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)

Hepatic amyloid deposition in the form of globular inclusions is a rare occurrence. Only 24 such cases have been reported to date. Its clinical and pathological significance are undefined. Unawareness of such a pattern can cause diagnostic confusion. We herein describe a case of globular hepatic amyloid in a patient with a B-cell lymphoma and chronic hepatitis C. The findings in our case support the literature data in that (1) it is often an incidental finding during workup for other coexisting conditions and (2) its morphology is peculiar but can be recognized with ease if one is aware of its existence. Our case also provides new insights into this condition. First, it represents the first nonautopsy case to demonstrate that globular hepatic amyloid is an indication of systemic amyloidosis, thus emphasizing the clinical importance of the recognition of this condition. Second, in our case, there was a coexisting B-cell lymphoma, the constituent cells of which showed immunoglobulin lambda light chain restriction, and patient's serum lambda light chain was elevated. However, light chain restriction was not demonstrated in the globular inclusions, and there was no evidence of monoclonal gammopathy by serum electrophoresis. Whether immunoglobulin light-chain abnormality played a causal role in this condition is to be determined.
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PMID:Globular hepatic amyloid: a diagnostic peculiarity that bears clinical significance. 1608 57

Lymphomas, both within and outside the central nervous system, are uncommon among patients with systemic lupus erythematosus (SLE). We describe a 58-year old Korean woman with SLE who presented with acute headache and confusion in the setting of prednisone and mycophenolate mofetil (MMF) therapy used to treat focal proliferative and membranous lupus nephritis. Three-dimensional brain magnetic resonance imaging (MRI) showed two peripherally ('ring') enhancing lesions within the basal ganglia, bilaterally, with associated mass effect and subfalcine herniation. A brain biopsy revealed an Epstein-Barr virus (EBV)-positive diffuse large B cell lymphoma. This is the first description of CNS lymphoma in a patient treated with MMF for lupus nephritis. While intracerebral lymphoma in the immunocompromised patient with lupus is rare, this disorder should be considered in the differential diagnosis of new-onset neurological symptoms among such patients.
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PMID:Central nervous system lymphoma associated with mycophenolate mofetil in lupus nephritis. 1633 85

'Lymphomatosis cerebri' (LC) is a term indicating a diffusely infiltrating form of primary central nervous system lymphoma (PCNSL) without evidence of a mass lesion. Not infrequently, diagnostic confusion is caused by its presentation on cranial magnetic resonance images (MRI), which is characterized by diffuse leukoencephalopathy without contrast enhancement. In this report, we describe a 53-year-old, immunocompetent man who had an insidiously progressive dementia and right weakness. On serial MRI in 4 months duration, diffuse white matter lesions without contrast enhancement gradually progressed, which was clinically consistent with his worsening condition. Biopsy specimen demonstrated nondestructive, diffusely infiltrating, large B-cell lymphoma, diagnosing LC. After the biopsy, corticosteroids were initiated, which dramatically alleviated his symptoms. Afterwards, he was treated by whole brain irradiation (total 36Gy) and discharged without noticeable deficits. Diagnosis of LC requires additional examinations generally not performed in the other white matter disorders. In suspected cases, biopsy should be performed to avoid deferring adequate cytostatic treatment.
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PMID:A case of 'lymphomatosis cerebri' diagnosed in an early phase and treated by whole brain radiation: case report and literature review. 1761 16

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

The autoimmune lymphoproliferative syndrome (ALPS) and ALPS-like syndrome are variable clinical conditions characterized by lymphoproliferative disease, autoimmune cytopenias and susceptibility to malignancy. A 59-year-old woman was admitted to the hospital for intractable generalized pain and stiffness with multiple swollen joints for 2 weeks. A low-grade fever, intermittent hypotension and confusion were associated with the pain. The evaluation revealed multiple joint bony erosions with effusion and a ruptured Baker's cyst and positive AFB testing on the joint biopsy of the right wrist. In addition, there were a macular skin rash with telangiectasia and perivascular lymphocyte infiltration, a cytopenia without abnormal cells, a hepatosplenomegaly, a pericardial thickness with effusion and pleural effusion. The patient was treated with anti-mycobacterial drugs, NSAIDs and glucocorticoids for 10 months. But with the symptoms worsening, the patient developed cervical lymph node enlargements and was diagnosed as a diffuse large B cell lymphoma with hemophagocytosis on biopsy.
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PMID:Autoimmune lymphoproliferative syndrome-like syndrome presented as lupus-like syndrome with mycobacterial joint infection evolved into the lymphoma. 1882 Sep 32

