Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0024141 (systemic lupus erythematosus)
 44,322 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 58-year-old man was admitted with generalized lymphadenopathy. On admission, the patient showed polyclonal hyper-gammopathy in a blood examination, positive results in the direct/indirect Coombs test, and an elevated cold agglutinin titer. Autoimmune thrombocytopenia with a high level of platelet-associated immunoglobulin G complicated the patient's condition. An enzyme immunoassay kit for human immunodeficiency virus (HIV) recombinant proteins p24, gp41, and gp36 showed positive results. Western blot analysis showed the presence of antibodies cross-reacting with HIV p24 gag protein. HIV RNA was not detected by means of a reverse transcriptase-polymerase chain reaction assay, so the patient was not an HIV carrier. Angioimmunoblastic T-cell lymphoma (AILT) was diagnosed on the basis of lymph node biopsy specimens. We speculated that in this case some of the numerous subtypes of polyclonal gamma globulin had coincidentally cross-reacted with HIV p24. Cross-reactive phenomena with HIV in patients with systemic lupus erythematosus have been well investigated, but to our knowledge our patient is the first case of such cross-reactivity involving AILT. Physicians should pay close attention to serologic tests to determine whether the patient truly is a viral carrier.
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PMID:Angioimmunoblastic T-cell lymphoma associated with an antibody to human immunodeficiency virus protein. 1295 12

A case of atypical lymphoplasmacytic and immunoblastic proliferation (ALPIBP) in the lymph nodes associated with well-documented systemic lupus erythematosus (SLE) is presented. A 30-year-old Japanese female with an 18-year history of SLE presented with right neck lymphadenopathy of 3 months duration. A biopsy specimen showed a diffuse effaced lymph node architecture without follicles and minimal sinuses. At high power field, a polymorphous population of small- to medium-sized lymphocytes, plasma cells, plasmacytoid cells, as well as large, basophilic transformed lymphocytes and immunoblasts diffusely infiltrated the paracortical area. Interestingly, the immunohistochemical study demonstrated large, irregularly shaped accumulations of follicular dendritic cells (FDCs) surrounding the small vessels, which is an immunohistochemical finding characteristic of angioimmunoblastic T-cell lymphoma (AILT). However, the present lesion showed the following differences to AILT: (a) absence of CD3+, CD4+ and CD10+ clear cells, which are tumor cells of AILT; (b) absence of pronounced arborizing vascular proliferation; (c) on molecular analysis, the present case demonstrated a polyclonal pattern converse to the monoclonal T-cell receptor gamma chain gene rearrangement in most AILTs (d) absence of EBV infected lymphoid cells, which are frequently detected AILT. As previously suggested, the present case indicates that a clinical correlation as well as immunohistologic and genotypic studies may be necessary to discriminate between ALPIBPs and AILT.
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PMID:Atypical lymphoplasmacytic and immunoblastic proliferation from systemic lupus erythematosus. A case report. 1616 50

We analyzed 66 cases of immune-mediated thrombophilia in patients with lymphoma reported in the literature. Sixty-one cases had a lupus anticoagulant, three an antibody to protein S, one to protein C, and one to ADAMTS 13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13). Lupus anticoagulants occurred in all histological subtypes of non-Hodgkin lymphoma, except mantle cell lymphoma, MALT (mucosa-associated lymphoid tissue) lymphoma, and angioimmunoblastic T-cell lymphoma, and rarely in Hodgkin lymphoma. The largest number of cases was described in splenic marginal zone and lymphoplasmacytic lymphoma. Lupus anticoagulants were highly associated with immunoglobulin M (IgM) paraproteinemia, autoimmune hemolytic anemia, and immune thrombocytopenia. About half of the patients had thrombotic events (antiphospholipid antibody syndrome). Venous thromboembolism was more than twice as common as arterial thrombosis; 6.5% had a catastrophic antiphospholipid antibody syndrome. The lupus anticoagulant could be eliminated by lymphoma treatment (chemoimmunotherapy or splenectomy) in more than one-third of patients. It is suggested that a search for lupus anticoagulant should be done in all patients with lymphoma, because a diagnosis of lupus anticoagulant may influence the management of lymphomas in some patients.
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PMID:Acquired immune-mediated thrombophilia in lymphoproliferative disorders. 2176 5

A 49-year-old woman with severe thrombocytopenia was admitted after an episode of syncope. She also had anemia, fever, pleural effusion and ascites, and multiple lymphadenopathies subsequently appeared. Her bone marrow showed increased megakaryocytes with mild fibrosis, whereas her lymph nodes lacked histologically specific findings. Her presentation was not consistent with multicentric Castleman's disease, angioimmunoblastic T-cell lymphoma, systemic lupus erhythematosus or any other well-recognized entities. Her clinical features were, however, thought to be compatible with TAFRO (thrombocytopenia, anasarca, fever, reticulin fibrosis, and organomegaly) syndrome. Corticosteroid therapy induced a partial remission of fever and systemic fluid retention, but thrombocytopenia persisted. After additional immunosuppressive therapy with cyclosporin A, her symptoms showed full resolution. [J Clin Exp Hematop 53(1) : 95-99, 2013].
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PMID:Complete resolution of TAFRO syndrome (thrombocytopenia, anasarca, fever, reticulin fibrosis and organomegaly) after immunosuppressive therapies using corticosteroids and cyclosporin A : a case report. 2380 Nov 40

