1/61. Autoimmune thyroiditis and myelosuppression following treatment with interferon-alpha for hepatitis c.CASE: We describe the case of a 48-year-old woman from thailand diagnosed with chronic hepatitis c, who experienced a suppression of all blood cell counts accompanied by a newly developed clinically manifested autoimmune thyroid disorder after treatment with interferon alpha-2b (INF-alpha) 46 days after beginning of therapy a decrease of platelet, red and white blood cell counts became obvious. Concomitantly we observed an increase of FT4 and FT3 with a totally depressed TSH level 80 days after starting INF-alpha administration. Antibody assessment resulted in detection of high numbers of antithyroid-microsomal antibodies and antithyroglobulin antibodies. Thyroid hormone levels normalized under treatment with methimazole/propylthiouracil within 4.5 months. However, two months after cessation of antithyroid therapy increasing TSH levels and decreasing FT4 levels indicated a new tendency towards a hypothyroid state. CONCLUSION: We classify this case as an interferon-alpha-induced disorder of thyroid function accompanied by myelosuppression. A close monitoring for thyroid dysfunction, e.g. evaluation of TSH-levels before and after administration of INF-alpha is mandatory.- - - - - - - - - - ranking = 1keywords = blood cell, white (Clic here for more details about this article) |
2/61. adrenoleukodystrophy associated with vitiligo and ulcerative colitis.adrenoleukodystrophy (ALD) is an X-linked inherited disorder of lipid metabolism usually presenting in childhood or early adolescence. It is a progressive disease with symptoms of adrenal insufficiency and central nervous system demyelination. The pathology results from the accumulation of very long-chain fatty acids and an inflammatory reaction in the brain white matter. We report a case of ALD associated with adrenal insufficiency and two autoimmune diseases: vitiligo and ulcerative colitis.- - - - - - - - - - ranking = 0.026613689126901keywords = white (Clic here for more details about this article) |
3/61. Intensive plasma exchange for severe autoimmune hemolytic anemia in a four-month-old infant.We report the smallest infant (7.5 kg) to receive intensive plasma exchange (52 PEs) therapy as treatment of autoimmune hemolytic anemia (AIHA). PE temporarily reduces circulating autoantibody levels and can be an effective adjunctive therapy with corticosteroids and cytotoxic drugs or other immuno-suppressants. Although his clinical course was prolonged and complicated by cytomegalovirus infection with spontaneous perforation of his colon, his recovery was complete. He has remained healthy for more than 2 years. Because of his small size, calcium gluconate was added to replacement fluids and calcium levels closely monitored. The apheresis machine and tubing were routinely primed with red blood cells and FFP substituted for 5% human albumin during the second half of all procedures to maintain adequate levels of procoagulant. Our experience suggests that intensive PE is helpful in controlling severe AIHA and should be considered even for very small patients.- - - - - - - - - - ranking = 0.48669315543655keywords = blood cell (Clic here for more details about this article) |
4/61. A subepidermal bullous eruption associated with IgG autoantibodies to a 200 kd dermal antigen: the first case report from the united states.We describe an 81-year-old white man in whom a subepidermal bullous eruption developed that clinically resembled bullous pemphigoid. The eruption promptly responded to oral tetracycline and niacinamide and topical clobetasol. Histologic examination of perilesional skin revealed neutrophilic infiltration with formation of papillary microabscesses and subepidermal cleavage. Direct immunofluorescence showed linear deposition of IgG and C3 along the basement membrane zone. By indirect immunofluorescence, circulating IgG autoantibodies bound exclusively to the dermal side of salt-split normal human skin. Immunoblot analysis demonstrated that the patient's autoantibodies reacted with a 200 kd dermal protein that was different from type VII collagen, the epidermolysis bullosa acquisita autoantigen. This patient represents the first confirmed case from the united states with a recently reported novel autoimmune subepidermal bullous disease associated with IgG autoantibodies to a 200 kd dermal antigen.- - - - - - - - - - ranking = 0.026613689126901keywords = white (Clic here for more details about this article) |
