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Query: UMLS:C0024141 (systemic lupus erythematosus)
 44,322 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We studied 30 children with systemic lupus erythematosus in order to detect the clinical, pathological and serological findings associated with the development of thrombocytopenic purpura and hemolytic anemia. A group of 13 of the 30 children revealed hematological manifestations as the most prominent changes of SLE. Thrombocytopenic purpura and hemolytic anemia were the beginning manifestation of SLE in 11 children. Different causes, for these hematological changes such as hypersplenism, renal microangiopathy or drugs reactions were excluded by history and examination. Interestingly, other immunohematological manifestations including splenomegaly and lymphadenopathy were more frequent in children with thrombocytopenic purpura and hemolytic anemia, than in those patients without these hematological complications. Positive antiplatelet antibodies were found in 4/6 children with thrombocytopenia and 2/5 with hemolytic anemia. A relation of antiplatelet with anticardiolipin antibodies occurred in 4 patients; 3 of them in children with thrombocytopenic purpura and one with hemolytic anemia. Anti-dsDNA and anti-Sm antibodies were positive in almost all patients. Four children shown a transition from anti-dsDNA to anti-Sm antibodies or viceversa and all of them revealed a significant variation in the titer of these antibodies by ELISA, in relation with disease activity. The presence of hematological manifestations associated with anti-platelet and anti-cardiolipin antibodies in children with SLE support that different mechanisms triggers autoimmunity in childhood.
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PMID:[Thrombocytopenic purpura and hemolytic anemia in children with generalized lupus erythematosus]. 193 Jul 14

Anemia is a common finding in children with systemic lupus erythematosus (SLE). Anemia in SLE may result from several mechanisms and more than one may be operative at any time. Anemia of chronic disease is the most common, but a low hemoglobin can be caused by auto-antibodies to red cells as part of the auto-immunity, or it may be the result of impaired erythropoietin production by kidneys involved in the SLE, gastrointestinal blood loss from anti-inflammatory therapy, increased red cell destruction from hypersplenism or a drug-induced immune phenomenon. We briefly review the important processes that can lead to a low hemoglobin in children with SLE, their clinical features and their treatment.
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PMID:Anemia in systemic lupus erythematosus. 213 50

We measured the calmodulin content in platelets in 13 normal persons and in 62 patients with hematological diseases. The level of platelet calmodulin was higher in patients with idiopathic thrombocytopenic purpura (ITP), systemic lupus erythematosus, myeloproliferative disorders, acute leukemia in a recovery phase, aplastic anemia, thrombosis and hypersplenism as compared to the controls. Among the patients with ITP, calmodulin was lower in responders than in nonresponders and those at the initial diagnosis. We also measured the volume, life-span and aggregation of the platelets and demonstrated a significant relationship between the calmodulin level and the platelet volume, and a negative relationship between the calmodulin level and platelet life-span, there was no correlation between the calmodulin level and platelet aggregation. We thus conclude that platelet calmodulin is inversely correlated with platelet turnover.
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PMID:Platelet calmodulin correlates with platelet turnover. 230 19

