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 patient with IgM-secreting B-cell lymphoma developed demyelinating peripheral neuropathy, nodular regenerative hyperplasia of the liver, coagulopathy, and terminal miliary lymphomatous spreading. Serologic auto-antibody activities directed against myelin associated glycoprotein, prothrombinase complex (i.e. lupus anticoagulant) and smooth muscle cells were detected. It is suggested that there is a link between the IgM gammopathy, the immunological abnormalities and the various clinico-pathological conditions.
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PMID:Peripheral neuropathy, coagulopathy and nodular regenerative hyperplasia of the liver in a patient with multiple serologic auto-antibody activities and IgM B-cell lymphoma. 255 47

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

Epstein-Barr virus associated T-Cell lymphoma mimicking malignant histiocytosis (MH) has been previously reported. We selected 10 autopsy cases of extranodal lymphoma or histiocytic tumor, which showed an EBV presence in the tumor cells as well as a fulminant clinical course. The detailed clinicopathologic features were thus clarified. A retrospective study was performed on ten adult patients, eight males and two females, and almost all cases presented with a fulminant clinical course, revealing pancytopenia, liver dysfunction and disseminated intravascular coagulopathy. Immunophenotypic and genotypic studies along with in situ hybridization (ISH) were performed. The autopsy findings mainly showed extra nodal involvement in the liver (10 patients), spleen (9 patients), intestinal tract (5 patients), bone marrow (5 patients), nasal cavity, lungs, adrenal glands, kidneys (2 patients) and brain. Histologically atypical pleomorphic lymphoid cells were observed to infiltrate with reactive histiocytes, some of which showed hemophagocytosis. Based on the histological and clinical findings, diagnosis of malignant histiocytosis was made. ISH showed an EBV-presence in almost all the tumor cells. The immunophenotype and/or genotype studies demonstrated T-cell lymphoma (2 patients), Histiocytic tumors (2 patients), B-cell lymphoma (1 patients), natural killer (NK) cell lymphoma (3 patients), and T/NK lymphoma (2 patients), in which T or NK could not be confirmed, due to a lack of fresh materials. Based on the above findings, the histological appearance of EBV-associated MH previously defined was shown to be common to extra-nodal malignant lymphomas having origin in various organs, although the cytological and genetic features were heterogenous.
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PMID:Heterogeneity of systemic extra-nodal Epstein-Barr virus-associated lympho-histiocytic tumor--ten autopsy cases of human immunodeficiency virus-negative Japanese. 925 51

Malignant histiocytosis-like B-cell lymphoma (MH-like BCL) is characterized clinically by hemophagocytic syndrome (HPS), bone marrow involvement at presentation, and an aggressive clinical course. Yet, it remains an ill-defined disease entity. We recently described five cases of MH-like BCL and suggested that these may be regarded as a peculiar variant of intravascular lymphomatosis (IVL), based on clinical and pathological observation. Interestingly, this type of lymphoma has been reported exclusively among Asians, with few reports from Western countries, and therefore we propose that this variant be noted as an Asian variant of IVL(AIVL). To evaluate the incidence of this variant, we performed a literature search for English or Japanese studies dealing with MH-like BCL or B-cell IVL associated with HPS (IVL with HPS). We found 18 reports of MH-like BCL and 16 reports of IVL with HPS and re-evaluated the clinicopathologic aspects of each study with respect to AIVL. Although several differences were noted, such as gender, presence of respiratory changes, bone marrow invasion and disseminated intravascular coagulopathy, there were also overlapping profiles and they appeared to be consistent with the AIVL single disease entity. Of particular note was the absence of peripheral lymphadenopathy and tumor formation which posed diagnostic problems in the initial phase of the presentations. Thus, based on analysis of the clinical findings of these 34 cases, the diagnostic criteria for AIVL consists primarily of cytopenia (anemia and/or thrombocytopenia), hepatosplenomegaly, absence of peripheral lymphadenopathy and tumor formation, and erythrocyte-hemophagocytosis along with intravascular proliferation of the neoplastic B cells. The pathologic findings of the 19 autopsied cases which fulfilled the criteria are also summarized, and they reveal morphologic, immunologic, and genotypic features of typical IVL.
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PMID:An Asian variant of intravascular lymphomatosis: an updated review of malignant histiocytosis-like B-cell lymphoma. 1034 74

Lymphomatoid granulomatosis (LYG)/angiocentric immunoproliferative lesions (AIL) consist of angiocentric and angiodestructive lymphoreticular proliferation predominantly involving the lungs and other extranodal sites, such as the central nervous system (CNS). This clinical entity is considered as a B cell process related to Epstein-Barr virus (EBV) infection and EBV positive diffuse large B-cell lymphoma. The CNS is involved in 20% of cases of LYG, but initial involvement is rare. In cases without pulmonary symptoms, diagnosis may be difficult. We report a rare case involving initial progression of CNS symptoms followed by a pulmonary abnormality.A 14-year-old girl suffered from high fever, ataxic gait and paraparesis. MRI revealed diffuse T2 high signals with multiple gadolinium enhancements in the cerebellum, brain stem and cerebral white matter. Her symptoms briefly improved after steroid therapy, but ataxia gradually progressed. Dyspnea due to pulmonary interstitial involvement appeared when she was 18 years old. Steroid therapy proved effective for respiratory symptoms. At 20 years old she suffered from disseminated intravascular coagulopathy (DIC) and hemophagocytic syndrome (HPS) with respiratory symptoms and repeated seizures. Her symptoms improved after the administration of cyclophosphamide. Mild hemiparesis and gait disturbance appeared when she was 22 years old. MRI revealed new lesions at the basal ganglia and subcortical white matter, brain atrophy and diffuse T2 high intensity of cerebral white matter. Cyclophosphamide was effective and there has been no recurrence of symptoms in the last 5 years. We reviewed the non-tumorous LYG/AIL involving the CNS, and discussed the clinical features, MRI imaging and diagnosis of the LYG/AIL.
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PMID:A case of lymphomatoid granulomatosis/angiocentric immunoproliferative lesion with long clinical course and diffuse brain involvement. 1287 57