5/61. Drug-induced linear iga bullous dermatosis after vancomycin discontinuance in a patient with renal insufficiency.linear iga bullous dermatosis (LABD) is an autoimmune, subepidermal, vesiculobullous disease that has been commonly associated with the use of vancomycin hydrochloride. Lesions typically appear during vancomycin therapy, 24 hours to 15 days after the first dose. A 65-year-old white man with renal insufficiency developed pruritic, tense bullae on the right chest, right medial arm, right flank, abdomen, and right upper thigh 14 days after his last dose of vancomycin. Histopathologic examination and immunofluorescence studies were diagnostic of LABD. vancomycin-related LABD may appear as long as 2 weeks after the drug is discontinued.- - - - - - - - - - ranking = 0.026613689126901keywords = white (Clic here for more details about this article) |
6/61. Autoerythrocyte sensitization (Gardner-diamond) syndrome.We describe the clinical presentation and course of a patient with autoerythrocyte sensitization (Gardner-diamond) syndrome, and review the literature for similar cases. A 37-yr-old female presented with recurrent episodes of painful ecchymotic bruising over the anterior aspect of both thighs. These episodes were precipitated by emotional stress. The diagnosis was confirmed by induction of similar lesions by intradermal injection of the patient's own washed red blood cells and hemoglobin. The lesions did not recur for 6 months after the cause of her emotional stress was relieved. Autoerythrocyte sensitization (Gardner-diamond) syndrome should be considered in the differential diagnosis of purpura, especially in patients with psychiatric problems.- - - - - - - - - - ranking = 0.48669315543655keywords = blood cell (Clic here for more details about this article) |
7/61. Insulin receptor antibodies inhibit insulin uptake by the liver: in vivo 123I-insulin scintigraphic scanning and in vitro characterization in autoimmune hypoglycemia.BACKGROUND: Insulin receptor antibodies can induce severe hypoglycemia or insulin resistance in rare autoimmune syndromes. in vitro properties of these antibodies occasionally explain the clinical features of the syndrome, but direct evidence of their in vivo activity is poor. We studied a 58-year-old male with rheumatoid arthritis who presented with hypoglycemic coma. methods AND RESULTS: Antibodies were detected by inhibition of 125I-insulin binding to human insulin receptor-3t3 cells by the patient's serum. By immunofluorescence, they were immunoglobulin g of all four subclasses, immunoprecipitated insulin receptors from biotin-labeled cells, and triggered phosphorylation of the beta subunit of the insulin receptor. Insulin binding on the patient's red blood cells was markedly reduced. A biodistribution study after intravenous 123I-Tyr A14 insulin showed a marked inhibition of tracer uptake by the liver, reaching 10% of the injected dose (controls, mean /- SD, 21.1 /- 1.7%; n = 10). time activity curves generated on the liver and on the heart were parallel, with a T1/2 of 11.5 minutes for both, suggesting that no specific uptake occurred in the liver, where tracer activity represented only the blood pool. Clearance of insulin from the blood was indeed slower than in controls and mainly occurred through the kidneys. Analysis of plasma 123I-insulin immunoreactivity and trichloroacetic acid precipitate showed that insulin degradation did not occur as in normal controls. CONCLUSIONS: In this patient with hypoglycemic syndrome, insulin receptor antibodies with in vitro insulin-like activity are capable of blocking in vivo the access of insulin to the liver receptor compartment, as directly demonstrated by the markedly altered biodistribution of intravenously injected 123I-insulin.- - - - - - - - - - ranking = 0.48669315543655keywords = blood cell (Clic here for more details about this article) |
