Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0079731 (B-cell lymphoma)
 16,671 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 43-year-old woman had multiple, painful nodules of the legs and edema of both lower extremities for 3 years and leukopenia for 8 years. This histologic findings of lobular histiocytic panniculitis with "beanbag" cells were typical of cytophagic panniculitis. This case is unique in that B cell lymphoma developed in the spleen, as verified by immunoperoxidase staining, and demonstrates that the disease not only may be a progressive disease per se but also may be associated with nonhistiocytic malignancy.
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PMID:Cytophagic panniculitis and B cell lymphoma. 387 44

The classical symptoms of Lyme borreliosis (LB) on the skin, erythema migrans, borrelia lymphocytoma and acrodermatitis chronica atrophicans (ACA) can be diagnosed clinically. In atypical cases, however, the diagnosis of a Borrelia burgdorferi (Bb) infection is based on the isolation of this bacterium from affected tissues. In early Lyme disease problems are arising in complicated erythema migrans which may represent reinfection, or with a failure of antibiotic treatment in some patients. The pathological changes in ACA presumably result from a chronic T-cell-mediated tissue injury with atrophy or sclerosis of connective tissue. On the other hand the presence of numerous plasma cells indicates activation of the humoral immune system where a progression into a malignant B-cell lymphoma is possible. In the recent past Bb could be cultivated from skin biopsies of a series of dermatoses of unknown origin, such as circumscribed scleroderma, dermatomyositis-like syndrome, relapsing nodular panniculitis, granuloma anulare and roseolar erythemas. New therapeutic strategies by administering antibiotics have been found to be effective in the described Bb induced dermatoses. Future studies should be focused on new standardized diagnostic procedures which make it possible to define the spectrum of dermatoses associated with a Bb infection.
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PMID:[The dermatologic spectrum of Lyme borreliosis]. 761 Jun 65

Eleven patients with angiocentric immunoproliferative lesion (AIL) of the skin were studied. Histologically, three patients were grouped into AIL grade II (AIL-II), whereas eight showed angiocentric lymphoma (AIL-III). All the patients' specimens exhibited lobular panniculitis. Infiltrating atypical lymphocytes in nine patients possessed electron-dense membrane bound granules in electron microscopy. Phenotypically, the lymphoid cells in the AIL-II patients were positive for CD3 epsilon; two of these showed a positive reaction to CD2, CD7, and CD8, but lacked natural killer-associated (NKa) antigens CD16, CD56, and CD57. In six AIL-III patients, lymphoma cells were positive for CD2 in all patients, CD56 in five, CD3 epsilon in four, CD7 in four, interleukin-2 beta receptor in four, a pore-forming protein in four, and CD30 in three patients. The remaining two AIL-III patients had B-cell lymphoma. By the Southern blot analysis, three patients with AIL-III showed a rearranged T-cell-receptor beta-gene or a deletion of its germline. The preceding results in nine of 11 patients suggest that abnormal or neoplastic large granular lymphocytes with the characteristics of T and NK cells have an important role in producing the angiocentric/angiodestructive features and lobular panniculitis. Clinically, all three patients with AIL-II and four with AIL-III showed liver dysfunction, cytopenia, and abnormal coagulopathy during the clinical course. Five patients with AIL-III died within 8 months. The histological grading of AIL, patients' age, and limited clinical stage of the disease seem to correlate with response to the treatment and prognosis.
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PMID:Angiocentric immunoproliferative lesions of the skin show lobular panniculitis and are mainly disorders of large granular lymphocytes. 852 4

Malignant angioendotheliomatosis is a rare intravascular (angiotropic) lymphoma. Patients most often present with cutaneous or central nervous system findings. We describe three patients with malignant angioendotheliomatosis involving the skin. The initial lesions in each were tender, indurated nodules on the lower extremities, resembling inflammatory panniculitis. Skin biopsies and immunohistochemical studies from all patients confirmed intravascular B-cell lymphoma. Two patients had visceral involvement, and molecular genetics studies showed clonal immunoglobulin gene rearrangement in one. Electron microscopy in this case showed increased fibrin and atypical lymphocytes within blood vessels. Malignant angioendotheliomatosis is a monoclonal intravascular lymphoma, usually of B-cell phenotype. Occlusion of small blood vessels with lymphoid cells, fibrin, and degenerating cellular debris causes the cutaneous lesions. An excisional biopsy through the depth of subcutaneous tissue may be necessary to confirm the diagnosis of malignant angioendotheliomatosis.
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PMID:Malignant angioendotheliomatosis is an angiotropic intravascular lymphoma. Immunohistochemical, ultrastructural, and molecular genetics studies. 859 32

