1/35. Multiple autoimmune haemopoietic disorders and insidious clonal proliferation of large granular lymphocytes.We report a patient with clonal proliferation of CD3 8 TCRalphabeta large granular lymphocytes (LGL) presenting multiple episodes of autoimmune cytopenia, including autoimmune neutropenia, idiopathic thrombocytopenic purpura, autoimmune haemolytic anaemia, and pure red cell aplasia. Each disorder appeared separately or as a combination during an 11-year clinical course. The increase of blood CD3 8 TCRalphabeta LGL was detected 6 years after the initial diagnosis of cytopenia, but the absolute number of LGL cells was always < 1.0 x 109/l. LGL cells were of monoclonal origin and had a chromosomal abnormality. LGL cells transiently responded to cyclosporine A therapy, which was also effective on all of these autoimmune cytopenias. Accordingly, an undetectable level of proliferation of a clonal LGL population could cause various autoimmune haemopoietic disorders.- - - - - - - - - - ranking = 1keywords = anaemia (Clic here for more details about this article) |
2/35. Acquired pure red cell aplasia--a case report.A married female patient of 36 years with chronic anaemia, because of pure erythroid aplasia with a haemolytic component and hypothyroidism due to antithyroid auto-antibodies, was subsequently discovered as a case of systemic lupus erythematosus (SLE). She was treated with corticosteroid and immunosuppressive therapy and her anaemia was corrected. The response of erythroid aplasia to corticosteroid and other immunosuppressive agents suggests that immunological factors play a role in erythroid aplasia in SLE. The occurrence of red cell aplasia in association with a variety of immune phenomenon supports the concept that in SLE, erythroid aplasia may be of immune aetiology.- - - - - - - - - - ranking = 2keywords = anaemia (Clic here for more details about this article) |
3/35. Acquired haemophilia in association with organ-specific autoimmune disease.A 72-year-old woman presented with multiple sites of soft tissue bleeding and a left ulnar neuropathy due to a compartment syndrome. There were also clinical signs of hypothyroidism. factor viii:C (FVIII) levels were 6% and a FVIII inhibitor with a titre of 9 Bethesda units was detected. She had biochemical evidence of hypothyroidism and high titres of antithyroid antibodies were detected. Two episodes of life- or limb-threatening haemorrhage were successfully treated with FEIBA. The inhibitor disappeared following 2 months of immunosuppression with oral cyclophosphamide and corticosteroids. She then developed autoimmune haemolytic anaemia, which responded to further immunosuppression with oral prednisolone. The association between acquired haemophilia and systemic autoimmune disorders such as systemic lupus erythematosis is well recognized, but our description of an association with organ-specific autoimmunity is more unusual.- - - - - - - - - - ranking = 1keywords = anaemia (Clic here for more details about this article) |
4/35. Idiopathic heart block: association with vitiligo, thyroid disease, pernicious anaemia, and diabetes mellitus.Out of 100 patients with chronic heart block 16 had one or more autoimmune disorders-namely, vitiligo (5,) hypothyroidism (4), Graves's disease (1), pernicious anaemia (2), and diabetes mellitus (9). All these disorders occurred with greater frequency than normal and were more prevalent than in a group of hospital inpatients of comparable age. autoantibodies were not increased. We suggest that among patients with idiopathic heart block there is a subgroup with multiple autoimmune disorders.- - - - - - - - - - ranking = 5keywords = anaemia (Clic here for more details about this article) |
5/35. Treatment of refractory antibody mediated autoimmune disorders with an anti-CD20 monoclonal antibody (rituximab).BACKGROUND: Rituximab, a chimeric monoclonal anti-CD20 antibody, has recently been used for the treatment of refractory antibody mediated autoimmune diseases such as immune mediated thrombocytopenia and haemolytic anaemia. PATIENTS: Because of its novel mechanism of action, rituximab was used to treat three patients with refractory systemic antibody mediated autoimmune disorders. The first patient, a 71 year old woman with idiopathic type II mixed essential cryoglobulinaemia, had both dermatological and neurological manifestations with marked renal disease attributed to her cryoglobulinaemia. Patient 2, a 73 year old woman with Goodpasture's syndrome, was refractory to conventional treatment (cyclophosphamide, prednisone, plasmapheresis). She had persistent haemoptysis and haematuria and positive antiglomerular basement membrane antibodies. The third patient, a 75 year old man with primary biliary cirrhosis, myelodysplasia, and systemic immune complex vasculitis, had progressive renal insufficiency, a macular erythematous rash, and severe thrombocytopenia. RESULTS: Treatment with rituximab resolved all clinical and laboratory manifestations in the three patients. CONCLUSIONS: Rituximab may be an important therapeutic agent for the treatment of patients refractory or intolerant to corticosteroid or cytotoxic treatment, or both.- - - - - - - - - - ranking = 1keywords = anaemia (Clic here for more details about this article) |
