Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0002878 (hemolytic anemia)
 7,530 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

An automated antiglobulin test showed that erythrocytes of a patient with an acute episode of Coombs-negative hemolytic anemia were strongly sensitized with nonagglutinating IgM molecules. The bound antibodies, after elution from red blood cell stroma, were found to be monomeric IgM, since they migrated with IgG molecules on an agarose column, although they were proved to be immunoglobulin M, not G, by a sensitive hemagglutination inhibition assay. The hemolysis subsided with steroid therapy, but ANA increased to a titer greater than 1,024 (with peripheral pattern) without other laboratory evidence of systemic lupus erythematosus. In addition, the patient demonstrated photosensitivity and nonscarring alopecia. We consider that this appearance of erythrocyte autoantibodies consisting of monomeric IgM was a symptom of atypical SLE.
 ...
PMID:Erythrocyte sensitization with monomeric IgM in a patient with hemolytic anemia. 674 33

Thrombotic thrombocytopenic purpura (TTP) and systemic lupus erythematosus (SLE) are both multisystem diseases and the latter can manifest hemopoietic abnormalities that may mimic TTP. This has led to diagnostic confusion and reports of the 2 diseases occurring in a single patient. We describe a 15-yr-old girl who presented at age 12 with purpura, fever, headaches, changes in conscious state, thrombocytopenia and microangiopathic hemolytic anemia and who was diagnosed despite the absence of renal disease, as having TTP. ANA and LE cells were negative then, and again 1 yr later. Three yr later she presented with the nephrotic syndrome with a positive ANA (1:100), elevated DNA antibody (79 U/ml, normal less than 25) and a circulating anticoagulant. Renal biopsy confirmed the presence of lupus nephritis. The association is discussed.
 ...
PMID:Systemic lupus erythematosus and thrombotic thrombocytopenic purpura. A case report and review of relationship. 702 83

Interferons (IFNs) have immunomodulatory properties such as direct increase in the production of pathogen autoantibodies, enhanced cytotoxic T cell and B cell activities, inhibition of T suppressor cell function and induction of HLA class I antigen expression. These actions of IFN induce autoimmune disorders including autoimmune thyroiditis, hemolytic anemia and thrombocytopenia, SLE, rheumatoid arthritis and psoriasis, however, these autoimmune diseases except for autoimmune thyroiditis, are rare among side effects of IFN therapy. Most of the patients showing these autoimmune disorders during IFN treatment have predisposal immunological abnormalities such as positive ANA and antithyroidal autoantibodies. We described here autoimmune disorders during and after IFN treatment among 1023 cases with chronic active type C hepatitis. The cases with SLE, thrombocytopenic purpura, rheumatoid arthritis and psoriasis showed good prognosis after cessation of IFN administration.
 ...
PMID:[Autoimmune disorders in interferon therapy]. 752 40

We describe a patient with abdominal pain and ascites, mesenteric lymphadenitis and peritoneal panniculitis. Initially her ANA was negative. The abdominal pain recurred again three years later and in between the two episodes she had had skin rash, alopecia, arthralgia, and positive Coombs' test-haemolytic anaemia. Her ANA became positive a few years after the initial episode.
 ...
PMID:Recurrent peritonitis with ascites as the predominant manifestation of systemic lupus erythematosus. 764 15

A 30-year-old man with long-standing localization-related epilepsy and mental retardation had seizures that were partially controlled with valproate (VPA) 500 mg four times daily. Routine examination showed severe thrombocytopenia with mild leukopenia and chronic low-grade hemolytic anemia. Pertinent laboratory results included positive ANA, rheumatoid factor, anti-NIA, circulating immune complexes, and antihistone antibody. The patient was treated with high dosage prednisone with partial improvement, but continued to have exacerbations at lower dosages. Fourteen months later, VPA was discontinued, and rapid improvement ensued. Prednisone was subsequently discontinued, and the patient has now maintained normal platelet counts for 18 months.
 ...
PMID:Systemic lupus erythematosus associated with use of valproate. 811 40