Several subtypes of human malignant lymphomas are known to be highly associated with the Epstein-Barr virus. These include the Burkitt's lymphoma, opportunistic (immune deficiency-associated) lymphoma, nasal T/NK lymphoma, Hodgkin's disease pyothorax-associated lymphoma, cutaneous panniculitis-type lymphoma, and mediastinal large B-cell lymphoma. Improvement of histopathological technology, the demonstration of EBV-encoded small RNAs(EBERs), as well as the molecular virological methods, contributed much in the progression of such EBV-associated lymphomas.
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PMID:[EBV-associated lymphoma]. 904 29

The presence of a granulomatous reaction in lesions of cutaneous lymphomas has been described in the past in several cases. Especially in mycosis fungoides, a "granulomatous" variant of the disease has been well characterized. We studied the clinicopathologic features of cutaneous lymphomas with prominent granulomatous reaction, including both cutaneous T-cell lymphomas and B-cell lymphomas (primary cutaneous lymphoma 22, secondary cutaneous lymphoma one). Biopsies of 23 patients with histopathologic features of cutaneous T-cell lymphoma or cutaneous B-cell lymphoma with prominent granulomatous reaction were included in this study. A prominent granulomatous reaction was defined as the presence of a granulomatous component exceeding 25% of the dermal infiltrate. There were 14 cases of mycosis fungoides, two of subcutaneous panniculitis-like T-cell lymphoma, four of small/medium pleomorphic T-cell lymphoma, one of follicle center cell lymphoma, one of large B-cell lymphoma, and one of secondary cutaneous peripheral T-cell lymphoma. Altogether, a prominent granulomatous reaction could be observed in 1.8% of all patients with cutaneous lymphoma (primary or secondary) registered in the files of the Department of Dermatology of the University of Graz (Graz, Austria), demonstrating that there is a distinct, albeit small, proportion of cases revealing this peculiar reaction pattern. In seven cases a misdiagnosis of granulomatous dermatitis preceded the correct diagnosis for a period of 1-216 months, suggesting that sequential biopsies and complete phenotypic and molecular genetic analyses should be carried out in cases of "unusual" granulomatous dermatitis.
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PMID:Cutaneous lymphomas with prominent granulomatous reaction: a potential pitfall in the histopathologic diagnosis of cutaneous T- and B-cell lymphomas. 1271 56

The best treatment of type II mixed cryoglobulinemia (MC) has still to be defined. Antiviral treatment for the frequent underlying infectious trigger hepatitis C virus (HCV) may be ineffective, contraindicated, or not tolerated in a fraction of cases, whereas current immunosuppressive treatments may lead to relevant complications. Selective B-cell blockade with rituximab was used in this study, based on favorable results in preliminary experience. Fifteen consecutive patients with type II MC (HCV-related in 12 of 15) were treated with rituximab, 375 mg/m(2) intravenously weekly for 4 weeks. Only medium- to low-dose steroids were allowed, if already administered at the time of recruitment. All patients had active disease, poorly controlled or difficult to manage with previous treatments, including corticosteroids in all. Efficacy and safety of rituximab therapy were evaluated in the following 6 months. The overall follow-up after rituximab treatment ranged from 9 to 31 months. Rituximab proved effective on skin vasculitis manifestations (ulcers, purpura, or urticaria), subjective symptoms of peripheral neuropathy, low-grade B-cell lymphoma, arthralgias, and fever. Nephritis of recent onset went into remission in one case. Laboratory features, that is, significantly decreased serum rheumatoid factor and cryoglobulins and increased C4, were consistent with the clinical efficacy. Treatment was well tolerated, with no infectious complications. Thrombosis of retinal artery or self-limiting panniculitis occurred in one patient each. Rituximab may represent a safe and effective alternative to standard immunosuppression in type II MC. Controlled studies are needed to better define drug indications and the cost-efficacy profile in the different systemic manifestations.
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PMID:Efficacy and safety of rituximab in type II mixed cryoglobulinemia. 1256 Feb 25

