1/65. Elevated reticulocyte count--a clue to the diagnosis of haemolytic-uraemic syndrome (HUS) associated with gemcitabine therapy for metastatic duodenal papillary carcinoma: a case report.In adults, the haemolytic-uraemic syndrome (HUS) is associated with probable causative factors in the minority of all cases. Cytotoxic drugs are one of these potential causative agents. Although metastatic cancer by itself is a recognized risk-factor for the development of HUS, therapy with mitomycin-C, with cis-platinum, and with bleomycin carries a significant, albeit extremely small, risk for the development of HUS, compared with all other cytotoxic drugs. Gemcitabine is a novel cytotoxic drug with promising activity against pancreatic adenocarcinoma. We are reporting on one patient with metastatic duodenal papillary carcinoma developing HUS while on weekly gemcitabine therapy. The presenting features in this patient were non-cardiac pulmonary oedema, renal failure, thrombocytopenia and haemolytic anaemia. The diagnosis of HUS was made on the day of admission of the patient to this institution. Upon aggressive therapy, including one single haemodialysis and five plasmaphereses, the patient recovered uneventfully, with modestly elevated creatinine-values as a remnant of the acute illness. Re-exposure to gemcitabine 6 months after the episode of HUS instituted for progressive carcinoma, thus far has not caused another episode of HUS.- - - - - - - - - - ranking = 1keywords = anaemia (Clic here for more details about this article) |
2/65. Anaemia, thrombocytopenia and coagulopathy due to occult diffuse infantile haemangiomatosis of spleen and pancreas.Diffuse infantile haemangiomatosis of the spleen is a very rare lesion. Large haemangiomas may cause trapping of platelets and coagulation disorders known as Kasabach-Merrit syndrome. We here report the case of an infant with splenic and pancreatic haemangiomatosis presenting with life-threatening thrombocytopenia, anaemia and intravascular coagulation. diagnosis was hampered by reactive erythroblastosis and non-conclusive radiological findings. While treatment with corticosteroids was ineffective, administration of antithrombin iii improved coagulation parameters. After splenectomy the child recovered promptly and has remained free of disease for 3 years to date. CONCLUSION: Occult visceral haemangiomatosis without visible cutaneous haemangiomas should be included in the differential diagnosis of thrombocytopenia, anaemia and consumption coagulopathy. antithrombin iii treatment may be considered to overcome bleeding problems in patients with Kasabach-Merrit syndrome.- - - - - - - - - - ranking = 2keywords = anaemia (Clic here for more details about this article) |
3/65. Management of severe and complicated malaria in the intensive care unit.malaria remains today one of the major health problems in the tropics with increased morbidity and mortality. The most serious complications are caused by plasmodium falciparum, which, in contrast to the benign malarias, may progress to a life-threatening multi-system disease. Our case concerns a young woman in the 14th week of pregnancy, admitted to the ICU in a coma, with pulmonary oedema, haemolytic anaemia, renal failure and thrombocytopenia as complications of P. falciparum malaria. The case is discussed and possible explanations for the clinical picture and complications of P. falciparum malaria are given in the light of experiences from the literature.- - - - - - - - - - ranking = 1keywords = anaemia (Clic here for more details about this article) |
4/65. 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) |
5/65. Excessive apoptosis of bone marrow erythroblasts in a patient with autoimmune haemolytic anaemia with reticulocytopenia.We report a patient with autoimmune haemolytic anaemia (AIHA) with reticulocytopenia, who showed excessive apoptosis of erythroblasts. Ultrastructural analysis of bone marrow cells showed that 50% of erythroblasts had characteristic features of apoptosis, which was confirmed by staining with Annexin-V. In addition, in contrast to normal erythroblasts, Ig staining of the entire erythroblast population could be shown. These data show that apoptosis may contribute to the mechanism of reticulocytopenia in AIHA.- - - - - - - - - - ranking = 5keywords = anaemia (Clic here for more details about this article) |
