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

The authors report a case of non Hodgkin lymphoma which, given its particular course and histomorphological features, can be classified as angiotropic large-cell lymphoma (i.e. proliferating angioendotheliomatosis). The most important characteristic is that the kidney was the target organ in this case, an observation rarely reported in the literature.
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PMID:Angiotropic large-cell lymphoma with predominant kidney involvement. A case report. 251 Nov 1

As pathologists, we are most concerned about overcalling reactive changes in the lung as carcinoma and the fact that malignant processes may be misinterpreted as benign processes in the lung is less well recognized. This review covers five such lesions. Well-differentiated adenocarcinomas, especially bronchioloalveolar carcinomas, are frequently undercalled, particularly in small biopsy and cytology specimens. In such cases, one must pay particular attention to the uniformity and monotony of the epithelium even though it may be extremely well differentiated. Spindle cell carcinomas may have necrosis and cavitation, interstitial growth, and a reactive fibroblastic reaction, and thus be mistaken as organizing inflammatory processes. Careful attention to the atypical cytological features, prominent vascular invasion, and getting immunohistochemical supports helps in recognizing them. Lymphoid lesions of the lung present a number of problems; small lymphocytic lymphomas and Hodgkin's disease are often misinterpreted as inflammatory processes. Intravascular lymphomatosis in the lung may be misinterpreted as an interstitial pneumonia if one does not appreciate the atypical lymphoid cells within the capillaries. The desmoplastic variant of sarcomatous mesothelioma may be extremely difficult to diagnose, because large portions of the tumors are composed of bland-appearing fibrous tissue. A case of desmoplastic mesothelioma presenting predominantly as a mediastinal mass is discussed, and problems in differential diagnosis are outlined. Angiosarcomas are rare tumors, but an appreciable percentage of them present as pulmonary metastases which may be interpreted as pulmonary hemorrhage or organizing infarction. The clinical and radiographic pattern, usually mimicking metastatic disease, and the fact that atypical spindle cells occlude small pulmonary arteries with surrounding alveolar hemorrhage are clues to the recognition of these lesions.
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PMID:Malignancies in the lung and pleura mimicking benign processes. 777 Jun 73

Angiotropic lymphoma (AL) is an unusual variant of extranodal lymphoma, characterized by massive proliferation of neoplastic lymphoid cells almost exclusively within blood vessels. Whereas the lymphoid origin of this disease is widely accepted it still remains unclear whether AL is a distinct entity that originates in the blood vessels or whether it represents a form of secondary intravascular dissemination of a primary solid lymphoma. The present case is unusual because death by right heart failure owing to extensive intravascular proliferation of neoplastic cells and subsequent occlusion of pulmonary blood vessels has not been described so far. In addition, the patient had suffered from a solid deposit of a large-cell B-lymphoma months before the angiotropic manifestation, suggesting that AL might develop out of more common types of non-Hodgkin's lymphomas.
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PMID:Angiotropic large-cell lymphoma presenting as pulmonary small vessel occlusive disease. 1092 30

Intravascular large B-cell lymphoma (IVLBL) is an uncommon form of non-Hodgkin lymphoma that is also known as malignant angioendotheliosis, intravascular lymphomatosis, and angiotropic large-cell lymphoma. The disease is characterized by a bizarre population of neoplastic cells, which are found systemically within vascular lumina. Although originally thought to be a neoplastic process of the endothelial cells, it has since been demonstrated, by molecular techniques and immunohistochemistry, that the neoplastic cells are of lymphoid origin. The differential diagnosis of these lesions includes granulocytic sarcomas that can be distinguished from IVLBL or other lymphomas by the presence of immunohistochemical positivity for myeloperoxidase. We describe a patient with a history of a myelodysplastic syndrome who subsequently developed IVLBL, which demonstrated immunohistochemical positivity for myeloperoxidase. To our knowledge, this represents the first case of a malignant lymphoma to demonstrate such findings.
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PMID:Myeloperoxidase-positive intravascular large B-cell lymphoma. 1141 84

Intravascular lymphomatosis is an unusual form of non-Hodgkin lymphoma characterized by intravascular proliferation of atypical lymphoid cells in multiple organs. It can cause systemic inflammatory response syndrome due to primary release of cytokines by the tumor cells or secondary release of cytokines after vascular occlusion by the tumor cells. It is a potentially fatal condition, because multiorgan failure can ensue due to thrombotic vascular occlusion. This is a very rare condition and most cases are diagnosed post mortem. We present a case of systemic inflammatory response syndrome and subsequent death from multiorgan failure in a patient with intravascular lymphoma.
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PMID:Intravascular lymphomatosis presenting as systemic inflammatory response syndrome. 1249 3

