurrence of three distinctneoplasms within a short period of time as well as the complex cytogenetic aberrations found in her bone marrow cells reflect a strong individual susceptibility to malignant disease in this patient.
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PMID:Acute myelofibrosis in a patient with diffuse large cell non Hodgkin's lymphoma and renal cancer. 1456 65

The occurrence of abnormal Hodgkin's and Reed-Sternberg cells in the peripheral blood in a patient suffering from Hodgkin's disease has been noticed exceptionally rare in a previous period, and especially rare in last ten years primarily due to successful treatment of this disease. The presence of atypical mononuclear cells in peripheral blood which cytomorphologically resembled Reed-Sternberg cells was registered in 8 patients till 1966. During the last decade, the presence of atypical mononuclear cells in the peripheral blood was used for their isolation, cultivation, and detailed immunophenotypic and genetic analysis. The analysis of mononuclear cells in rare patients with Hodgkin's disease was established that they belong to the B-lymphoid cells with expression of CD30 and CD15 antigens. The examination of presence of Hodgkin's cells in the peripheral blood of patients with Hodgkin's disease is important for patients with advanced stage of the disease in which autologous stem cell transplantation and high dose chemotherapy is planned. The authors present a 33-year-old patient, who noticed enlarged neck lymph nodes in September 2000, high temperature and loss in weight. On physical examination enlarged neck lymph nodes 5 x 8 cm and hepatosplenomegaly were found. There was anemia and thrombocytopenia, and normal WBC count with 24% of lymphoid elements in differential formula. On histologic examination of lymph nodes, Hodgkin's disease, type nodular sclerosis with mixed cellularity was found. Histology of bone marrow showed nodal lymphomatous infiltration. Immunohistochemistry with monoclonal antibodies of concentrate of peripheral blood cells showed expression of CD30+ and CD15+, immunophenotypically and morphologically matching Reed-Sternberg cells. Cytogenetic analysis of mononuclear cells of the bone marrow showed normal karyotype. The patient was in clinical stage IV/V of the disease and chemotherapy with 9 cycles of ABVD + Mp protocol was applied. He is still in remission.
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PMID:[Hodgkin's and Reed-Sternberg cells in the peripheral blood of a patient with advanced stage Hodgkin's disease]. 1505 20

Autoimmune Lymphoproliferative Syndrome (ALPS) is generally the result of a mutation in genes associated with apoptosis, like Fas, Fas ligand, Casp 8 and Casp 10. As a result, the normal homeostasis of T- and B-lymphocytes is disturbed and a proliferation of polyclonal T lymphocytes occurs. This leads to hepatosplenomegaly and lymphadenopathy and in most patients also to autoimmune phenomena like anemia and thrombocytopenia. The proliferating T cells are TCRalphabeta and/or TCRgammadelta positive but lack both CD4 and CD8. Hence they are termed double negative (DN) T cells. In addition, there is an increase of CD5 positive B cells. Individuals with germline mutations in the Fas gene have a high risk to develop non Hodgkin lymphomas (x 14) as well as Hodgkin lymphomas (x 51), in particular NLP Hodgkin lymphoma. Somatic mutations of Fas are frequently acquired during the normal germinal center reaction. Non Hodgkin lymphomas carry somatic mutations of the Fas gene in 11% and of the Casp 10 gene in 14.5% of the patients. In Hodgkin lymphomas, Fas mutations can be demonstrated in Reed-Sternberg cells in 10-20% of the patients. These data implicate a role for Fas-mediated apoptosis in preventing lymphomas. Inherited defects in receptor-mediated lymphocyte apoptosis represent a risk factor for lymphomas and somatic mutations of these genes may also play a role in the development and/or progression of lymphomas.
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PMID:Development of lymphoma in Autoimmune Lymphoproliferative Syndrome (ALPS) and its relationship to Fas gene mutations. 1516 Sep 2