BALB/c mice rendered tolerant to alloantigens by neonatal injection of semi-allogeneic (C57BL/6 x BALB/c)F1 spleen cells develop a thrombocytopenia in association with an autoimmune lupus-like syndrome. The possible mechanisms involved in the thrombocytopenia were investigated. The development of thrombocytopenia was first detected at 3 weeks of age coinciding with the start of the other autoimmune manifestations and was always related to a state of tolerance and B cell chimerism. There was a significant increase of megakaryocytes in bone marrow and spleens from thrombocytopenic tolerant mice and radiolabeled platelets from these mice were more rapidly eliminated from the bloodstream than normal platelets when injected into normal recipients. A significant correlation between the spleen weight and the decrease of the circulating platelets was observed, although some mice with severe thrombocytopenia had only a moderate spleen enlargement. Thrombocytopenia significantly correlates with the levels of platelet-associated IgG (PAIgG) but not with anti-single-stranded DNA antibodies or circulating immune complexes. Platelets from mice with high levels of PAIgG had a shorter life-span when injected into normal mice than those from mice with low or normal PAIgG. The possibility that PAIgG are partially due to antibodies reacting specifically with platelet membrane components was analyzed. First, F(ab')2 Ig fragments from tolerant mice were shown to bind to normal platelets, in contrast to F(ab')2 Ig fragments from normal mice. Second, some monoclonal antibodies produced by hybridomas derived from tolerant mice reacted in vitro with platelets and induced a transient thrombocytopenia after i.v. injection into normal mice. These data suggest that the thrombocytopenia observed in tolerant mice is the result of a peripheral hyperdestruction of platelets associated with (a) hypersplenism, (b) nonspecific fixation of immunoglobulins, probably as immune complexes and (c) with autoantibodies reacting specifically with platelets. It may represent an interesting model for human chronic idiopathic thrombocytopenia.
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PMID:Thrombocytopenia associated with the induction of neonatal tolerance to alloantigens: immunopathogenic mechanisms. 279 84

The most common hematologic and oncologic indications for splenectomy in childhood are hereditary spherocytosis, chronic idiopathic thrombocytopenic purpura, hypersplenism, and Hodgkin's disease. Because of the increased incidence of septic complications after splenectomy, benefits to be gained from the operation should be weighed against the risks. A retrospective study was done on the charts of 42 consecutive children with hematologic and oncologic disorders, who underwent splenectomy between 1967 and 1982. The incidence of septic complications after splenectomy was 12%; sepsis, however, only occurred in patients with severe underlying diseases (three patients with Hodgkin's disease, one patient with systemic lupus erythematosus, and one patient with chronic pseudo-malignant immunoproliferation). In contrast, none of the patients who were splenectomized for other reasons (mainly hereditary spherocytosis and chronic immune thrombocytopenic purpura) had a septic complication. Two patients with end-stage Hodgkin's disease (5%) experienced fatal septic complications. Although splenectomy is well established for diagnostic and therapeutic considerations in patients with Hodgkin's disease, not all of them might benefit from this operation, and studies with a more limited approach to splenectomy might prove to be of the same therapeutical value.
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PMID:Hematological and oncological indications for splenectomy in children. 392 22

Out of 2,474 bone marrow biopsies we have observed 330 cases (13.3%) with presence of lymphoid nodules (LN). LN were frequent in old age (24.6% over 80 years), in females (17%) and in some diseases, such as rheumatoid arthritis and systemic lupus erythematosus (73.7% of the cases), partial aplasia (34%), hypersplenism (30.4%), hemopoietic dysplasia (25%), chronic renal failure (20.4%), polycythemia vera (20.2%), idiopathic thrombocytopenic purpura (18.8%), acute leukemia (17.7%). Nodular lymphoid hyperplasia of the bone marrow was found especially in systemic autoimmune diseases (26.3%), hypersplenism (9.8%), preleukemia (7.3%) and acute leukemia (4.2%). The presence of excessive medullary LN could indicate a bone marrow microenvironment damage, possibly of autoimmune origin.
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PMID:Lymphoid nodules and nodular lymphoid hyperplasia in bone marrow biopsies. 393 2

Systemic lupus erythematosus (SLE) remains a disease of unknown origin, characterized by major alterations of both the cellular and the humoral arms of immunity. Hematological changes, including anaemia, leucopenia and thrombocytopenia, occur in more than one half of patients with this disease. Anaemia is the most common hematological abnormality seen in SLE. Its possible causes are anaemia of chronic disease (ACD), auto-immune haemolytic anaemia and hypoplastic anaemia. Leucopenia affects both granulocytic and lymphocytic lines and may be caused by autoantibodies. The influence of drugs, hypersplenism and marrow suppression are also possible. Thrombocytopenia occurs frequently and is almost invariably autoimmune. Patients with SLE are at increased risk of thrombosis. Haematological abnormalities in patients with this disease require careful long-term monitoring and prompt therapeutic intervention.
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PMID:[Hematologic problems in systemic lupus erythematosus]. 765 19