Intravascular lymphoma, also known as malignant angioendotheliomatosis or angiotropic lymphoma, is a rare non-Hodgkin lymphoma that is usually fatal. It often presents with cutaneous and/or nervous system involvement, but the disease can involve any organ system. Clinical symptoms result from the occlusion of small vessels by tumor cells and fibrin. We present a case of cutaneous intravascular lymphoma successfully treated with rituximab, a recombinant antibody to CD20 antigen found on B lymphocytes.
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PMID:Regression of cutaneous intravascular lymphoma with rituximab. 1295 38

Intravascular large B-cell lymphoma (LBCL) is a rare and aggressive subtype of diffuse LBCL characterized by disseminated intravascular proliferation of neoplastic lymphocytes. Obstruction of blood flow by tumor cells in a variety of organs can cause an array of clinical changes, including alteration of the neural and spinal system and the respiratory system, as well as skin lesions. It is usually very difficult to diagnose intravascular LBCL in a patient simply from clinical symptoms or laboratory examinations. We here document our findings that serum prostatic acid phosphatase levels in both males and a female (2.2-24.0 microg/L) reflect the presence of intravascular LBCL, changing synchronously in response to chemotherapy. To determine whether prostatic acid phosphatase (PAP) might be a useful tumor marker for early diagnosis, we reviewed five intravascular LBCLs. Immunohistochemically, tumor cells in all cases were positive for anti-PAP antibody. The results were further confirmed in one case by Western-blot analysis and in another by the detection of amplified messenger RNA for PAP in microdissected tumor cells, respectively. PAP has not been detected in 17 lymphomas (diffuse LBCL, 8 cases; follicular lymphoma, 3 cases; T-cell lymphoma, 3 cases; Hodgkin lymphoma, 3 cases) by Western blot analyses. We conclude that serum PAP is a useful tumor marker for intravascular LBCL and that it deserves further investigation in this context.
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PMID:Prostatic acid phosphatase is a possible tumor marker for intravascular large B-cell lymphoma. 1537 56

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

Intravascular large B-cell lymphoma (IVLBCL) is a distinct disease entity of non-Hodgkin lymphoma which is characterized in the current WHO classification by the selective growth of tumour cells in the lumina of small vessels of various organs. This rare disease entity has a high incidence of central nervous system (CNS) involvement at diagnosis. Although several retrospective analyses indicate that the treatment of this lymphoma has improved in the post-rituximab era, risk of CNS recurrence at 3 years still reaches ~25%. Considering the poor prognosis of IVLBCL patients with CNS recurrence, elucidating the optimal treatment strategies for this serious complication is vital in order to further improve its clinical management. Meanwhile, the efficacy of CNS prophylaxis for specific subtypes of lymphoma, for example Burkitt lymphoma and acute lymphocytic leukemia/lymphoblastic lymphoma, has been established, and several risk factors of CNS relapse for diffuse large B-cell lymphoma (DLBCL) were indicated by previous analyses. Considering that almost all IVLBCL patients have similar risk factors, CNS prophylaxis could also be effective, and thereby give rise to better clinical outcomes, in the treatment of IVLBCL, especially for patients without CNS involvement at initial diagnosis. Intensity regimens for CNS and systemic chemotherapy could be useful for patients with CNS involvement at initial diagnosis. This review describes the current understanding of clinical outcome and CNS involvement in IVLBCL, and discusses the optimal treatment strategy and future perspectives for CNS involvement of this rare lymphoma.
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PMID:[Treatment strategy for central nervous system involvement in intravascular large B-cell lymphoma]. 2151 26

Intravascular large B-cell lymphoma (IVLBCL) is a rare, aggressive and often fatal non-Hodgkin lymphoma characterized by preferential growth of malignant B-cells within the lumina of small vessels. Rituximab plus anthracycline-based chemotherapy is the current standard regimen for IVLBCL, however it has minimal efficacy in relapsed or refractory diseases. Recent clinical trials have shown a significant anti-lymphoma activity of mammalian target of rapamycin (mTOR) inhibitors in relapsed and refractory diffuse large B-cell lymphoma (DLBCL); however, the activation status of the mTOR pathway and the therapeutic potential of mTOR inhibitors in IVLBCL have not yet been studied. Here we described the clinicopathological features of 3 cases of IVLBCL diagnosed at our institutions, and evaluated the activation status of the mTOR signaling in these tumors. Our results showed that the mTOR complex 2 pathway was selectively upregulated in IVLBCL, as evidenced by a predominant nuclear localization of the activated form of mTOR (p-mTOR at Ser2448) with concomitant overexpression of nuclear p-Akt (Ser473) and vascular endothelial growth factor (VEGF)-A in the lymphoma cells. These data suggest that overactivation of mTOR pathway may play a role in lymphomagenesis of IVLBCL and mTORC2 inhibitors may be beneficial in treating IVLBCL.
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PMID:Intravascular large B-cell lymphoma: report of three cases and analysis of the mTOR pathway. 2213 26


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