Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0268596 (EMA)
 2,520 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 77-year-old woman with primary esophageal non-Hodgkin's lymphoma in clinical stage IEA (Ann Arbor Classification) developed pain and difficulty in swallowing. An upper gastrointestinal examination revealed a submucosal tumor from the upper to the middle portion of the esophagus. Histopathological examination at endoscopic biopsy with endoscopic partial incision showed non-Hodgkin's lymphoma (diffuse type--large cell). Immunohistological examination of tumor cells disclosed LCA (+), CD3(DAKO) (+), MT1 (+), UCHL1 (+), MB1 (+), MxPanB (-) and EMA (-) reactivity and showed T cell lymphoma. The clinical stage was determined to be IEA after further work-up. Improvement of swallowing difficulty and esophageal findings on upper gastrointestinal series were noted after modified CHOP therapy and radiotherapy (total 50 Gy).
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PMID:Primary esophageal non-Hodgkin's lymphoma. 163 71

We report a case of CD7+ stem cell lymphoma. A 47-year-old man presented with general malaise and lumbago in April 1997. The patient exhibited swollen left cervical lymph-nodes and an intra-abdominal bulky mass. He was referred to us because lymph-node biopsy specimens indicated a diagnosis of diffuse type malignant lymphoma. An abdominal CT scan disclosed large retroperitoneal, para-aortic, and mesenteric root masses. Bone marrow involvement was shown by bone marrow biopsy specimens, though no circulating blasts were detected at presentation. The patient was treated with high-dose CHOP therapy without any benefit. Though ESHAP therapy was performed as salvage chemotherapy, the abdominal masses did not shrink at all. The patient died of tumor progression in November 1997. In the terminal stage, the lymphoma cells emerged in the peripheral blood and thus became available for analysis. The cells expressed CD5, 7, 34, 38, 71, but were negative for CD1, 2, 3, 4, 8, 10, 13, 14, 16, 19, 20, 21, 25, HLA-DR, and EMA. An immunoglobulin heavy chain gene rearrangement band was detected by Southern blot analysis. However, no T cell receptor lambda or beta chain gene rearrangement bands were detected.
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PMID:[Chemotherapy-resistant CD7-positive stem cell lymphoma presenting with intra-abdominal mass]. 1002 52

A 52-year-old previously healthy Caucasian woman presented with superior vena cava syndrome, secondary to compression of a bulky anterior mediastinal mass involving the right lung. Fine-needle aspiration biopsy of the mediastinum yielded large epithelioid cells intermingled with small mature lymphocytes. The epithelioid cells are LCA positive, expressing cytoplasmic CD3 diffusely and TIA-1 focally, but negative for EMA, CD4, CD8, CD15, CD20, CD30, and CD56. The TIA-1+ cytoplasmic granules correlated to the azurophilic granules in Diff-Quik-stained cells, pink granules in Ultrafast Papanicolaou-stained cells, and dense core granules in electron microscopy. In situ hybridization for Epstein-Barr viral RNA was negative. The background small lymphocytes were composed of a majority of CD4+ T-lymphocytes and minority of CD8+ T-lymphocytes. The patient responded well to six cycles of CHOP chemotherapy, followed by radiation with a total dose of 4140 cGy delivered to the mediastinum in 23 fractions. On the chest X-ray taken 6 mo later, there was minimal apical fibrosis with no evidence of an acute intrathoracic pathology. To the best of our knowledge, this case may be the first report of cytotoxic large T-cell lymphoma of the mediastinum.
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PMID:TIA-1+ cytotoxic large T-cell lymphoma of the mediastinum: case report. 1189 19

