Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0409974 (lupus)
 22,386 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 61-year-old woman experienced a high fever with anemia and APTT prolongation after suffering a herpes zoster virus infection. Physical examination revealed a large splenomegaly without lymphadenopathy. Laboratory evaluations were positive for lupus anticoagulant (LA) and monoclonal IgM-kappa protein. LA was associated with the presence of anti-beta2GPI antibody, anti-cardiolipin antibody, and anti-prothrombin antibody. Moreover, the results of factors IX, XI, and XII assays and CRP and FDP-E were disturbed. A splenectomy was performed, and a splenic marginal zone lymphoma (SMZL) was diagnosed. All hematological findings rapidly recovered after the splenectomy. No thrombotic events occurred after the splenectomy even though thrombosis prophylaxis was not performed. The clinical course suggested that the SMZL-producing antibody induced immunological abnormalities in the labolatory tests. Since the patient suffered disease progression soon after the splenectomy, an autologous peripheral stem cell transplantation with rituximab administration was performed.
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PMID:[Splenic marginal zone lymphoma associated with antiphospholipid antibodies]. 1555 43

Splenic lymphoma with villous lymphocytes (SLVL) is a rare lymphoproliferative disorder characterized by the presence of typical lymphoid cells with villous projections and monoclonal immunoglobulin (M-Ig) in about 30% of patients. The simultaneous presence of more than one M-Ig in SLVL has not been reported. We present two patients with SLVL, each with three serum M components associated with the presence of rheumatoid factor (RF) and antiphospholipid antibodies (APLA) together with fatal thromboembolic events. Both patients presented with splenomegaly and typical bone marrow cytology with 30-50% infiltration of lymphoid cells that had the characteristics of villous lymphocytes. Immunohistochemistry of bone marrow histology showed CD20++, CD43-/+, CD5-, IgM+, lambda+ and kappa-. In serum, two M-IgM lambda components were combined with M-IgG lambda in case 1 and with M-IgA lambda in case 2. In both cases, M-IgM displayed RF as well as lupus anticoagulant activity and free monoclonal lambda (lambda) light chains were present. In addition to M-IgM, in case 1 M-IgG also behaved like an APLA. One patient was splenectomized. Both patients suffered thromboembolic complications and died 3 and 8 months after presentation with signs of massive pulmonary thromboembolism.
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PMID:Multiple M components in two patients with splenic lymphoma with villous lymphocytes. 1600 26

We present a case concerning a patient with splenic marginal zone lymphoma (SMZL) and isolated prolonged activated partial thromboplastin time (aPTT) caused by lupus anticoagulant. Von Willebrand factor (VWF) activity and antigen were immeasurable by latex particle immunoturbidimetric assays, and several coagulation factor levels were decreased. However, VWF activity and antigen were normal when analyzed by other methods. Also, coagulation factor levels were normal if an aPTT reagent with low lupus anticoagulant sensitivity or a chromogenic method was applied. Altogether, the initial findings were because of lupus anticoagulant interference and in fact, the patient had normal VWF activity and coagulation status. Interference of lupus anticoagulant in clot-based assays is well known but has not previously been described in VWF assays. This is furthermore the first report in which lupus anticoagulant activity in SMZL cannot be ascribed to a monoclonal immunoglobulin. In our study, aPTT normalized after treatment, suggesting resolution of lupus anticoagulant. APTT could thus be a marker of treatment response in SMZL. Whether treatment decreases the thrombosis risk due to lupus anticoagulant remains unknown.
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PMID:Interference from lupus anticoagulant on von Willebrand factor measurement in splenic marginal zone lymphoma: a case report. 2548 87