8/61. Successful treatment of thrombocytopenia and hemolytic anemia with IvIG in a patient with lupus-like syndrome after mismatched related PBSCT.hematopoietic stem cell transplantation (HSCT) is a treatment option for autoimmune diseases but can also cause clinical features similar to those of autoimmune diseases. In some of these cases the autoimmune-like condition is associated with autoimmune cytopenia, a complication that can be unresponsive to established treatment strategies and which may be fatal. The majority of cases reported on immune hemolytic anemia have been of alloimmune origin due to ABO red blood cell antigen incompatibilities between donor and recipient. We now report a patient with a lupus-like syndrome, presenting with severe thrombocytopenia and hemolytic anemia 9 months after HLA-mismatch, ABO compatible-related PBSCT who experienced no response to high-dose steroids, but who had a sustained response to repeated IvIG therapy.- - - - - - - - - - ranking = 0.48669315543655keywords = blood cell (Clic here for more details about this article) |
9/61. Autoimmune neutropenia with cyclic oscillation of neutrophil count after steroid administration.A 16-year-old female patient was evaluated for pancytopenia. She had a white blood cell count of 1.6 x 10(9)/L with 0.02 neutrophils and a platelet count of 19 x 10(9)/L. In the bone marrow, mature granulocytes were markedly decreased in number, but no atypical cells were present. Antineutrophil antibody was demonstrated by flow cytometry, and the level of platelet-associated immunoglobulin g was increased. A diagnosis of autoimmune neutropenia and thrombocytopenia was made. Interestingly, neutrophil and platelet counts fluctuated cyclically after the initiation of prednisolone therapy. The neutrophil count fluctuated between 0.1 x 10(9)/L and 7 x 10(9)/L, and the platelet count fluctuated between 19 x 10(9)/L and 175 x 10(9)/L, in 4-week cycles. Following splenectomy, neutrophil and platelet counts normalized. We believe the immune mechanism of recurrent neutropenia in this patient differs from that in other patients with cyclic neutropenia reported with stem cell disorders.- - - - - - - - - - ranking = 0.51330684456345keywords = blood cell, white (Clic here for more details about this article) |
10/61. Hypoglycaemia due to insulin autoimmune syndrome: report of two cases with characterisation of HLA alleles and insulin autoantibodies.OBJECTIVE: Insulin autoimmune syndrome (IAS) has been reported mainly in japan and so far only 27 IAS cases have been described from outside asia. We describe two unrelated Portuguese patients with IAS and characterise their insulin autoantibodies and HLA alleles. patients: Patient 1, a 24-year-old white female suffered an episode of unconsciousness in the late postprandial state and blood glucose was found to be 33 mg/dl with serum insulin levels of >3980 microIU/ml (normal range 0-30 microIU/ml). She was receiving monthly injections of penicillin g for the prophylaxis of recurrent tonsillitis. Patient 2, was a 19-year-old white female, with episodes of sweating, hand tremor, weakness and hunger occurring in the postprandial state and blood glucose levels during the attacks of 28-56 mg/dl. Very high insulin levels (602-708 microIU/ml) were present. methods AND RESULTS: Anti-insulin antibodies, determined by a semi-quantitative method, were strongly positive in both patients (91.7% in patient 1 and 88.6% in patient 2; normal range < or =7%). Sephadex G-100 chromatography of the sera showed most of insulin immunoreactivity present in the void volume which was retained by an affinity column with anti-human-immunoglobulin g antibodies (87% and 95% from patients 1 and 2 respectively). Scatchard plot analysis and molecular typing of the DRB1 gene revealed a polyclonal antibody and DRB1*0406 in patient 1, and a monoclonal antibody and DRB1*0403 in patient 2. CONCLUSIONS: These two Portuguese patients with IAS had different HLA-DR4 subtypes and insulin autoantibodies: DRB1*0406 and a polyclonal antibody in a patient treated with penicillin, and DRB1*0403 and a monoclonal antibody in a patient with "idiopathic" IAS.- - - - - - - - - - ranking = 0.053227378253802keywords = white (Clic here for more details about this article) |
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