Plasma cell leukemia (PCL) is a very rare variant of multiple myeloma (MM) occurring in about 2% of newly diagnosed patients. Plasma cell leukemia may develop during the course of MM (secondary PCL) or it can occur without any prior sign of MM (primary PCL). We report a case of aggressive primary PCL with unusual clinical, cytogenetic and molecular features. A 36-year-old male patient was first seen because of fever and bone pain. On the skin of his chest, back, abdomen, and palpebras, there were nodular infiltrations resembling urticaria. White blood cell count was 10.8 x 10(9)/l with 41% plasmacytes. Bone marrow aspiration was hypercellular, 93.5% of cells were atypical plasmacytes and plasmablasts. The cytogenetic analysis of G-banded chromosomes in bone marrow cells yielded the trisomy 8. The skin biopsy specimen showed intensive infiltrates of uninucleated blastic cells similar to those found in the bone marrow. Immunophenotyping of bone marrow and skin neoplastic cells showed CD45+, CD45Ro+, CD68+, CD38+ and cytoplasmic kappa light chain +. The neoplastic cells stained negatively for lambda light chain, CD3, CD20, CD30, EMA, CD15, CD34, CD56 and factor VIII. The pattern of IgL genes rearrangement in the bone marrow aspirate, peripheral blood mononuclear cells, and skin specimens was examined by PCR analysis. All studied specimens showed three different IgK gene configurations suggesting that the neoplastic cells originated as a result of oligoclonal lymphoproliferation process. The patient received two courses of VAD (vincristine, doxorubicin, dexamethasone) without improvement and three courses of CHOP with only temporary stabilization of the disease. He died 5 months after the diagnosis of PCL because of disease progression and pneumonia.
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PMID:Aggressive primary plasma cell leukemia with skin manifestations, trisomy 8 and molecular oligoclonal features. 1214 88

Non-Hodgkin's lymphoma is known to involve the bladder, either primarily or secondary. Anaplastic large cell lymphoma has not been documented at this site before. We report a case of a systemic anaplastic cell lymphoma in a 22-year-old man who presented with multisystemic symptoms and signs, including those suggestive of bladder disease. He was HIV-negative. Biopsy of the bladder showed a high grade lymphoma composed of large cells with nuclei containing prominent nucleoli. The tumor cells expressed CD30, CD45, EMA and ALK-1. A diagnosis of anaplastic large cell lymphoma, of null cell phenotype was made. The lymph node biopsy showed similar features on hematoxylin and eosin section. The patient was put on a regime of CHOP and is disease-free 33 months after diagnosis.
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PMID:Systemic anaplastic large cell lymphoma presenting as a bladder neoplasm. 1516 Sep 69

Anaplastic lymphoma kinase (ALK)-positive diffuse large B-cell lymphoma (DLBCL) is a rare, recently defined tumor distinct in many aspects from ALK-positive anaplastic large cell lymphoma (ALCL). We present two additional cases of ALK+DLBCL recently diagnosed in our department and a review of literature. A 48-year old man presented with a large upper neck mass growing slowly over 18 months. Histologically the tumor was diagnosed as an ALK-positive diffuse large B-cell lymphoma. with plasmablastic features. Large, frequently intrasinusoidal tumor cells expressed CD138, EMA, weakly IgA and kappa, but were negative for other B-cell markers, T-cell markers and CD30. The ALK staining was cytoplasmic with the increased intensity in the Golgi area. At the diagnosis the patient manifested with the stage IIIB. Three courses of CHOP resulted in partial and only transient remission. The patient died of massive bleeding from his decomposing tumor 3 months after the diagnosis. A 49-year old man complaining of abdominal pain revealed abdominal lymphadenomegaly and a gastric infiltrate, involving the deep portions of the gastric wall. The tumor showed immunoblastic/anaplastic morphology, with some Reed-Sternberg-like cells positive for ALK. ALK immunostaining was cytoplasmic, weak in a routine immunostain, enhanced with double (proteinase + pressure cooker) antigen retrieval. FISH was consistent with the t(2;5)/nucleophosmin(NPM)-ALK rearrangement. The tumor demonstrated similar "null" B/T phenotype with positivity for IgA, lambda, EMA and LCA. The patient (stage IVB) currently undergoes chemotherapy. ALK-positive DLBCL affects mostly middle-aged men, shows generally poor but stage-dependent prognosis (at least 60% mortality rate), presents typically as a lymph node-based disseminated disease, and very rarely involves the bone marrow. Genetic studies showed that the majority of ALK+DLBCL cases are characterized by the clathrin (CLTC)-ALK fusion and in a few cases the NPM-ALK rearrangement has been found.
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PMID:ALK-positive diffuse large B-cell lymphoma: two more cases and a brief literature review. 1592 Oct 12