6/65. Familial dyserythropoietic anaemia and thrombocytopenia due to an inherited mutation in GATA1.Haematopoietic development is regulated by nuclear protein complexes that coordinate lineage-specific patterns of gene expression. Targeted mutagenesis in embryonic stem cells and mice has revealed roles for the X-linked gene Gata1 in erythrocyte and megakaryocyte differentiation. GATA-1 is the founding member of a family of dna-binding proteins that recognize the motif WGATAR through a conserved multifunctional domain consisting of two C4-type zinc fingers. Here we describe a family with X-linked dyserythropoietic anaemia and thrombocytopenia due to a substitution of methionine for valine at amino acid 205 of GATA-1. This highly conserved valine is necessary for interaction of the amino-terminal zinc finger of GATA-1 with its essential cofactor, FOG-1 (for friend of GATA-1; refs 9-12). We show that the V205M mutation abrogates the interaction between Gata-1 and Fog-1, inhibiting the ability of Gata-1 to rescue erythroid differentiation in an erythroid cell line deficient for Gata-1 (G1E). Our findings underscore the importance of FOG-1:Gata-1 associations in both megakaryocyte and erythroid development, and suggest that other X-linked anaemias or thrombocytopenias may be caused by defects in GATA1.- - - - - - - - - - ranking = 6keywords = anaemia (Clic here for more details about this article) |
7/65. Relapsing polychondritis and myelodysplasia: a report of two cases and review of the current literature.Relapsing polychondritis (RP) is a rare multisystem disorder. We describe two case reports of patients with RP, one of whom developed myelodysplasia subtype refractory anaemia (RA) and the other, refractory anaemia with ringed sideroblasts (RARS). We also review the literature of association between RP and haematological disorders.- - - - - - - - - - ranking = 2keywords = anaemia (Clic here for more details about this article) |
8/65. splenectomy for refractory Evans' syndrome associated with antiphospholipid antibodies: report of two cases.The main haematological manifestations seen in patients with antiphospholipid antibodies (aPL) are thrombocytopenia, usually mild, and haemolytic anaemia with a positive coombs test. Owing to the shared characteristics with idiopathic thrombocytopenic purpura, similar rules are followed in the treatment of these cytopenias. Two patients with severe aPL associated cytopenias, who required splenectomy after being refractory to steroids, immunosuppressive agents, and other treatments (intravenous gammaglobulin, danazol), are described, and previously reported cases are reviewed.- - - - - - - - - - ranking = 1keywords = anaemia (Clic here for more details about this article) |
9/65. Severe epistaxis in brucellosis-induced isolated thrombocytopenia: a report of two cases.brucellosis can present initially with its haematological findings including anaemia, leukopenia, and thrombocytopenia and may mimic primary haematological diseases. We present two patients with complaints of severe epistaxis and isolated thrombocytopenia which was initially diagnosed as idiopathic thrombocytopenic purpura but which was finally attributed to brucellosis. Their platelet count reverted to normal within 2-3 weeks of initiating antibrucellosis treatment with recovery from the disease.- - - - - - - - - - ranking = 1keywords = anaemia (Clic here for more details about this article) |
10/65. Congenital thrombocytopenia and radio-ulnar synostosis: a new familial syndrome.The association of bone marrow failure and skeletal defects has been frequently noted, however, the genetic basis for most of these syndromes remains unclear. We describe a previously uncharacterized autosomal dominant syndrome of amegakaryocytic thrombocytopenia associated with radial-ulnar synostosis. The clinical features of this syndrome appear to be distinct from other similar conditions, including Fanconi's anaemia and thrombocytopenia-absent radii (TAR). The physical findings at diagnosis and clinical management of each case are detailed, as well as a discussion of this disorder in the context of other syndromes in which marrow failure and skeletal defects are prominent features. We also review recent developments in molecular genetics that may provide important clues to the underlying aetiology of this condition.- - - - - - - - - - ranking = 1keywords = anaemia (Clic here for more details about this article) |
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