Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0079731 (B-cell lymphoma)
 16,671 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Ataxia telangiectasia (A-T) is a rare autosomal recessive disorder characterized by cerebellar ataxia, oculocutaneous telangiectasia, and variable degrees of humoral and cellular immunodeficiency. Affected individuals are known to exhibit a high incidence of lymphoma and leukemia. Because of increased chemosensitivity, the treatment of A-T patients with malignancies requires extremely careful planning and caution with respect to the use of chemotherapy. The authors report on a 12-year-old boy with A-T who developed B-cell lymphoma. He received a half-dose of the drugs administered according to the acute lymphoblastic leukemia (ALL) protocol issued by our children's cancer study group (9104 Standard Risk Protocol, Tokai Pediatric Oncology Study Group). As a result, he continues to be in complete remission and free of treatment complications 32 months after the diagnosis of B-cell lymphoma.
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PMID:Ataxia telangiectasia associated with B-cell lymphoma: the effect of a half-dose of the drugs administered according to the acute lymphoblastic leukemia standard risk protocol. 978 9

We present an unusual case of diffuse large B-cell lymphoma within pontocerebellar angle schwannoma in 62-year-old woman. The patient suffered for 5 months with V, VII and VIII nerves paresis and with cerebellar ataxia. CT scan demonstrated large hyperdensive mass in cerebellopontine angle translocating cerebellar hemisphere and cerebral trunk. The patient was subjected to surgery and the tumour was removed totally by suboccipital retromastoidal right craniectomy approach. Histopathological examination revealed schwannoma infiltrated with high grade B-cell lymphoma. The patient did well following surgery without any other lymphoma manifestations, and she died from a heart attack 20 months later. Solitary lymphoma of pontocerebellar angle coexisting with schwannoma is an unusual finding, thus our case is the first report.
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PMID:Coexistence of diffuse large B-cell lymphoma within pontocerebellar angle schwannoma. 1223 Feb 55

A 71-year-old woman was admitted to our hospital with asthenia, weight loss, fever, cognitive impairment and shortness of breath. Physical examination showed hemiparesis and cerebellar ataxia. There was no superficial lymphadenopathy. Blood tests showed raised levels of C-reactive protein and lactate dehydrogenase. Bone marrow aspiration and biopsy were negative. [18F]fluorodeoxyglucose (FDG)-positron emission tomography (PET)/computed tomography (CT) showed intense uptake within a right apical nodule and intense and diffuse uptake of FDG in the lungs without corresponding structural CT abnormality. Lung biopsy showed intravascular large B-cell lymphoma (IVLBCL). FDG-PET findings in IVLBCL and causes of diffuse FDG lung uptake with and without CT abnormalities are discussed.
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PMID:Diffuse pulmonary uptake on FDG-PET with normal CT diagnosed as intravascular large B-cell lymphoma: a case report and a discussion of the causes of diffuse FDG uptake in the lungs. 2227 25

Ataxia-telangiectasia (A-T) is an autosomal recessive disorder characterized by cerebellar ataxia, telangiectasia, immune defect, and predisposition to leukemia/lymphoma. Because of their hypersensitivity to DNA-damaging agents, patients with A-T may require special consideration. However, an optimal strategy for these patients has not been established. Here, we describe an A-T female with diffuse large B-cell lymphoma successfully treated with rituximab combined with reduced-dose chemotherapy. Given the high incidence of hematopoietic malignancies in patients with A-T, and the hypersensitivity of these patients to DNA-damaging agents, we discuss whether a reduced-dose chemotherapy with a molecular targeting agent may be of merit.
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PMID:Successful treatment of diffuse large B-cell lymphoma in a patient with ataxia telangiectasia using rituximab. 2338 3

Ataxia-telangiectasia (AT) is a rare autosomal recessive disorder that is characterized by progressive cerebellar ataxia, telangiectasia, immunodeficiency, and a predisposition to leukemia/lymphoma. Here we report a rare case of lymphoma of the tongue accompanied by AT. Tumour extirpation was performed and diffuse large B-cell lymphoma was diagnosed following pathologic examination. A whole-body survey showed no other enlarged lymph nodes or tumour. The female patient then received a modified dosage of COPAD (cyclophosphamide, vinblastine, pirarubicin, and prednisolone) plus rituximab to avoid severe complications. As of follow-up after 3 years and 5 months, she remains in complete remission. Patients showing AT need careful surveillance and long-term continuous follow-up.
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PMID:Primary Diffuse Large B-cell Lymphoma Arising in the Tongue Accompanied by Ataxia-telangiectasia: A Case Report. 2626 30

5-Aminolevulinic acid (5-ALA)-induced fluorescence-guided resection is a widely used procedure for patients with malignant gliomas. However, the clinical application of 5-ALA for surgery in primary central nervous system lymphoma (PCNSL) is uncommon. Here, we present a case of PCNSL treated using 5-ALA-induced fluorescence-guided resective surgery. A 70-year-old woman presented with cerebellar ataxia, and magnetic resonance imaging revealed an irregularly shaped and homogenously enhanced mass with surrounding brain edema in the vermis that extended to the right hemisphere of the cerebellum. Under the preoperative diagnosis of a malignant glioma in the cerebellum, the patient underwent 5-ALA-induced fluorescence-guided surgery. Under blue light illumination, the tumor revealed strong 5-ALA-induced fluorescence. The tumor was identified as a diffuse large B-cell lymphoma. After partial resection, the patient received adjuvant chemotherapy and radiotherapy. Importantly, the neurological deficit of the patient improved, and recurrence of the tumor was not observed 21 months post-surgery. Together with previous reports, this case study emphasizes the efficacy of the surgical application of 5-ALA for PCNSL.
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PMID:5-Aminolevulinic Acid-Induced Fluorescence in Cerebellar Primary Central Nervous System Lymphoma: A Case Report and Literature Review. 2644 50