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)

An acute terminal phase of a Bence Jones kappa plasma cell myeloma developed 22 months after chemotherapy is presented. The patient's symptoms were fever, cytopenias, adenomegalies and hepatomegaly. A lymph node biopsy showed an immunoblastic polymorphic kappa B cell lymphoma, expressing CD30 antigen. After new polychemotherapy the patient died because of bleeding and infection. Autopsy revealed a decrease in the tumour with defective immunophenotype. The few reported cases have been reviewed with emphasis on clinical aspects, prognosis and morphology, The significance of CD30 positivity (activation marker) in a high-grade lymphoma is discussed.
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PMID:Malignant B cell immunoblastic lymphoma expressing CD30 antigen in the terminal phase of a multiple myeloma. 256 14

The natural history of follicular lymphoma is to accrue large cells and become diffuse, resulting in progression/transformation to a higher-grade lymphoma. Histologic transformation occurs in approximately 60% of patients. Most often, follicular lymphomas transform into diffuse large cell lymphoma, but transformation to lymphomas classified using the Working Formulation as diffuse mixed, large cell immunoblastic, or small noncleaved cell also have been reported. Evidence of transformation may be found over time in sequential biopsy specimens, or may coexist in the same biopsy specimen. Here, we describe six cases of follicular lymphoma, large cell in five cases and mixed in one case, that transformed into a diffuse or sinusoidal CD30 antigen-positive large cell lymphoma with anaplastic cytologic features. Both the follicular and diffuse/sinusoidal components were of B-cell lineage, positive for the CD20 antigen and negative for the CD3 and CD43 antigens. The neoplastic cells expressed monotypic immunoglobulin light chain in five cases, three kappa and two lambda. BCL-2 protein was positive in four tumors, in both the follicular and diffuse/sinusoidal components in three cases, and only in the latter component in one case. Using the polymerase chain reaction (PCR), three of six cases had monoclonal immunoglobulin heavy chain gene rearrangements. The t(14;18) was not amplified in any case. Using reverse transcriptase (RT)-PCR, the t(2;5) was amplified in one of four tumors. This report highlights the heterogeneity of B-lineage anaplastic large cell lymphomas and indicates the need to consider antecedent follicular lymphoma in any B-cell lymphoma with anaplastic cytologic features.
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PMID:Transformation of follicular lymphoma into CD30-large cell lymphoma with anaplastic cytologic features. 915 76

The tumor necrosis factor (TNF) receptor-associated factor 1 (TRAF1) is a member of the recently defined TRAF family. It takes part in the signal transduction of the TNF receptor 2 (TNFR2), the lymphotoxin-beta receptor (LT-betaR), CD40, CD30, and LMP1; is induced by LMP1 in vitro; and protects lymphoid cells from apoptosis. To identify the cells in which TRAF1 is active in vivo, we studied TRAF1 transcripts in normal lymphoid tissue, in Epstein-Barr virus (EBV)-induced lymphoproliferations, and in malignant lymphomas with special reference to those that overexpress the cytokine receptor CD30 and CD40 of the TNF receptor family at the single-cell level using a radioactive in situ hybridization. In normal lymphoid tissue, TRAF1 message proved to be absent from all resting B and T cells as well as from macrophages and accessory cells (follicular dendritic cells and interdigitating cells) and present in few perifollicular and intrafollicular lymphoid blasts. In contrast, there was a high and consistent TRAF1 overexpression in EBV-induced lymphoproliferations and Hodgkin's disease. Nearly all non-Hodgkin's lymphoma show low or no TRAF1 expression. Only some cases of diffuse large B-cell lymphoma showed a moderate to high TRAF1 signal. Several of the latter cases were EBV+. These data confirm that TRAF1 is an inducible molecule and indicates its deregulation in the mentioned disorders with the potential of a blockage of the apoptotic pathway.
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PMID:Tumor necrosis factor receptor-associated factor 1 is overexpressed in Reed-Sternberg cells of Hodgkin's disease and Epstein-Barr virus-transformed lymphoid cells. 988 24

