1/28. Excellent effect of steroid plus azathioprine in a young woman with pernicious anaemia and systemic lupus erythematosus.We describe a 29-year-old woman who developed pernicious anaemia 2 years after the diagnosis of systemic lupus erythematosus. This is a rare association despite the relationship between the autoimmune aetiologies of these two conditions. Seven other cases have been described, but our report demonstrates a case with an excellent response to steroid and azathioprine.- - - - - - - - - - ranking = 1keywords = anaemia (Clic here for more details about this article) |
2/28. Waldenstrom's macroglobulinaemia with intracerebral haemorrhage.A 65-year-old male was admitted for evaluation of severe anaemia, recurrent epistaxis, axillary lymphadenopathy, and hepatomegaly. The diagnosis of Waldenstrom's macroglobulinaemia was made on the basis of clinical and laboratory findings. The patient developed intracerebral haemorrhage without associated hypertension and with normal coagulation profile.- - - - - - - - - - ranking = 0.2keywords = anaemia (Clic here for more details about this article) |
3/28. Rapidly progressive lupus glomerulonephritis and concomitant microangiopathy in an adolescent.We describe our experience managing a 16-year-old girl with systemic lupus erythematosus (SLE) who presented concomitantly with rapidly progressive glomerulonephritis (RPGN) and a thrombotic microangiopathic hemolytic anemia (TMAHA). Her renal biopsy showed evidence of diffuse proliferative glomerulonephritis without glomerular microthrombi. The patient was treated with a combination of intravenous corticosteroids and cyclophosphamide, as well as plasmapheresis, with an excellent response resulting in complete disease remission. The purpose of our report is to make health professionals more aware of TMAHA as a complication of SLE, since the occurrence of TMAHA may confuse the clinical picture, and since its treatment with plasmapheresis is life saving, if performed early.- - - - - - - - - - ranking = 0.40567495085386keywords = hemolytic (Clic here for more details about this article) |
4/28. Systemic lupus erythematosus in children: a case report and review.A 12-year-old Papua New Guinean female presented initially with nonspecific clinical symptoms, fever of unknown origin and anaemia. She subsequently developed multisystem disease involving the respiratory, gastrointestinal, central nervous, musculoskeletal and cutaneous systems. She was diagnosed to have systemic lupus erythromatosus (SLE) and started on treatment. Unfortunately the patient defaulted from follow-up after treatment, which covered seven months only, to present with acute respiratory distress from which she died within 24 hours. A relevant literature review with the clinical features of systemic lupus erythematosus in children is described.- - - - - - - - - - ranking = 0.2keywords = anaemia (Clic here for more details about this article) |
5/28. Severe autoimmune hemolytic anemia following rituximab therapy in a patient with a lymphoproliferative disorder.Rituximab (chimeric anti-CD20 IgG1 monoclonal antibody) is effective in the treatment of relapsed/refractory low-grade lymphomas of B-cell origin as well as in diffuse large B-cell lymphoma. Several reports also demonstrated the efficacy of rituximab for the treatment of autoimmune cytopenia, especially for cold agglutinin disease. We report the first case, to our knowledge, of rituximab-related autoimmune hemolytic anemia. The pathophysiological mechanisms remain unknown, although the drug could act through massive cytokines liberation after destruction of CD20 positive cells by rituximab.- - - - - - - - - - ranking = 2.0283747542693keywords = hemolytic (Clic here for more details about this article) |
6/28. Haemoglobin concentration and prognosis in new cases of heart failure.Anaemia is common in severe chronic heart failure and is reported to be a predictor of death. We investigated 552 patients (median age 76 years, range 29-95; 54% men [n=296]), in whom the duration of heart failure was sufficiently short that it would be unlikely to affect haemoglobin concentrations. By contrast with studies in established chronic heart failure, haemoglobin was not independently associated with prognosis when age and serum creatinine concentration were included in the analysis. The adverse effects of anaemia on survival might be a consequence of chronic heart failure rather than a separate process causing disease progression.- - - - - - - - - - ranking = 0.2keywords = anaemia (Clic here for more details about this article) |