The course and severity of systemic lupus erythematosus (SLE) in children is generally similar to the adult form with potential serious organ system involvement, there are, however, factors that influence the prevalence and clinical behavior of the disease. Our objective was to analyse the organ system involvement and immunological findings in Kuwaiti children with SLE in relation to gender and age of onset and compare these findings to that in published reports. Organ system involvement and serologic profiles were analysed in 35 children with SLE. The major organ systems studied were: renal, hematological, cardiac, pulmonary, hepatic and the central nervous system. The prevalence of ANA, anti-dsDNA, anti-Sm, SSA, SSB and anti-cardiolipin antibodies were studied in addition to complement C3 and C4 levels. The results showed that a high percentage of children had hematological involvement (34%); thrombocytopenia (23%) and hemolytic anemia (20%). Renal involvement was proven by biopsy in only 10 children (29%). Neuropsychiatric manifestations were seen in five (14%) of patients. Males had a tendency for major organ involvement relative to females. All patients had positive ANA tests. All males had positive anti-dsDNA tests compared to 86% of female patients. The most significant finding in this study is the high frequency of hematological manifestations and the relatively low incidence of renal disease and neuropsychiatric abnormalities in Kuwaiti children with SLE.
 ...
PMID:Systemic lupus erythematosus in Kuwaiti children: organ system involvement and serological findings. 1546 94

The objective of the study was to analyse the prevalence and clinical significance of hypocomplementemia in a large series of patients diagnosed either with systemic lupus erythematosus (SLE) or with primary antiphospholipid syndrome (APS) and its association with the main clinical, hematological and immunological features of these diseases. Between 1992 and 2003, complement determinations (C3 and C4 levels, CH50 activity) were performed in 597 consecutive patients diagnosed with SLE (530 women and 67 men, mean age 32.6 years) and 70 with primary APS (57 women and 13 men, mean age 38.7) visited in our department. Complement determinations are routinely made at the first visit of patients and yearly during the follow-up. SLE and primary APS were diagnosed according to current classification criteria. Hypocomplementemia was detected in 371 (62%) of SLE patients. Compared with patients with normal complement values, those with hypocomplementemia showed a higher prevalence of female gender (P < 0.001), fever (P = 0.021), nephropathy (P < 0.001), cutaneous vasculitis (P = 0.023), positive anti-dsDNA antibodies (P = 0.012) and cryoglobulinemia (P < 0.001). In addition, patients with hypocomplementemia showed a higher prevalence of APS-related features such as hemolytic anemia (P = 0.001) and antiphospholipid antibodies (P < 0.001). Hypocomplementemia was prospectively related to accumulated hospitalization in SLE patients but not with the accumulated number of lupus flares or with the survival after follow-up of five years. In contrast, 33 (47%) patients with primary APS presented low complement values, which were associated with a higher prevalence of livedo reticularis (P = 0.022), thrombocytopenia (P = 0.004), lupus anticoagulant (P = 0.013), positive IgM-aCL (P = 0.039), positive ANA (P = 0.002) and anti-dsDNA (P = 0.046). The diagnostic value of hypocomplementemia in patients with SLE is based on the association with disease activity, immune-complex mediated manifestations (glomerulonephritis, cryoglobulinemia) and APS-related features (livedo reticularis, hemolytic anemia and aPL). Hypocomplementemia was found in nearly half of patients with primary APS, and was associated with some APS features (livedo reticularis, thrombocytopenia, aPL) but also with SLE-related immunological markers (ANA and anti-dsDNA), identifying a subset of patients with primary APS with a higher risk of evolving to SLE. These results clearly support the routine determination of complement factors in the clinical follow-up of patients with SLE and primary APS.
 ...
PMID:Hypocomplementemia in systemic lupus erythematosus and primary antiphospholipid syndrome: prevalence and clinical significance in 667 patients. 1554 May 10

