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)

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

Intravascular B-cell lymphoma is a rare aggressive systemic neoplasm with cutaneous and neurological presentations, which commonly eludes the diagnosis ante mortem. First reported in 1959 as "angioendotheliomatosis proliferans" by Pfleger and Tappeiner, it is a subtype of extranodal diffuse large-B-cell lymphoma defined by an intravascular proliferation of clonal lymphocytes. We describe a case of intravascular lymphoma in a 68-year-old female who presented with altered mental status and indurated, erythematous, ecchymotic plaques with overlying telangiectasia and ulceration. The diagnosis was made by skin biopsy of an abdominal plaque revealing large hyperchromatic cells filling the lumina of several small blood vessels within the dermis and subcutis. CD20 and CD79a immunostains were strongly positive, confirming the diagnosis of intravascular large-B-cell lymphoma. On review of a previous biopsy from another institution, which was reported to be nondiagnostic, we were able to find tumor cells in the blood vessels but only very focally. The presence of a brisk, perivascular, nonneoplastic lymphocytic infiltrate may have obscured the identification of tumor cells. This case illustrates an unusual subtype of extranodal diffuse large-B-cell lymphoma, which demonstrates protean clinical presentations, requires microscopic examination for diagnosis, but can be easily overlooked on skin biopsy.
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PMID:Intravascular B-cell lymphoma: the role of skin biopsy. 1849 38

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

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