We report detailed clinical and pathologic features of four cases of anaplastic lymphoma kinase-positive diffuse large B-cell lymphoma (ALK-DLBCL), a rare entity with only 29 currently reported cases. This study is the third largest of all reported series. Biopsies from four adult patients aged 41, 49, 53, and 71 years (three lymph nodes and one nasopharyngeal mass) exhibited immunoblastic/plasmablastic morphology. By immunohistochemistry and/or flow cytometry, they expressed cytoplasmic ALK-1, CD138, VS38 (3/3), monoclonal cytoplasmic light chain, CD45, EMA, CD4, and CD57 (2/3), and were negative for CD3, CD30, CD56, and TIA-1. Two showed variable CD79a expression, and one had rare CD20(+) cells. Two of three cases exhibited rare CD43(+) reactivity. One case showed scattered cytokeratin(+) cells, which could possibly lead to a misdiagnosis of carcinoma. After CHOP and radiotherapy, two stage I patients were free of disease at 58 and 36 months, whereas a stage IV patient was dead of disease at 22 months.
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PMID:ALK-positive diffuse large B-cell lymphoma: report of four cases and review of the literature. 1727 65

A 67-year-old man suffered from a left cervical lymph node swelling and tenderness. Biopsy of the cervical lymph node showed pleomorphic large cells containing large atypical nuclei. Immunohistochemical stains of these cells were positive for CD30, but negative for CD3 and CD20. After the biopsy, his left cervical skin was ulcerated. Biopsy of the left cervical skin was performed. Large pleomorphic cells with constricted nuclei and Reed-Sternberg-like cells existed in the dermis and epidermis. Immunohistochemical stains of the former cells were positive for CD30, CD45 and PAX5, but negative for CD3, CD10, CD20, CD15, Bcl-2, EBER ISH, EMA and ALK. He was diagnosed with diffuse large B cell lymphoma, anaplastic variant. He achieved complete remission with CHOP chemotherapy. CD30-positive DLBCL, anaplastic variant is a rare B cell lymphoma. Most of the patients presented with primary nodal disease, and skin involvement of lymphoma is very rare. This is the report of a rare case of CD30-positive DLBCL, anaplastic variant, with both nodal and skin lesions.
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PMID:CD30-positive anaplastic variant diffuse large B cell lymphoma: a rare case presented with cutaneous involvement. 2083 60

Plasmablastic lymphoma (PBL) has initially been described as a rapidly progressive and almost invariably fatal diffuse large-cell lymphoma with plasmablastic features, exclusively involving the jaw and oral mucosa in HIV-positive patients. Although its clinical features may help in differential diagnosis, an extra-oral localization in a patient without HIV makes it more difficult to suspect clinically. We describe a very rare case of gastric PBL primarily involving stomach in a middle age man without an HIV infection. A biopsy was performed and its findings revealed a diffuse, monomorphous proliferation of the tumor cells with features of immunoblasts, MUM-1, EMA, and lambda light chains positive. Serology was negative for the human immunodeficiency virus (HIV), HBsAg, and hepatitis C virus infection. The patient started treatment with CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone) chemotherapy, but unfortunately died before the second cycle was given. To our knowledge, this is the second case of gastric PBL presented in HIV-negative patients. The findings in this case suggest that PBL should be included in the differential diagnosis of gastrointestinal tumors.
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PMID:Unusual presentation of gastric plasmablastic lymphoma in HIV-negative patient. 2147 44

We report the case of a 62-year-old white male who presented with a 2.6-cm ulcerating mass on the skin of the left buttock and ipsilateral inguinal lymphadenopathy. Microscopic sections of the skin lesion showed a nodular and plaque-like growth pattern of a mixed cellular infiltrate throughout the dermis and subcutaneous tissue with prominent myxoid change. There was a dominant population of medium-sized mitotically active atypical cells that expressed CD30, CD4 and EMA. These atypical cells were mixed with eosinophils, neutrophils, mature lymphocytes and histiocytes. Tissue from the inguinal lymphadenopathy showed similar pathologic features, although no residual lymph node tissue was present. A diagnosis of secondary anaplastic large cell lymphoma, myxoid variant, with skin and lymph node/perinodal soft tissue involvement was rendered at the time of complete excision of the buttock mass. The patient received five cycles of CHOP (cyclophosphamide, doxorubicin, vincristine and prednisone) chemotherapy with complete resolution of lymphadenopathy and no residual cutaneous disease. He was disease-free by PET/CT scan and physical examination at 16 months after chemotherapy. We present this case to highlight the histopathologic and immunophenotypic features of this entity with a discussion of the differential diagnosis and a review of the literature.
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PMID:Myxoid variant of anaplastic large cell lymphoma involving the skin: a case report. 2261 58


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