We described a case of thrombotic thrombocytopenic purpura (TTP) with systemic lupus erythematosus (SLE). A-60-year old woman was admitted to our hospital because of fever, disconsciousness, and general fatigue. 32 years ago, she was diagnosed as SLE with Raynaud's phenomenon, rash, photosensitivity, arthritis, lymphocytopenia, and ANA. Her SLE was well controlled with 10 mg predonisolone as a maintance dose until several weeks ago. On admission, severe thrombocytopenia (0.7x10(4)/microl) and other laboratory data revealed microangiopathic hemolytic anemia and renal dysfunction, Immediately after diagnosed as TTP, plasma exchange and corticosteroid therapy started. In spite of the treatment, disconsciousness progressed and systemic convulsion occurred and died 4 days after admission. Autopsied examination revealed diffuse microvascular hyalinized thrombi in heart, kidney, liver, spleen, and pancreas. Some microvascular thrombi were detected in lymph nodes, bone marrow, intestine. Pathological diagnosis of TTP was made on microvascular hyalinized platelet thrombi in organs. Von Willebrand factor-cleaving protease (VWF-CP) activity in plasma on set is less than 0.5 percent of normal and inhibitor for VWF-CP was detected. We here report a valuable case for analysis of pathogenesis in SLE-TTP.
 ...
PMID:[A case of thrombotic thrombocytopenic purpura with systemic lupus erythematosus]. 1707 94

A 13-year-old girl presented to our emergency with a one week history of fever and skin rash and new onset of chorea for the last three days. There was a long standing history of right predominant headache; followed by personality change, fatigue, arthralgia and weight loss over the last few months. Previous investigations by head CT and ophthalmological examination did not explain the symptoms. Further investigations revealed peri- and pancarditis with aortic insufficiency, a renal involvement with elevated creatinin, protein- and hematuria and a hemolytic anemia. Diagnosis of lupus eythematodes was confirmed by high ANA, anti-dsDNS and Anticardiolipin antibodies. Within the first 48 hours after admission there was significant deterioration with reduced vigilance and dysarthria. MRI of the brain and dopplersonography of cerebral vessels showed a complete thrombosis of the right medial cerebral artery with a small net of collaterals, irregularities of the left cerebral artery due to vasculitis and several subacute leftsided ischemias. Immunosuppressive therapy with high-dose corticosteroids and cyclophosphamid together with antithrombotic therapy induced an improvement of neurologic, renal and cardiac function.
 ...
PMID:[Vasculitis as a reason of chronic headache]. 1854 40

Scleroderma renal crisis (SRC) occurs in 5-10% of SSc patients, who may present with an abrupt onset of hypertension, acute renal failure, headaches, fevers, malaise, hypertensive retinopathy, encephalopathy and pulmonary oedema. Patients at greatest risk of developing SRC are those with diffuse cutaneous or rapidly progressive forms of SSc, and treatment with a recently commenced high dose of corticosteroid. Laboratory tests may demonstrate hypercreatinaemia, microangiopathic haemolytic anaemia (MAHA), thrombocytopaenia and hyperreninaemia. Renal crisis is also linked to a positive ANA speckled pattern, antibodies to RNA polymerase I and II, and an absence of anti-centromere antibodies. Early, aggressive treatment with angiotensin-converting enzyme inhibitors has improved prognosis in SRC, although 40% of the patients may require dialysis, and mortality at 5 yrs is 30-40%. Median time to recovery is 1 yr, and typically occurs within 3 yrs. Prognosis is worse for males, but may not be related to corticosteroid use, presence of MAHA or severity of renal pathology. Modification of endothelin over-activity, which is implicated in the pathogenesis of SRC, may offer a future therapeutic approach.
 ...
PMID:Renal complications and scleroderma renal crisis. 1948 21


1 2 Next >>