1/32. colchicine-induced bone marrow suppression: treatment with granulocyte colony-stimulating factor.bone marrow aplasia is a frequent complication of colchicine poisoning. This typically occurs on day 3 to 5 postexposure, and the blood cell counts remain depressed for a week or more. Unfortunately, because patients suffering from colchicine toxicity develop multiple organ complications and sepsis, the morbidity and mortality associated with bone marrow depression is high. In this article, we present three cases of colchicine toxicity in which granulocyte colony-stimulating factor (G-CSF) was used to treat bone marrow depression. In all three cases, there was a dramatic increase in the white cell count and, to a lesser extent, the platelet count. In view of the critical nature of the bone marrow depression and multi-organ toxicity induced by colchicine, we believe that consideration of the use of G-CSF to shorten the duration of neutropenia is warranted.- - - - - - - - - - ranking = 1keywords = suffering (Clic here for more details about this article) |
2/32. Asplenia in a patient with Fanconi's anemia-like congenital aplastic anemia.Fanconi's anemia (FA) is an autosomal recessive disease manifested by pancytopenia resulting from bone marrow failure, variable physical anomalies and cancer susceptibility. A seven-year-old girl with Fanconi's anemia-like congenital aplastic anemia and concurrent asplenia without the congenital heart defects or the abdominal heterotaxia is reported. Asplenia was indicated using denatured red cells labelled with 51Cr, abdominal ultrasonography and computerized tomography. Immunological studies showed immunoglobulins (IgG, IgA, IgM), C3 and C4 levels within normal limits and the percentage of CD3, and C4 cells and the CD4/CD8 ratio decreased. The patient had not been exposed to recurrent pneumococcal infections. We think that isolated asplenia may occur in patients with Fanconi's anemia-like congenital aplastic anemia without the congenital heart diseases or abdominal heterotaxia.- - - - - - - - - - ranking = 0.15623672800899keywords = physical (Clic here for more details about this article) |
3/32. Acute intravascular hemolysis in two patients transfused with dry-platelet units obtained from the same ABO incompatible donor.Since 1989 we have been collecting dry-platelets on a routine basis. Dry-platelets are those collected along with 25-30 ml of contaminating plasma cell with separators such as the Amicus, AS 104 and the Excel Pro. Platelets are resuspended in non plasma media for storage and for at least 60 hours their viability and functionality are not impaired. In this article we report on two hemolytic crises determined by two O Rh D units of single donor platelets (SPD) taken from the same donor in a double-apheresis session. The two split units were administered to two A Rh D patients suffering from metastatic breast cancer and severe aplastic anemia (SAA) respectively. In both cases the hemolytic reaction was of the intravascular type, with a drop in hemoglobin (Hgb) level from 8.6 to 5.4 and from 8.8 down to 5.3 g/dl respectively. From the patients' RBC only alpha agglutinins were eluted and donor's indirect antiglobulin test (IAT) was negative with extended panel RBCs. In the first case the clinical course after erythroexchange (Erex) was uneventful whereas in the second one, that suffering from SAA, after Erex, acute renal failure and shock did complicate the clinical course and the patient died seven days after the incriminated platelet transfusion.- - - - - - - - - - ranking = 2keywords = suffering (Clic here for more details about this article) |
4/32. umbilical cord blood transplantation from unrelated HLA-matched donor in an adult with severe aplastic anemia.A 23-year-old male suffering from severe aplastic anemia (SAA) weighing 60 kg was successfully treated by unrelated allo-CBSCT (cord blood stem cell transplantation). A six-loci HLA-identical umbilical cord blood (UCB) was infused after conditioning with low-dose cyclophosphamide (CTX) and antilymphocyte globulin (ALG). The prophylaxis of GVHD consisted of CsA and MTX. The infused cord blood provided 1.89 x 10(7) nucleated cells per kg, CD34-positive cells: 0.89%. neutrophils >0.5 x 10(9)/l were reached 10 days after transplant, and platelets greater than 50.0 x 10(9)/l at day 26. RBC and platelet transfusion independence were reached on days 15 and 18. The patient developed grade 1 skin GVHD 10 months after engraftment of the donor cells. Microsatellite dna fingerprinting indicated a stable and persistent donor-recipient mixed chimerism, whilst the circulating red cells remain of host origin.- - - - - - - - - - ranking = 1keywords = suffering (Clic here for more details about this article) |