Anaplastic large cell lymphoma (ALCL) has been recognized recently as a distinct clinicopathologic entity, restricted to a subset of CD30-positive diffuse large cell lymphomas of T/null lineage. Some of the characteristic features of ALCL, such as CD30 antigen expression and the presence of large pleomorphic lymphoid cells infiltrating lymph node sinuses, can be found rarely in diffuse large B-cell lymphomas. We collected 11 such cases, and their clinical, morphologic, and immunophenotypic features are reviewed. The age of the patients ranged from 36 to 82 years (mean, 63.2 years) with a male to female ratio of 1:1.2. All neoplasms were nodal with a sinusoidal infiltrative pattern, although four neoplasms also had foci of confluent growth. Eight tumors were composed predominantly of large pleomorphic cells with occasional Reed-Sternberg-like cells. The other three tumors had a higher proportion of large monomorphic lymphoid cells. Necrosis and admixed granulocytes were other common features. Immunophenotypically, all cases were positive for CD30 and CD20 or CD79a. All eight cases examined for anaplastic lymphoma kinase-1 immunoreactivity were negative. In situ hybridization for Epstein-Barr virus RNA was performed in eight cases; two were positive. Excluding one consultation case with no available clinical follow-up data, six patients died of the disease within 3 years and one had disease relapse within 1 year. We conclude that an unusual variant of diffuse large B-cell lymphoma can closely mimic ALCL. However, these neoplasms can be distinguished from ALCL by virtue of their B-lineage and lack of anaplastic lymphoma kinase-1 expression. Evidence of Epstein-Barr virus infection can be found in a small subset of these neoplasms.
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PMID:Sinusoidal CD30-positive large B-cell lymphoma: a morphologic mimic of anaplastic large cell lymphoma. 1075 32

Fifteen years after their first description by one of the authors (HS) anaplastic large cell lymphoma (ALC-lymphoma, ALCL) now represents a generally accepted group of large cell lymphomas. Essential defining features comprise of a proliferation of large lymphoid cells with strong expression of the cytokine receptor CD30 and a characteristic growth pattern. Using molecular and clinical criteria three entities of ALC-lymphoma have been identified: primary systemic anaplastic lymphoma kinase (ALK)-positive ALC-lymphoma, primary systemic ALK-negative ALC-lymphoma and primary cutaneous ALC-lymphoma. The ALK expression in the primary systemic ALC-lymphoma entity is caused by chromosomal translocations, most commonly t(2;5), and can nowadays be reliably detected by immuno-histology. ALK-positive ALC-lymphoma predominantly affects young male patients and if treated with chemotherapy has a favourable prognosis. They show a broad morphological spectrum, with the "common type", the small cell variant and the lymphohistiocytic variant being most commonly observed. The knowledge of the existence of these variants is essential in establishing the correct diagnosis. ALK-negative ALC-lymphomas occur in older patients, equally affecting both genders and have an unfavorable prognosis. The morphology and the immuno-phenotype of primary cutaneous ALC-lymphoma shows an overlap with that of lymphomatoid papulosis. Both diseases have an excellent prognosis and secondary systemic dissemination is only rarely observed. The ALC-lymphomas described above derive from T cells and are generally accepted as biological entities. In contrast, large B-cell-lymphomas with anaplastic morphology are now believed not to represent an own entity but a morphologic variant of diffuse large B-cell lymphoma. Malignant lymphomas with morphological features of both Hodgkin- and ALC-lymphoma have formerly been classified as ALCL Hodgkin-like. Recent immuno-histological analysis of these cases however suggests that ALCL Hodgkin-like does not represent an own lymphoma entity. Most of these cases are likely to be examples of tumor cell rich classical Hodgkin lymphoma, while a minority of these cases appear to fall either into the category of ALK-positive or ALK-negative ALC-lymphoma.
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PMID:[The many faces of anaplastic large cell lymphoma]. 1084 Aug 18

Anaplastic large cell lymphoma (ALCL) represents a generally recognized group of large cell lymphomas. Defining features consist of a proliferation of predominantly large lymphoid cells with strong expression of the cytokine receptor CD30 and a characteristic growth pattern. With the use of molecular and clinical criteria, 3 entities of ALCL have been identified: primary systemic anaplastic lymphoma kinase (ALK)(+) ALCL, primary systemic ALK(-) ALCL, and primary cutaneous ALCL. ALK expression is caused by chromosomal translocations, most commonly t(2;5). ALK(+) ALCL predominantly affects young male patients and, if treated with chemotherapy, has a favorable prognosis. It shows a broad morphologic spectrum, with the "common type," the small cell variant, and the lymphohistiocytic variant being most commonly observed. The knowledge of the existence of these variants is essential in establishing a correct diagnosis. ALK(-) ALCL occurs in older patients, affecting both genders equally and having an unfavorable prognosis. The morphology and the immunophenotype of primary cutaneous ALCL show an overlap with that of lymphomatoid papulosis. Both diseases have an excellent prognosis, and secondary systemic dissemination is only rarely observed. The described ALCL entities usually derive from cytotoxic T cells. In contrast, large B-cell lymphomas with anaplastic morphology are believed to represent not a separate entity but a morphologic variant of diffuse large B-cell lymphoma. Malignant lymphomas with morphologic features of both Hodgkin disease and ALCL have formerly been classified as Hodgkin-like ALCL. Recent immunohistologic studies, however, suggest that ALCLs Hodgkin-like represent either cases of tumor cell-rich classic Hodgkin disease or (less commonly) ALK(+) ALCL or ALK(-) ALCL. (Blood. 2000;96:3681-3695)
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PMID:CD30(+) anaplastic large cell lymphoma: a review of its histopathologic, genetic, and clinical features. 1109 48