We describe the case of a 47-year-old man admitted to the Department of Hematology because of fever, enlarged cervical and supraclavicular lymph nodes, hepatosplenomegaly and non-specific lung infiltrations. The histopathological examination of the cervical lymph node revealed Hodgkin's lymphoma (HL) NS type I. Clinical evaluation revealed stage IVB according to Ann Arbor classification and the presence of 5 unfavorable prognostic factors according to the International Prognostic Index. Despite BEACOPP chemotherapy (bleomycin, etoposide, doxorubicin, cyclophosphamide, vincristine, procarbazine, and prednisone), the enlarged lymph nodes, lung infiltrations and fever persisted. Microbiological and serological tests did not lead to the identification of any viral or bacterial pathogens. Bronchoscopy showed chronic inflammation and post-tuberculosis (TB) scars in bronchi without acid-fast bacilli in bronchoalveolar lavage (BAL) in culture and polymerase chain reaction (PCR) tests. However, the biopsy of the supraclavicular lymph node revealed multiple, caseating and necrotizing granulomatous lesions with scattered Reed-Sternberg (R-S) cells. The auramin staining presented acid-fast bacilli and allowed the diagnosis of productive and caseating TB coexisting with HL. The 4 tuberculostatics regimen and ABVD chemotherapy (adriamycin, bleomycin, vincristine, dacarbazine) resulted in a complete clinical response after 3 months of treatment. In conclusion, the association between HL and TB must be considered, especially in countries where the latter is endemic. The diagnosis may be difficult due to similarities in the clinical course, laboratory tests and imaging procedures.
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PMID:Hodgkin's lymphoma and tuberculosis coexistence in cervical lymph nodes. 1562 42

The BCL-2 family has been implicated in the pathogenesis of various hematopoietic malignancies, including follicular non-Hodgkin lymphoma and B-cell chronic lymphocytic leukemia. To identify genes that act synergistically in BCL2-enforced leukemogenesis, we developed a murine B-cell lymphoma/leukemia model based on the IL-3-dependent Balb/C pro-B line (FL5.12). FL5.12 cells were stably transfected with antiapoptotic BCL-2 alone or in combination with proapoptotic BAX or nonfunctional mutant BAX, thereby creating various levels of imbalance within the BCL-2 family. Transfectants were intravenously injected into normal Balb/C mice. Whereas FL5.12 cells did not provoke leukemia, mice injected with stable transfectants died of leukemia over time. Disease incidence and latency time depended on the degree of imbalance in the BCL-2 family, supporting a model whereby BCL2 drives tumorigenesis. All mice presented with hepatosplenomegaly and leukemic FL5.12 cells in peripheral blood and bone marrow compartments. Leukemic conversion was accompanied by secondary genetic aberrations leading to clonal IL-3-responsive leukemia. Cellular transformation was independent of alterations in c-Myc or downstream apoptotic pathway. Leukemic clones retained a normal DNA damage response leading to elevated P53 and P21 levels and cell cycle arrest upon irradiation. In conclusion, our mouse model may prove a valuable tool to identify genes that cooperate in BCL2-enforced lymphoma/leukemogenesis.
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PMID:Novel murine B-cell lymphoma/leukemia model to study BCL2-driven oncogenesis. 1564 25

Several lymphoproliferative disorders may be interpreted as multicentric Castleman's disease (MCD) clinicopathologically. These include HIV infection, autoimmune-disease-associated lymphadenopathy, idiopathic plasmacytic lymphadenopathy with polyclonal hyperimmunoglobulinemia, "idiopathic MCD", POEMS syndrome (polyneuropathy, anasarca, organomegaly, endocrinopathy, M-proteins, and skin lesions), and non-Hodgkin's lymphomas. Among these, idiopathic MCD appears to be relatively rare. We report on the clinicopathologic and immunohistologic findings of five cases of idiopathic MCD and discuss the problems regarding their differential diagnosis. Some of the characteristic clinical findings of POEMS syndrome, including hepatosplenomegaly, skin change, endocrine abnormalities and anasarca, were present in all five cases. However, during the course of disease, minimal diagnostic criteria for POEMS syndrome, i.e., monoclonal plasma cell proliferation and sensory motor neuropathys, were absent in all five cases. The serum interleukin-6 level and the vascular endothelial growth factor level were found to be elevated in two of the cases examined. Various autoantibodies were detected in three cases. However, none of them fulfilled the diagnostic criteria for any of the definite autoimmune-disease. Histologically, three lesions exhibited a mixed type of Castleman's disease, and two exhibited the hyaline-vascular type. The majority of the germinal centers were of the hyaline-vascular or epithelioid germinal type, with a few hyperplastic germinal centers. The interfollicular area was characterized by prominent vascularity. Moderate to large sheets of plasma cells were observed in three mixed type cases. The polytypic nature of B-lymphocytes was demonstrated by immunohistochemistry and polymerase chain reaction. Immunohistochemical study demonstrated that the majority of germinal centers exhibited a tight/concentric pattern of FDC network. Few CD57-positive T-cells were observed in the hyaline-vascular and epithelioid follicles. The lack of CD57-positive T-cells appears to be related to the formation of abnormal germinal centers in the MCD.
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PMID:Idiopathic multicentric Castleman's disease. A clinicopathologic and immunohistochemical study of five cases. 1599 40


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