1/19. Autoimmune thrombocytopenia: a complication of fludarabine therapy in lymphoproliferative disorders.We describe two cases of autoimmune thrombocytopenia precipitated by fludarabine therapy in patients with chronic lymphatic leukaemia. Both were treated with high dose steroids and initially responded with recovery of normal platelet counts. One patient developed recurrent autoimmune thrombocytopenia on two occasions following re-exposure to the drug when his disease had become refractory to all other treatments. A retrospective review of the case notes of 45 patients with lymphoproliferative disorders treated with fludarabine over the past 6 years indicated the development of autoimmune thrombocytopenia in 4.5% (two out of 45) and autoimmune haemolytic anaemia in 6.7% (three of the 45).- - - - - - - - - - ranking = 1keywords = anaemia (Clic here for more details about this article) |
2/19. Immune cytopenias as the presenting finding in primary sjogren's syndrome.A diagnostic delay of several years in primary sjogren's syndrome is common, even in patients who present with sicca symptoms. It is much more likely in cases with prominent symptomatic extraglandular involvement. We report on three such patients who presented as Coomb's positive haemolytic anaemia, systemic symptoms with agranulocytosis and gingival bleeding due to immune thrombocytopenia, to alert clinicians to the fact that primary sjogren's syndrome may present as clinically significant immune-mediated cytopenia in the absence of sicca symptoms. sjogren's syndrome, a common autoimmune disorder, should be considered in the differential diagnosis of apparently 'idiopathic' cytopenias and actively sought by directed history, Schirmer test and autoantibody screening.- - - - - - - - - - ranking = 1keywords = anaemia (Clic here for more details about this article) |
3/19. thrombocytopenia in systemic-onset juvenile chronic arthritis: report of two cases with unusual bone marrow features.Two children with systemic-onset juvenile chronic arthritis (S-JCA) suffering from severe, symptomatic thrombocytopenia are reported. Case 1 is a 10-year-old girl who had fever, chronic polyarthritis and hepatosplenomegaly for 6 months and severe gum bleeding and generalised petechiae for 1 week. Immune thrombocytopenic purpura (ITP) was indicated by bone marrow findings of megakaryocytic hyperplasia, and her thrombocytopenia responded well to steroid therapy without recurrence, but progressive, deforming polyarthritis with anaemia and thrombocytosis persisted. Case 2 is a 7-year-old girl who presented with spiking fever, arthritis and skin rashes and was suspected of having S-JCA. She developed a severe nosebleed with impending shock while receiving full-dose aspirin. pancytopenia and elevated liver enzymes were also noted. Bone marrow aspiration revealed erythroid hyperplasia, maturation arrest of myeloid series with increased blasts, and adequate megakaryocytes. Her thrombocytopenia resolved in 1 week without specific treatment. Her subsequent clinical course confirmed the diagnosis of S-JCA, while thrombocytopenia did not recur during follow-up.- - - - - - - - - - ranking = 1keywords = anaemia (Clic here for more details about this article) |
4/19. Induction of remission with intravenous immunoglobulin and cyclophosphamide in steroid-resistant Evans' syndrome associated with dermatomyositis.Evans' syndrome is characterised by the simultaneous or sequential occurrence of Coombs'-positive haemolytic anaemia (AIHA) and immune thrombocytopenia without underlying aetiology. It has been found to be associated with collagen vascular diseases, especially systemic lupus erythematosus (SLE) and scleroderma. However, Evans' syndrome with dermatomyositis is very rare. A 59-year-old woman, who had been taking high-dose prednisolone for a month and cyclosporin for 10 days for dermatomyositis, developed purpura on the left popliteal fossa. The platelet and haemoglobin levels decreased to 77,000/mm3 and 9.8 g/dl, respectively. Antiplatelet antibody was positive. thrombocytopenia responded to intravenous immunoglobulin (IVIG) for a short time, but further decreased in a week. Her blood film showed features of haemolytic anaemia. Laboratory findings showed reticulocytosis and a positive direct Coombs' test. bone marrow examination showed a mild hyperplasia of erythroid precursors and megakaryocytes. The patient was successfully treated with cyclophosphamide in addition to oral prednisolone. AIHA in connective tissue disease may develop gradually and show a benign clinical course in most patients. Therefore, we suggest that patients with dermatomyositis and anaemia should always be checked for haemolysis if there is no other explanation.- - - - - - - - - - ranking = 3keywords = anaemia (Clic here for more details about this article) |
5/19. Autoimmune thrombocytopenia associated with the first cycle of fludarabine therapy in the treatment of relapsed non-Hodgkin's lymphoma.Fludarabine phosphate is a purine analogue now commonly used in the treatment of low-grade lymphoid malignancies. An increased incidence of autoimmune haemolytic anaemia is reported with the use of fludarabine for the treatment of chronic lymphocytic leukaemia (CLL). CLL already confers a high risk of autoimmune disorders and, although these are recognized in non-Hodgkiin's lymphoma (NHL), they are less common. Immune thrombocytopenia occurring in patients with CLL treated with fludarabine has been reported and we describe a further case in a patient with relapsed NHL. Possible mechanisms of the effect of fludarabine on autoimmune disorders are discussed.- - - - - - - - - - ranking = 1keywords = anaemia (Clic here for more details about this article) |