6/35. Coeliac disease and autoimmune cholangitis: a case report.Autoimmune cholangitis can be associated with other autoimmune disorders. The case is described of a 58-ear-old female who developed severe microcytic anaemia resistant to oral iron treatment. Evaluation of the patient led to the diagnosis of coeliac disease, a rarely described association. Gluten-free diet and treatment with oral haematinics led to reversal of the anaemia.- - - - - - - - - - ranking = 2keywords = anaemia (Clic here for more details about this article) |
7/35. Mycophenolate mofetil in the treatment of autoimmune HCV-associated haematological disorders showing steroid resistance or dependence.We report two cases of hepatitis c virus (HCV) associated autoimmune haematological disorders successfully treated with an unusual protocol (mycophenolate mofetil: MMF). The first case was a male patient with chronic HCV infection who developed, during interferon (IFN)/ribavirin therapy, severe autoimmune thrombocytopenia unresponsive to steroids. MMF was then administered and, simultaneously, the steroid dose was gradually reduced until withdrawal. Following this strategy, a progressive increase in platelet count and complete negativity of anti-PLT antibodies were achieved without changes in HCV-rna quantitative determination. The second case was a woman with HCV liver cirrhosis with severe anaemia and coombs test positivity partially responsive to continuous administration of steroid high doses. However, this treatment unmasked a severely painful vertebral osteoporosis. For this reason we introduced MMF and simultaneously steroid therapy was progressively reduced until withdrawal. Haemoglobin reached a normal value and the coombs test became negative within 60 days. These case reports suggest that MMF may represent an interesting therapeutic approach for autoimmune HCV associated haematological disorders.- - - - - - - - - - ranking = 1keywords = anaemia (Clic here for more details about this article) |
8/35. Pernicious anaemia in Africans.Ten cases of pernicious anaemia seen over a 15-year period (1973-1988) in a Lagos hospital are presented. Their ages ranged from 34 to 67 with a mean of 53.6 years. Females outnumbered males 6 to 4. Complications seen include gastric carcinoma, myelopathy, peripheral neuropathy, skin hyperpigmentation, hair depigmentation and diarrhoea. Reluctance to consider the diagnosis owing to firmly held notions of its rarity and a penchant for empirically treating chronic anaemias with all available haematinics and blood transfusion are probably contributory to its underdiagnosis. The fact that seven of the patients presented were seen in the last three years and three of them in the last one year raises the possibility of an increasing incidence of pernicious anaemia in Africans. The disease may be much less rare in Africans than once believed, and medical education should emphasize its existence and advocate greater care in the management of chronic anaemias.- - - - - - - - - - ranking = 8keywords = anaemia (Clic here for more details about this article) |
9/35. Two potential mechanisms of oxaliplatin-induced haemolytic anaemia in a single patient.Only two patients with adequately documented oxaliplatin-induced autoimmune haemolytic anaemia have previously been reported. We report here a third patient with an immune-haematological finding favouring a bispecific mechanism (immune-complex and penicillin type) of oxaliplatin-induced haemolysis.- - - - - - - - - - ranking = 5keywords = anaemia (Clic here for more details about this article) |
10/35. Guillain Barre syndrome precipitated by the use of antilymphocyte globulin in the treatment of severe aplastic anaemia.This report describes the case of a 54 year old woman with very severe aplastic anaemia who was treated with antilymphocyte globulin (ALG) and developed Guillain Barre syndrome (GBS). No antecedent infective aetiology was identified. Although there are numerous reports of autoimmune disease after treatment with ALG in aplastic anaemia, and GBS after immunosuppressive treatment, there are none reporting GBS after the use of ALG for severe aplastic anaemia. The occurrence of autoimmune disease after immunosuppressive treatment, in particular ALG, is discussed, together with the possible mechanisms that result from T cell depression.- - - - - - - - - - ranking = 7keywords = anaemia (Clic here for more details about this article) |
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