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)

Intravascular lymphomatosis (IL) is a rare entity only recently included in lymphoma classification, whose main feature is the exclusive or predominant growth of neoplastic cells within blood vessels. The vast majority of the patients affected by IL belong to the 7th or 8th decade of life and present with skin rash or CNS diffuse necrotic or demyelinating lesions. Case report. SS, a 13-year-old girl, was admitted to a Neurosurgery Unit because of endocranic hypertension, where, after CT and MRI documenting a IV ventricle 3 cm diameter tumor, she was submitted to complete tumor excision: extemporary diagnosis was suggestive of medulloblastoma. When referred to us she had persistent fever with normal blood and spinal fluid cultures. Whole CNS MRI did not give evidence of residual or metastatic disease while CSF cytology showed only pleiocytosis. Treatment was started according to our ongoing protocol for medulloblastoma with pre-radiation chemotherapy. Before delivering radiotherapy (RT), upon review of histologic specimens, the definitive diagnosis of IL B-phenotype was made. The girl was re-admitted and, after a complete re-staging, chemotherapy was intensified according to our schedule for high-grade B-cell lymphoma and CNS was irradiated up to a total dose of 25 Gy. She remained alive in continuous complete remission at 21 months after diagnosis. The case here reported is unique for age, tumor presentation, and, so far, favourable outcome, in spite of the delayed histological diagnosis.
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PMID:Intravascular lymphomatosis (IL) in a child mimicking a posterior fossa tumor. 1134 80

A novel cell line, designated OHK, was established from ascites of a 59-year-old Japanese woman with diffuse large B-cell lymphoma showing a peculiar serosal tropism, as seen in primary effusion lymphomas (PEL). OHK exhibited a large pleomorphic morphology with irregular nuclei and distinct nucleoli, and included immunoblastic and Reed-Sternberg-like giant cells. On ultrastructural examination, rich intermediate filaments, and well-developed Golgi apparati and rough endoplasmic reticulum, were seen. Immunophenotypically, OHK lacked T and B cell-associated antigens, and had CD10, CD30, CD33 and CD138 antigens. Although OHK cells did not express immunoglobulin (Ig) protein, Southern blot analysis demonstrated clonal rearrangements of Ig heavy and light chain genes. These observations suggest that OHK cells are derived from preterminally differentiated B cells, and that they have features of PEL. Kaposi's sarcoma-associated herpesvirus and Epstein-Barr virus were not detected. OHK displayed hyperploid karyotypes with multiple structural abnormalities, and produced some cytokines such as macrophage-colony-stimulating factor (M-CSF), granulocyte-CSF, interleukin 6 and transforming growth factor beta 1. In particular, vascular endothelial growth factor (VEGF), whose stimulation of vascular permeability is thought to be critical to the pathogenesis of PEL, was also produced in large quantities. These results indicate that OHK may be a useful tool for the investigation of PEL.
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PMID:Establishment and characterization of a Kaposi's sarcoma-associated herpesvirus- and Epstein-Barr virus-negative malignant lymphoma cell line (OHK) with primary effusion lymphoma immunophenotype. 1184 5

Serum levels of hepatocyte growth factor (HGF), a potent angiogenic factor, increase during various haematological malignancies. In this study, we examined serum HGF in 59 patients with non-Hodgkin's lymphoma (NHL). Serum HGF levels in NHL patients were increased, as were levels in patients with multiple myeloma, chronic myeloproliferative disorders, and myelodysplastic syndrome. Some 29 patients with T-cell lymphoma, including 20 with adult T-cell leukemia/lymphoma, exhibited a significant increase in serum HGF, as did 23 with B-cell lymphoma. The levels of serum HGF correlated with increased neutrophil counts (r=0.487, p<0.0001), and also paralleled a neutrophil increase in NHL patients who received granulocyte-colony stimulating factor (G-CSF) at the nadir of neutrophil count following chemotherapy. Additionally, in in vitro experiments, HGF secretion from polymorphonuclear neutrophils and its expression in bone marrow myeloid cells were stimulated by G-CSF. Although HGF has been thought to be involved in the pathogenesis of NHL through its angiogenic activities, these results suggest that HGF production by neutrophils and myeloid lineage cells may also contribute to an increase in serum HGF in NHL patients.
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PMID:Possible involvement of neutrophils in a serum level increase of hepatocyte growth factor in non-Hodgkin's lymphoma. 1570 13