6/19. Treatment of life-threatening post-haemorrhagic anaemia with cell-free haemoglobin solution in an adolescent Jehovah's Witness.Severe blood loss carries an increased risk of a fatal outcome in patients who do not consent to the use of blood products. For patients who base this decision on a religious belief system, such as the jehovah's witnesses, important distinctions may exist between the appropriateness of various blood products. We describe the first successful use of purified human cell-free haemoglobin solution in a paediatric patient, in this case in the management of life-threatening anaemia in a 14-year-old female Jehovah's Witness with immune thrombocytopenia.- - - - - - - - - - ranking = 5keywords = anaemia (Clic here for more details about this article) |
7/19. Refractory Evans' syndrome treated with allogeneic SCT followed by DLI. Demonstration of a graft-versus-autoimmunity effect.Evans' syndrome, a combination of autoimmune haemolytic anaemia and autoimmune (idiopathic) thrombocytopenic purpura, is generally harder to treat and more refractory than the single entities. In a male patient with refractory disease, predominantly thrombocytopenic, an allogeneic reduced intensity BMT from his human leukocyte antigen (HLA)-identical sister was followed by a dramatic platelet peak while he was still experiencing initial engraftment (presumably of autologous origin), but subsequently by progressive relapse associated with mixed chimerism. Five gradually incremental DLI achieved complete donor chimerism, which was associated not only with grade II graft-versus-host disease (GVHD), but also with complete clinical and biological remission for 2 years post-transplant. Long-term FU is necessary before claiming that allogeneic stem cell transplantation (SCT) is capable of curing an autoimmune blood disease. However, there is evidence for a graft-versus-autoimmunity effect in this case.- - - - - - - - - - ranking = 1keywords = anaemia (Clic here for more details about this article) |
8/19. Double relapse of incomplete form of thrombotic thrombocytopenic purpura.BACKGROUND: Although several etiologies can be identified in thrombotic thrompocytopenic purpura (TTP), idiopathic cases are still frequent. Incomplete forms are rare and the diagnosis should be suspected in case of thrombocytopenia and microangiopathic haemolytic anaemia. Relapses are frequent in the complete classic form but rarely reported in incomplete forms. According to the literature, mortality and morbidity are significantly improved with plasma exchange (PE). Nevertheless, the management and treatment of relapses remain problematic. CASE REPORT: A 35-year-old woman presented with a double relapse of an incomplete TTP form in the second (24 months) and the fourth year (40 months) after the initial episode. The patient underwent plasma infusion, PE, and was then started on corticosteroids. She also received antiplatelet agents. The main biological indicators were platelet count, haematocrit, lactate dehydrogenase level and schistocytes. Following this treatment, the patient's condition rapidly improved. CONCLUSION: In this incomplete form of TTP, two relapses occurred, with the same presentation. Standard therapy was effective in this case.- - - - - - - - - - ranking = 1keywords = anaemia (Clic here for more details about this article) |
9/19. Development of common variable immunodeficiency in a patient with Evans syndrome treated by autologous stem cell transplantation.We describe a case report of a patient who developed common variable immunodeficiency (CVID) after autologous haematopoietic stem cell transplantation (SCT) for recurrent Evans syndrome. The disease manifested as attacks of haemolytic anaemia, thrombocytopenia and neutropenia from the age of 12 years. Presence of autoantibodies to blood elements was confirmed together with C4 deficiency. The patient also suffered from dermatitis herpetiformis Duhring without signs of coeliac disease. Autologous T cell-depleted peripheral blood stem cell (PBSC) transplant following conditioning regimen was performed at the age of 20 years. Immunological reconstitution was incomplete and 2 years after SCT he fulfilled laboratory criteria for common variable immunodeficiency (CVID). The patient was found to be a carrier of a risk haplotype for development of CVID DRB1*03/DQB1*0201. We conclude that T cell-depleted SCT here performed for autoimmune manifestations can hasten development of CVID in genetically predisposed patients.- - - - - - - - - - ranking = 1keywords = anaemia (Clic here for more details about this article) |
10/19. Oesophageal carcinoma and refractory idiopathic thrombocytopenic purpura: a challenging combination.We report the case of a 72-year-old lady who presented with fatigue and bruising. Initial investigations were compatible with iron deficiency anaemia and idiopathic thrombocytopenic purpura (ITP). The anaemia and recent heartburn symptoms led to endoscopy, which revealed an extensive oesophageal squamous cell carcinoma. The tumour was inoperable due to local extension and the patient was not deemed fit enough for aggressive chemotherapy. A course of radical local radiotherapy was given. The patient's ITP, which had initially been responsive to high-dose intravenous immunoglobulin, subsequently became refractory to all therapeutic modalities. The patient developed significant dysphagia but therapeutic options were limited due to her severe thrombocytopenia. The patient eventually died from a combination of gastrointestinal and intrapulmonary haemorrhage. This case is of interest for two reasons. First, the development of refractory ITP appeared to mirror the progression of the carcinoma, and to our knowledge this is the first case of ITP associated with oesophageal carcinoma. Second, despite recent endoscopic advances in palliating oesophageal tumours, this case highlights the difficulties that can still occur.- - - - - - - - - - ranking = 2keywords = anaemia (Clic here for more details about this article) |
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