Post-transplant lymphoproliferative disorders (PTLDs) are potentially fatal, often Epstein-Barr virus (EBV)-driven neoplasias developing in immunocompromised hosts. Initial treatment usually consists of a reduction in immunosuppressive therapy and/or rituximab with or without chemotherapy. However, patients who relapse do poorly, and new treatment options are warranted. With the introduction of the immunoconjugate brentuximab vedotin, the CD30 antigen has become an effectively targetable molecule. Therefore, we investigated the frequency and level of CD30 expression in PTLDs. We identified 108 patients with PTLDs diagnosed during 1994-2011, of whom 62 had adequate paraffin-embedded tissue for tissue microarray construction. Immunohistochemical expression of CD30 was consistently detected in all types of PTLD (overall 85.25%), including the monomorphic subtypes, and was correlated with a more favorable outcome. For diffuse large B-cell lymphoma (DLBCL)-type PTLD this was regardless of EBV status, and remained significant in multivariate analysis. Cell-of-origin had no independent prognostic value in our series of DLBCL PTLD.
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PMID:Occurrence and prognostic relevance of CD30 expression in post-transplant lymphoproliferative disorders. 2524 78

The CD30 antigen is strongly expressed on neoplastic cells in classical Hodgkin lymphoma (HL), anaplastic large cell lymphoma (ALCL) and other hematologic malignancies (such as DLBCL and cutaneous TCL), while is almost undetectable on healthy tissues, representing an ideal immunotherapeutic target. Since unconjugated anti-CD30 antibody (SGN-30) demonstrated limited clinical activity, researchers' effort aimed to create an antibody-drug conjugate (ADC), leading to discovery of SGN-35 (brentuximab vedotin), in which an anti-CD30 antibody is linked to the antimitotic agent monomethyl auristatin E (MMAE). In the first phase I study in CD30+ hematologic malignancies (the majority of patients with HL), the maximum tolerated dose was fixed respectively at 1.8mg/Kg every 3 weeks, overall response rate (ORR) and complete response (CR) rate were 38% and 24%. In 2 subsequent phase II studies, amazing results were reported, that permitted accelerated FDA approval for relapsed/refractory patients and led to the development of many clinical trials including BV as first-line HL and ALCL treatment. Moreover, as CD30 antigen may be expressed by other malignancies, the potential therapeutic application is increasing, including at least diffuse large B-cell lymphoma, T-cell lymphomas other than ALCL and cutaneous lymphoproliferative disorders. BV is administrated as outpatient regimen and is usually well tolerated; sensorial peripheral neuropathy represents the most common toxic effect, although it is dose-dependent and at least partially reversible in most cases, after dose reduction and/or treatment ending.
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PMID:Therapeutic Use of Brentuximab Vedotin in CD30+ Hematologic Malignancies. 2759 44

CD30 is a member of the tumor necrosis factor receptor superfamily, member 8 (TNFRSF8), and its normal expression is restricted to activated T and B cells. In tumor cells, CD30 expression is most commonly associated with lymphoid malignancies (Hodgkin and non-Hodgkin lymphomas) and is a therapeutic target using anti-CD30 antibody. CD30 expression has been reported also in mostly adult non-lymphoid malignancies, raising the possibility of CD30-targeted therapy for additional tumors. In this study, we examined the incidence of CD30 expression in 251 hematopoietic and 334 non-hematopoietic cases of pediatric tumors. As expected, strong and membranous CD30 staining was seen in anaplastic large cell lymphoma, classical Hodgkin lymphoma, and embryonal carcinoma while variable staining was seen in diffuse large B cell lymphoma. In addition, positive CD30 staining was also seen in cases of neuroblastoma (33 of 56), neoplasm with chondroid differentiation (8 of 25), myeloid neoplasms (11 of 120), hemangioma (2 of 12), and mature teratoma (1 of 11). In neuroblastoma, the CD30 expression was generally restricted to cells with ganglion differentiation; staining of ganglion cells was also seen in the one positive case of mature teratoma. In neoplasm with chondroid differentiation, the positive cases were chondrosarcoma (3 of 5), chondroblastic osteosarcoma (2 of 10), and chondroblastoma (3 of 7). In acute myeloid leukemia, the CD30 positive cases were more common in AML with monocytic differentiation but did not correlate with any specific molecular change. We conclude that CD30 expression in pediatric tumors is more general than anticipated and future studies are warranted to understand the biologic and therapeutic significances.
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PMID:CD30 Expression in Pediatric Neoplasms, Study of 585 Cases. 2852 33