7/28. Invasive group A streptococcal infection after tonsillectomy.tonsillectomy is a very common procedure in childhood. Infectious complications after tonsillectomy are infrequently reported. We describe two children with severe group A beta-hemolytic streptococcal infection after tonsillectomy, and we review the literature about bacteremia and infectious complications after tonsillectomy.- - - - - - - - - - ranking = 0.40567495085386keywords = hemolytic (Clic here for more details about this article) |
8/28. Dermatologic and immunologic findings in the immune dysregulation, polyendocrinopathy, enteropathy, X-linked syndrome.BACKGROUND: The immune dysregulation, polyendocrinopathy, enteropathy, X-linked (IPEX) syndrome is a rare genodermatosis associated with dermatitis, enteropathy, type 1 diabetes, thyroiditis, hemolytic anemia, and thrombocytopenia. IPEX results from mutations of FOXP3, a gene located on the x chromosome that encodes a dna-binding protein required for development of regulatory T cells. If untreated, affected males die early in life from malabsorption and other complications. To our knowledge, this syndrome has never been described in the dermatology literature. OBSERVATIONS: We studied an 11-year-old boy with IPEX. mutation analysis revealed a G-->A transition (1150G>A) in exon 11, resulting in a putative substitution of Ala-->Thr at residue 384, within the dna-binding site. Histopathologic examination of an active skin lesion revealed psoriasiform dermatitis. The lesions improved with clobetasol ointment. The patient also displayed alopecia universalis, which had been present since age 18 months, accompanied by longitudinal ridging of the nails. Lymphocyte challenge tests revealed a profound inability to synthesize interferon gamma (INF-gamma) and dysregulated production of other cytokines. CONCLUSIONS: IPEX is an often fatal genodermatosis associated with multiple autoimmune disorders. Cutaneous findings may include dermatitis, bullae, urticaria, alopecia universalis, and trachyonychia. Recognition of this life-threatening disorder is crucial for optimal treatment and genetic counseling.- - - - - - - - - - ranking = 0.40567495085386keywords = hemolytic (Clic here for more details about this article) |
9/28. An immediate hemolytic reaction induced by repeated administration of oxaliplatin.BACKGROUND: platinum-based chemotherapy agents have been associated with potentially fatal acute immune-mediated hemolytic anemia. The target antigen, cause of the positive direct antiglobulin test (DAT) and mechanism of hemolysis have been the subject of controversy. CASE REPORT: We report a patient who developed a DAT-positive hemolytic episode after a red cell (RBC) transfusion was delivered during the infusion of her 17th cycle of oxaliplatin. Standard pretransfusion testing was uncomplicated; however, after infusion, the serum was no longer compatible with the transfused units and a strong (4 ) panreactive IgG antibody was detected. RESULTS: The patient's serum from 10 days after the episode, only when therapeutic concentrations of oxaliplatin were added, reacted with all RBCs tested using the indirect antiglobulin test (IAT) (3 ). The effect was retained with a purified IgG fraction and almost eliminated with IgG-depleted serum. immunoprecipitation analysis revealed a band with the molecular weight of the Band 3 anion channel only in the presence of the patient's serum and oxaliplatin. CONCLUSION: Our investigations indicated that oxaliplatin interacted with both an IgG antibody and a RBC membrane epitope probably located on the Band 3 anion channel.- - - - - - - - - - ranking = 2.4340497051231keywords = hemolytic (Clic here for more details about this article) |
10/28. Sustained remission of CIDP associated with Evans syndrome.A patient with chronic inflammatory demyelinating polyneuropathy (CIDP) developed Evans syndrome (hemolytic anemia/thrombocytopenia) 17 months after onset of symptoms despite different immunomodulatory treatments. A therapeutic approach with the chimeric monoclonal anti-CD20 antibody rituximab induced substantial improvement of CIDP and hematologic recovery. The patient remains in sustained remission 17 months after completion of therapy. Rituximab may represent a successful therapy in otherwise refractory CIDP.- - - - - - - - - - ranking = 0.40567495085386keywords = hemolytic (Clic here for more details about this article) |
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