An important component in the diagnosis of atypical parkinsonian disorders is the exclusion of secondary causes. Paraneoplastic causes of parkinsonism are extremely rare. We describe a case which presented initially as probable progressive supranuclear palsy (PSP) but on follow-up displayed a rapidly progressive course, unexplained fever, peripheral neuropathy and an abnormal CSF. We highlight the difficulties faced in formulating a diagnosis for this unusual case prior to the discovery of an occult B-cell lymphoma, and discuss its relevance in the exclusion criteria for PSP. A paraneoplastic cause should be considered if disease progression is unusually rapid.
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PMID:Paraneoplastic progressive supranuclear palsy syndrome in a patient with B-cell lymphoma. 1582 84

A 69 year-old man developed sudden-onset multidirectional, constant, involuntary ocular movements associated with vertigo, truncal ataxia and involuntary movements of the lower limbs. These features were typical of opsoclonus-myoclonus-ataxia syndrome (OMS). MRI of the brain was normal. CSF studies showed a single oligoclonal IgG band. A chest x-ray showed a 2-centimeter lesion in the periphery of the left lung. Fine needle aspiration biopsy of this lesion revealed large B-cell lymphoma. OMS can be either idiopathic or a paraneoplastic manifestation of underlying malignancy. 20 of OMS cases are paraneoplastic in origin; breast and lung cancer are responsible for 70 of these. Association of this syndrome with non-Hodgkins lymphoma is rare, with only one case previously reported.
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PMID:Paraneoplastic Opsoclonus-Myoclonus Syndrome: initial presentation of non-Hodgkins lymphoma. 1593 16

The purpose of this article is to review the current data on the risk of CNS relapse in patients with lymphoma and the efficacy of CNS directed prophylactic therapy. CNS relapse occurred in 30-50% of those with Burkitt lymphoma and acute lymphoblastic leukaemia/lymphoma prior to the introduction of intensified regimens that include CNS prophylaxis. Most patients with AIDS-related-lymphoma receive a short course of intrathecal prophylaxis but a re-evaluation of type and targeting of CNS prophylaxis is needed. Patients with diffuse large B-cell lymphoma (DLBCL) have a 5% overall risk of CNS relapse but a high risk sub-population can be identified on the basis of raised LDH and >1 extranodal site, testicular or primary breast involvement. CNS prophylaxis for selected patients with DLBCL may be justified by risk but its benefit is not yet proven. Intravenous methotrexate > or = 3 g/m(2) achieves therapeutic levels in CSF and parenchyma and in combination with intrathecal methotrexate would be a reasonable option for prophylaxis.
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PMID:CNS prophylaxis in lymphoma: who to target and what therapy to use. 1688 38

Primary central nervous system lymphoma (PCNSL) is an extranodal non-Hodgkin lymphoma arising from and confined to the neuraxis. It represents about 3-4% of all primitive brain neoplasms and is mainly located in deep supratentorial regions. A ventricular involvement is quite frequent, but in the vast majority of cases it is secondary to an extension from a subependymal location. Amongst the primary ventricular forms occasionally reported, a choroid plexus origin is extremely rare; to date, only three cases have been described in the English literature. We report the case of a 71-year-old left-handed woman admitted to our department after a progressive 2-week history of confusion, motor speech disturbances and left hemiparesis. A brain MRI scan displayed an intraventricular lesion located in the right atrium, about 2.5cm in its major axis, homogeneously enhancing after gadolinium administration, surrounded by edema in the homolateral deep hemispheric region; the main diagnostic hypothesis was meningioma. She underwent a parieto-occipital craniotomy and a navigation-assisted posterior parietal transcortical approach, achieving complete removal of the intraventricular lesion originating from the choroid plexus. The histological diagnosis was of high-grade diffuse large B-cell lymphoma. Both an MRI scan of the entire craniospinal axis and a thoracic and abdominal CT scan showed no other neoplastic lesions; a bone marrow biopsy was normal; a final diagnosis of primary central nervous system lymphoma was made. CSF cytological analysis did not show any signs of lymphoma dissemination. Adjuvant chemotherapy with methotrexate was administered, and at the last follow-up (26 months) our patient is clinically and radiologically disease-free. Primary choroid plexus lymphoma is a very rare tumor that should be included in the differential diagnosis of intraventricular neoplasms. It usually occurs in the lateral ventricles (with a predilection for the atrium) of adult people (>50 years of age), apparently with a male prevalence. Generally, the radiological picture mimics that of a meningioma, despite the fact that meningiomas usually reach a greater volume before clinical onset. Surgery is almost always necessary to obtain an adequate tissue sample and frequently leads to a radical removal of the lesion. Low-grade marginal zone B-cell, T-cell and high-grade diffuse large B-cell (present case) forms have been described. With a proper adjuvant therapy a relatively good outcome is observed.
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PMID:Primary high-grade B-cell lymphoma of the choroid plexus. 1792 35