Herein we report a case of the simultaneous occurrence of angioimmunoblastic T-cell lymphoma (AITL) and systemic lupus erythematosus (SLE) in a 76-year-old woman. She presented with fever, night sweats, and general malaise. A laboratory examination revealed leukopenia, anemia, polyclonal hypergammaglobulinemia, hypocomplementemia, positive results for anti-nuclear antibodies and anti-double strand DNA (anti-dsDNA) antibodies, and mild proteinuria. A computed tomography scan of the abdominal cavity showed multiple swollen intra-abdominal and intra-pelvic lymph nodes. A biopsy specimen obtained from the peri-iliac lymph node confirmed the diagnosis of AITL, while renal biopsy results were consistent with lupus nephritis, International Society of Nephrology and Renal Pathology Society class V. These results indicated that our patient developed SLE concomitantly with AITL. These findings will lead to further understanding of the pathogenic mechanism of SLE.
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PMID:Systemic lupus erythematosus as the concomitant manifestation of angioimmunoblastic T-cell lymphoma. 2540 Dec 31

A 76-year-old woman was referred to our hospital because of fever, hemorrhagic skin lesion with pruritus, and severe thrombocytopenia. Anemia; marked eosinophilia; and elevated ALP, CRP, and soluble IL-2 receptor levels were observed on admission. Both anti-nuclear antibody and Coombs tests were positive. Computed tomography revealed bilateral pleural effusion, ascites, abdominal lymphadenopathy, and mild hepatosplenomegaly. A thorough examination for the initial differential diagnoses excluded the possibility of myeloid/lymphoid neoplasms with eosinophilia and gene rearrangement, infectious diseases, and eosinophilic granulomatosis with polyangiitis. Remaining possibilities included angioimmunoblastic T-cell lymphoma (AITL) and systemic inflammatory disorders. Although AITL was plausible, there was no histological evidence to support the diagnosis. The patient was then administered prednisolone alone, which led to a lasting resolution of her symptoms. The atypical AITL course raised the suspicion of a misdiagnosis; thus the possibility of an inflammatory disease was reconsidered. TAFRO syndrome was suspected owing to its characteristic clinical features (thrombocytopenia, anasarca, fever and organomegaly). Since a definitive diagnosis required the exclusion of systemic lupus erythematosus (SLE), anti-double-stranded DNA antibody was tested in the initial frozen serum sample. An unexpected positive result led to the final diagnosis of SLE. Here, we report a rare case of SLE lacking typical symptoms and exhibiting various hematological abnormalities, such as eosinophilia.
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PMID:[Systemic lupus erythematosus with marked eosinophilia and clinical features mimicking TAFRO syndrome]. 2997 44

Several conditions have clinical and laboratory features that can mimic those present in Systemic Lupus Erythematosus (SLE). Some of these "SLE mimickers" are very common, such as rosacea which can be mistaken for the butterfly rash, while others such as Kikuchi disease, type-1 interferonopathies, Castleman's disease, prolidase deficiency, angioimmunoblastic T-cell lymphoma, Evans' syndrome in the context of primary immune deficiencies and the autoimmune lymphoproliferative syndrome are exceptionally uncommon. A proper diagnosis of SLE must therefore be based upon a complete medical history as well as on the adequate constellation of clinical or laboratory findings. While there is no single test that determines whether a patient has lupus or not, the search for auto-antibodies towards nuclear antigens is a key step in the diagnosis strategy, keeping in mind that ANAs are not specific for SLE. In case of persistent doubt, patients should be referred to reference centers with experience in the management of the disease.
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PMID:Rare diseases that mimic Systemic Lupus Erythematosus (Lupus mimickers). 3083 56

New-onset systemic lupus erythematosus (SLE) is uncommon in elderly patients. We report the case of a 71-year-old woman who was diagnosed with SLE based on clinical manifestations of fever, alopecia, bicytopenia, hepatomegaly, lymphadenopathy, glomerulonephritis, positive antinuclear antibody (ANA) and anti-double stranded DNA (anti-dsDNA) antibody. Renal biopsy was consistent with lupus nephritis and excision biopsy of a right inguinal lymph node was initially reported as having features of reactive hyperplasia. However, a more careful review of the lymph node biopsy subsequently confirmed a concurrent angioimmunoblastic T-cell lymphoma. This case illustrates the importance of investigating secondary causes and possible alternative diagnoses in patients who present with atypical features of connective tissue disease, and the challenges in diagnosing a rare form of lymphoma.
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PMID:Angioimmunoblastic T-cell Lymphoma: A Mimic for Lupus. 3308 50