A 31-yr-old man presented with a 1-wk history of fever, chills, weakness, headaches, and a significant 20-lb weight loss over the preceding 2 months. His past medical history was relevant for liver amebiasis during childhood. Two days before admission, the patient noticed jaundice. He denied abdominal pain or other GI symptoms, and there was no history of alcohol intake, medications, or illicit drugs. His physical examination revealed generalized jaundice, hepatosplenomegaly, and bilateral leg edema. Neurologically, the patient was agitated, with periods of disorientation, and he had bilateral flapping. His blood tests revealed pancytopenia, renal failure, liver failure, and coagulopathy. Because the patient had a fever, hepatosplenomegaly, and pancytopenia, a further workup also included a bone marrow and liver biopsy. No conclusive diagnosis could be made from the above tests, and the patient died 5 days after admission. Postmortem evaluation, including flow cytometry and gene rearrangement in the tissue obtained from the liver, revealed large B cell lymphoma. This case illustrates an unusual presentation of hepatic non-Hodgkin's lymphoma. Current information regarding this entity is scant, mainly owing to its rarity. We present a review of the literature, including the incidence, presentation, treatment, and prognosis of primary hepatic lymphoma.
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PMID:Primary hepatic non-Hodgkin's lymphomas: case report and review of the literature. 1468 34

Hepatic lymphoma metastasis is rare, and should always be considered in the differential diagnosis of hepatic malignancy. A 52-year-old man presented with a four-day history of fever, fatigue, yellowish skin and nausea. His past medical history was unremarkable. There was no history of alcohol intake or medications. His physical examination revealed generalized jaundice and hepatomegaly. His blood tests showed liver failure and coagulopathy. Abdominal ultrasonography illustrated hepatomegaly. A further work-up included bone marrow and liver biopsy. The pathology report was B-cell lymphoma. He was treated with chemotherapy, and his laboratory findings during follow-up showed steady improvement. In conclusion, lymphoma metastasis to liver can be a cause of liver dysfunction. A high index of suspicion is required for the diagnosis. We emphasize the importance of obtaining tissue sample in all patients with suspicious lesion in any organ to avoid missing the rare but curable pathologies.
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PMID:Hepatic lymphoma metastasis presenting with severe acute liver failure: a rare case. 1533 18

A 37-year-old man was referred for massive splenomegaly. In November 2005, he was diagnosed with non-Hodgkin's B-cell lymphoma in the setting of splenomegaly and thrombocytopenia. His laboratory results showed a coagulopathy owing to lupus anticoagulant. A computed tomography scan showed a 36 x 26 x 11 cm spleen and a prominent and sinuous splenic artery. The authors performed a laparoscopic splenectomy with an initial ligation of the splenic artery. The patient tolerated the procedure well and was discharged home on the fourth postoperative day in stable condition. Discussed in this paper is the safety and feasibility of the minimally invasive approach in massive splenomegaly.
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PMID:Laparoscopic splenectomy for massive splenomegaly: technical aspects of initial ligation of splenic artery and extraction without hand-assisted technique. 1850 72

We report a 59-year-old man with intravascular large B-cell lymphoma (IVLBCL) associated with hemophagocytic syndrome, disseminated intravascular coagulopathy, and lung involvement. G-banding analysis of the metaphase spreads obtained from the bone marrow showed that the lymphoma cells were near-tetraploid and included two homologues of the 14q+ chromosome. Spectral karyotyping revealed that complex translocations occurred among chromosomes 3, 12, 14, and 19, and additional materials of 14q+ were from chromosome 19 with the breakpoint at 14q32 and 19q13. To the best of our knowledge, this is the first report describing t(14;19)(q32;q13) in IVLBCL.
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PMID:Intravascular large B-cell lymphoma associated with t(14;19)(q32;q13) translocation. 2192 86

Severe disseminated varicella zoster virus (VZV) infection rarely occurs in patients who are not recipients of hematopoietic stem cell transplantation. This report concerns severe disseminated VZV infection in a diffuse large B cell lymphoma (DLBCL) patient treated with rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisolone (R-CHOP). The patient was an 82-year-old male with DLBCL who had a history of type II diabetes mellitus. He incurred VZV infection with severe hepatitis and disseminated intravascular coagulopathy after three courses of R-CHOP. When the VZV infection occurred, anti-VZV IgG was not detected and lymphopenia was observed. We initiated treatment with acyclovir, immunoglobulin, and thrombomodulin alpha, and rescued this patient. We suggest that the use of chemotherapy for immune-suppressed elderly lymphoma patients may involve the risk of severe VZV infection.
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PMID:Severe hepatitis associated with varicella zoster virus infection in a patient with diffuse large B cell lymphoma treated with rituximab-CHOP chemotherapy. 2290 49


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