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

Primary central nervous system lymphoma (PCNSL) is a rare intracranial tumor, with an annual incidence of six per million population. Anaplastic variant of primary CNS diffuse large B-cell lymphoma is less common; to our knowledge, there is only one other case report in the world literature. We describe a 71 year old immunocompetent female without significant past medical history who presented with confusion and a homogeneously enhancing midline mass. The patient underwent craniotomy for tumor biopsy, followed by high-dose methotrexate-based chemotherapy despite a remarkably low performance status. Histologically, this tumor was composed of undifferentiated polymorphic tumor cells, multi-nucleated giant cells, extensive necrosis, and conspicuous mitotic activity, mimicking undifferentiated metastatic tumors. Immunohistochemical stains demonstrated immunopositivity of tumor cells for CD20, MUM-1, and BCL-6, and negative staining for CD3, CD10, and CD30. The clinical course, diagnostic workup, pathologic correlates, and treatment outcomes are described.
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PMID:Primary CNS anaplastic diffuse large B-cell lymphoma mimicking undifferentiated metastatic tumors: a case report. 1961 13

A significant amount of literature is available on treatment monitoring and response assessment in lymphoma using FDG-PET, yet confusion exists concerning the potential and limitations of FDG-PET for determining the presence of residual disease during chemotherapy (interim FDG-PET). This article reviews the role of interim FDG-PET in 3 important scenarios: untreated diffuse large B-cell lymphoma, untreated Hodgkin lymphoma, and relapsed or refractory aggressive lymphoma in transplant-eligible patients, and provides recommendations on the use of this imaging modality in these settings.
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PMID:An update on the role of interim restaging FDG-PET in patients with diffuse large B-cell lymphoma and Hodgkin lymphoma. 2020 64

Childhood head and neck cancers are relatively uncommon. Of all head and neck cancers occurring in children, non-Hodgkin's lymphoma (NHL) is the most common, others being rhabdomyosarcoma and nasopharyngeal carcinoma. In the head and neck region, sinuses are the second commonest primary site of NHL after neck lymph nodes. These can be of several different types depending on the predominant cell type and histologic appearance, the most common histological variant being diffuse large B-cell lymphoma. In an attempt to simplify the classification and to develop a universally acceptable classification and staging, they have been classified and staged numerous times over the last three decades, adding more confusion to the topic. Clinical presentations vary according to the histological type. The low grade lymphomas present with a nasal cavity or para-nasal sinus mass associated with obstructive symptoms and/or lymphadenopathy, while high grade lymphomas present with aggressive signs and symptoms including non-healing ulcer, epistaxis, septal perforation and bony destruction. The primary treatment consists of chemotherapy and / or radiation therapy, which is able to achieve remission in two-third of the patients, however, prognosis remains poor with cumulative five-year survival rates at about 30% for all the types of sino-nasal NHLs. Newer targeted therapy (monoclonal antibodies) and combination therapies (including stem cells) are currently being tested in order to improve survival rates in these patients. This article aims at providing an overview of clinico-epidemiologic characteristics, staging system currently in use, management, prognosis and possibilities of future research in the field of childhood sinonasal NHLs.
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PMID:Non-Hodgkin's lymphoma of the sino-nasal tract in children. 2047 39

Lymphomas that develop in human immunodeficiency virus (HIV) infected patients are predominantly aggressive B-cells lymphomas. The most common HIV-associated lymphomas include Burkitt lymphoma, diffuse large B-cell lymphoma (that often involves the CNS), primary effusion lymphoma, and plasmablastic lymphoma (PBL). Of these, PBL is relatively uncommon and displays a distinct affinity for presentation in the oral cavity. In this manuscript we report a previously undescribed primary leptomeningeal form of PBL in a patient with acquired immunodeficiency syndrome. A 40-year-old HIV positive man presented with acute onset confusion, emesis, and altered mental status. Lumbar puncture showed numerous nucleated cells with atypical plasmocyte predominance. CSF flowcytometry showed kappa restriction with CD8 and CD38 positivity and negative lymphocyte markers, while the MRI showed diffuse leptomeningeal enhancement. As the extensive systemic work-up failed to reveal any disease outside the brain, an en bloc diagnostic brain and meningeal biopsy was performed. The biopsy specimen showed sheets of plasmacytoid cells with one or more large nuclei, prominent nuclear chromatin, scattered mitoses, and abundant cytoplasm, highly suggestive of plasmablastic lymphoma. HIV-associated malignancies have protean and often confusing presentations, which pose diagnostic difficulties posed to the practicing neurological-surgeons. Even in cases where an infectious cause is suspected for the meningeal enhancement, neoplastic involvement should be considered, and cytology and flow-cytometry should be routinely ordered on the CSF samples.
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PMID:Primary leptomeningeal plasmablastic lymphoma. 2135 53


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