Lymphoma involving the pituitary gland is very rare and usually results from metastatic spread of systemic lymphoma. We present a case of primary central nervous system (CNS) large B cell lymphoma that manifested as pituitary apoplexy. A 45-year-old woman presented with headache, and then rapidly developed a third nerve palsy and bitemporal hemianopsia. Imaging suggested a pituitary macroadenoma, with spontaneous necrosis, extending into the suprasellar region, compressing the optic chiasm and invading the right cavernous sinus. The patient underwent transsphenoidal resection which revealed a vascular, firm tumor. An aggressive decompression of the optic chiasm was performed with complete resolution of both visual fields and third nerve palsy. Final pathology showed B cell lymphoma. Systemic work-up including bone marrow aspiration and CSF studies showed no other foci of lymphoma, and the patient was HIV-negative. Chemotherapy with methotrexate, vincristine, procarbazine, and dexamethasone was administered for primary CNS lymphoma. This is an uncommon diagnosis of which the clinician should be aware in order to tailor surgical intervention and provide early institution of proper therapy.
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PMID:Primary central nervous system lymphoma mimicking pituitary apoplexy: case report. 1820 50

Symptomatic nervous system leukemic infiltration is rarely observed in CLL. Various clinical manifestations including headache, confusion, cranial nerve palsies, focal central deficits and peripheral neuropathies have been seldom reported, occurring in less than 1% of patients. We report herein 2 CLL patients with unusual clinical presentations of nervous system invasion. They presented multiple progressive peripheral deficits due to meningoradiculitis. In both, CSF immunophenotyping analysis identified a majority of T cells (>90%), and less than 10% of B-CLL cells expressing CD5, CD19 and CD20. Our analyses revealed the transformation of CLL into an aggressive B-cell lymphoma in one case (Richter's syndrome). A post mortem study showed massive infiltration of cranial nerves and spinal roots by large B lymphomatous cells. In the other case, CNS oriented chemotherapy led to remission and total neurological recovery. In practice, the etiological diagnosis of neurological deficits in CLL patients is difficult. CSF analysis may be useful, requiring viral PCR, repeated cytological studies and immunophenotyping analysis. Although rare, leptomeningeal leukemic localization has to be discussed, even in the absence of overt Richter syndrome, and may require an early therapeutic test.
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PMID:Multifocal deficits due to leukemic meningoradiculitis in chronic lymphocytic leukemia. 1910 Sep 98

This study describes the development of an human granulocyte-macrophage colony-stimulating factor DNA cationic-lipid complexed autologous tumour cell vaccine (hGM-CSF CLDC ATCV) and its implementation, following a chemotherapy treatment protocol, in a randomized, placebo-controlled, double-blinded clinical trial in pet dogs with naturally occurring lymphoma. We hypothesized that the use of this vaccine would result in an antitumour immune response leading to improved first remission duration and overall survival in dogs with B-cell lymphoma when compared with chemotherapy alone. Immune stimulation generated by hGM-CSF CLDC ATCV was assessed by means of surrogate in vivo analysis (delayed-type hypersensitivity [DTH]) as well as an ex vivo cellular assay (lymphocyte proliferation assay). The vaccine approach considered in the current report did not result in clinically improved outcomes. A small measure of immunomodulation was documented by DTH and several modifications to the approach are suggested. This report illustrates the feasibility of clinical trials with vaccine strategies using companion animals with non-Hodgkin's lymphoma.
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PMID:Human granulocyte-macrophage colony-stimulating factor DNA cationic-lipid complexed autologous tumour cell vaccination in the treatment of canine B-cell multicentric lymphoma. 1975 80


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