The clinicopathologic characteristics of malignant lymphomas vary according to geography. The aim of this study was to determine the relative frequency of cutaneous lymphomas and to examine the clinical relevance of the WHO classification in Korean cases of cutaneous lymphoma. The Korean Dermatopathology Research Group conducted a clinicopathologic review of a nationwide collection of 80 cutaneous lymphomas, diagnosed at 23 institutes over a recent 3-year period. The clinical records, haematoxylin & eosin-stained slides and immunohistochemical stains from 80 patients with malignant lymphomas of the skin were reviewed. In our study, the most frequent cutaneous lymphoma was mycosis fungoides. Compared with Western countries, Korea had higher rates of NK/T cell lymphoma and subcutaneous panniculitis-like T-cell lymphoma and a much lower rate of B-cell lymphoma. The occurrence rates for various subtypes of malignant lymphoma in Korea are distinct from those in Western countries. The EORTC classification is not fully appropriate in dealing with Korean cases of cutaneous lymphoma, because NK/T cell lymphoma is not included in the EORTC classification for cutaneous lymphoma.
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PMID:Characteristics of cutaneous lymphomas in Korea. 1461 33

In this report the case of intravascular large B-cell lymphoma (angiotropic lymphoma, intravascular angioendotheliomatosis, malignant endotheliomatosis) with panniculitis features is present. The diagnosis was based on finding of typical CD20 positive tumorous cells within capillaries, veins, and small arteries in the biopsy of subcutaneous adipose tissue. An accurate and early diagnosis together with combined chemotherapy resulted in good response and remission of the disease.
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PMID:Intravascular large B-cell lymphoma presenting as cutaneous panniculitis. 1467 22

The bcl-3 gene at chromosome 19q13 encodes a member of the IkappaB family involved in regulating the NFkappaB pathway. Originally identified by its involvement in the rare t(14:19)(q32;q13), BCL-3 expression has never been analyzed in a wide variety of lymphomas. We assessed BCL-3 expression in 353 cases of non-Hodgkin lymphoma and Hodgkin lymphoma using formalin-fixed, paraffin-embedded tissue specimens, a monoclonal antibody specific for BCL-3, and immunohistochemical methods. Of 172 B-cell lymphomas, 10 (6%) were positive for BCL-3, including six of 23 (26%) diffuse large B-cell lymphoma, one of 17 (6%) small lymphocytic lymphoma, one of 26 (4%) follicular lymphoma, and two of 49 (4%) mantle cell lymphoma. All other B-cell neoplasms were negative, including marginal zone lymphoma (n=24, 11 extranodal, nine nodal, four splenic), Burkitt lymphoma (n=10), lymphoplasmacytic lymphoma (n=10), lymphoblastic lymphoma (n=8), and plasmacytoma (n=5). Of 111 T/NK-cell lymphomas, 25 (23%) were positive for BCL-3, including 13 of 40 (32%) anaplastic large-cell lymphoma, three of 10 (30%) angioimmunoblastic T-cell lymphoma, two of eight (25%) extranodal NK/T-cell lymphoma of nasal type, three of 12 (25%) mycosis fungoides, one of five (20%) enteropathy-type T-cell lymphoma, and two of 21 (10%) peripheral T-cell lymphoma unspecified. All other T-cell neoplasms were negative, including lymphoblastic lymphoma (n=6), prolymphocytic leukemia (n=6), and subcutaneous panniculitis-like T-cell lymphoma (n=3). Of 70 Hodgkin lymphomas, of all types, 29 (41%) were positive for BCL-3. The relatively high frequency of BCL-3 expression in some non-Hodgkin and Hodgkin lymphoma types raises the possibility that BCL-3 is involved in the pathogenesis of these tumors, and may be a target of new therapies.
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PMID:Immunohistochemical detection of BCL-3 in lymphoid neoplasms: a survey of 353 cases. 1510 10


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