The aim of this study was to explore application value of detecting platelet associated antibody and platelet membrane glycoprotein in the diagnosis and prognosis for immune thrombocytopenia. The platelet associated immunoglobulin (PAIg) and platelet membrane glycoprotein (CD41, CD61, GPIIb/IIIa) in 76 cases of immune thrombocytopenia and 30 healthy subjects were determined by FCM. The results showed that PAIg level in ITP patients included PAIgG (31.25 +/- 18.06)%, PAIgM (32.41 +/- 15.51)%, PAIgA (23.39 +/- 16.67)% which were remarkedly higher than in health control (10.48 +/- 5.05)%, (9.40 +/- 4.42)% and (7.23 +/- 3.61)% (P < 0.001). In patients with secondary immune thrombocytopenia (chronic aplastic anemia, SLE, Evans syndrome, liver cirrhosis hypersplenism, etc), PAIg level was higher than that in control group, while the platelet membrane glycoprotein in the blood of these patients was lower than that in control group. The level of PAIg decreased (P < 0.05) after treatment, but platelet membrane glycoprotein increased (P < 0.01). The result suggested that measurements for platelet membrane glycoprotein and platelet associated antibody by FCM were practical with high sensitivity, rapidity and simplicity used as a routine method in diagnosis and evaluation of the therapeutic effects in immune thrombocytopenia patients.
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PMID:[Significance of detecting platelet associated antibody and platelet membrane glycoprotein for diagnosis of immune thrombocytopenia]. 1515 39

The use of (51)Cr-labeled red blood cells to determine erythrocyte survival in circulation is not a routine procedure. Data are acquired by frequent counting of small-volume blood samples to determine the rate of red cell survival over time. Results are entirely quantitative and can involve semilogarithmic graphing techniques. An accurate study can positively identify hemolytic anemia, a disorder characterized by premature destruction of erythrocytes. Additionally, anatomic sites of premature hemolysis will demonstrate a higher radioactive count than the background organ. This procedure review presents a dual survival and sequestration study positive for hemolytic anemia and hypersplenism in an 83-y-old man with systemic lupus erythematosus. (51)Cr red cell survival and splenic sequestration studies may be considered when clinical history and laboratory studies cannot establish a definitive diagnosis.
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PMID:Determination of hemolytic anemia through the study of (51)Cr red cell survival and splenic sequestration. 1848 42

Immune thrombocytopenic purpura (ITP) is the most common cause of thrombocytopenia in children and adolescents. However, there are a number of other diagnoses that are often mistaken for ITP. A 10-year retrospective chart review was performed at the Children's Hospital of Alabama to characterize ITP. Initially, 492 patients who had the coded diagnosis of ITP (ICD 287.3) were identified. However, 83 (17%) of patients were found to have alternative diagnoses on chart review. Of the 83 patients, 13 patients (3%) represented coding errors or study classification errors. The 70 remaining patients (14%) had an alternative explanation for their thrombocytopenia, consisting of 31 different diagnoses. The most common diagnoses were familial thrombocytopenia (10%), systemic lupus erythematosus (9%), hypersplenism (9%), neonatal alloimmune thrombocytopenia (7%), Wiskott-Aldrich syndrome (7%), or systemic infection (6%). In total, 16 of the patients (23%) were ultimately diagnosed with one of a number of congenital syndromes with concurrent thrombocytopenia. Although this review confirms that most children with thrombocytopenia are diagnosed with ITP, 14% of the study population manifested other diagnoses. The clinician evaluating a child with thrombocytopenia must keep an open mind about the possible diagnosis and perform a comprehensive and thoughtful evaluation based on the clinical picture. ITP must be a diagnosis of exclusion as misdiagnosis in a child with thrombocytopenia may have a significant impact on morbidity and mortality.
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PMID:Thrombocytopenic syndromes masquerading as childhood immune thrombocytopenic purpura. 2109 29


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