5/32. Normocytic anemia.anemia is a common problem that is often discovered on routine laboratory tests. Its prevalence increases with age, reaching 44 percent in men older than 85 years. Normocytic anemia is the most frequently encountered type of anemia. anemia of chronic disease, the most common normocytic anemia, is found in 6 percent of adult patients hospitalized by family physicians. The goals of evaluation and management are to make an accurate and efficient diagnosis, avoid unnecessary testing, correct underlying treatable causes and ameliorate symptoms when necessary. The evaluation begins with a thorough history and a careful physical examination. Basic diagnostic studies include the red blood cell distribution width, corrected reticulocyte index and peripheral blood smear; further testing is guided by the results of these studies. Treatment should be directed at correcting the underlying cause of the anemia. A recent advance in treatment is the use of recombinant human erythropoietin.- - - - - - - - - - ranking = 0.15623672800899keywords = physical (Clic here for more details about this article) |
6/32. Stem cell transplantation for paroxysmal nocturnal haemoglobinuria in childhood.Paroxysmal nocturnal haemoglobinuria (PNH) is a clonal haematopoietic disorder characterized by chronic or intermittent intravascular haemolysis, variable cytopenia and an increased risk of thrombosis. Stem cell transplantation (SCT) is a curative therapeutic option, but its risks must be carefully weighed against the natural course of PNH. World-wide experience with SCT for PNH in the paediatric age group is scarce. We report on two adolescents suffering from PNH with life-threatening complications who were successfully transplanted from unrelated donors. Indications and techniques of SCT in childhood PNH are discussed and an overview of the literature is given.- - - - - - - - - - ranking = 1keywords = suffering (Clic here for more details about this article) |
7/32. Recovery of normal hematopoiesis after severe bone marrow aplasia induced by interferon-alpha in a patient with chronic myelogenous leukemia.We describe an interesting case of a patient with chronic myelogenous leukemia (CML) who developed sustained severe bone marrow aplasia after 2 years and 11 months of interferon-alpha (IFN-alpha) therapy but demonstrated recovery of normal hematopoiesis when treated with immunosuppressive therapy with granulocyte-colony stimulating factor (G-CSF). Administration of G-CSF resulted in a partial recovery of hematopoiesis, and after starting immunosuppressive therapy, the patient was no longer dependent on blood transfusions. Moreover, her bone marrow had no philadelphia chromosome-positive clones. According to the results of the present case, bone marrow recovery can be achieved with immunosuppressive therapy and a fatal outcome avoided, even in CML patients suffering from sustained bone marrow aplasia during IFN-alpha treatment.- - - - - - - - - - ranking = 1keywords = suffering (Clic here for more details about this article) |
8/32. Membranous nephropathy after allogeneic hematopoietic stem cell transplantation in a patient with aplastic anemia: a case report.nephrotic syndrome has been described as one of the clinical forms of chronic graft-versus-host disease (cGVHD), but a limited number of cases have been described. We experienced a young female patient with nephrotic syndrome developed 22 months after allogeneic hematopoietic stem cell transplantation (HSCT) for severe aplastic anemia. She had been well after successful management for gut-limited cGVHD until she developed a clinical nephrotic syndrome with hypoalbuminemia of 2.0 g/dL and 24-hr urine protein of 6.88 g/dL. On physical examination and laboratory findings, there was no other evidence of cGVHD. Clinical and renal biopsy findings were consistent with cGVHD-related membranous nephropathy, and immunosuppressive agents with cyclosporine and prednisone were prescribed. After 3 month of treatment, the proteinuria decreased to normal range; and the patient from nephrotic syndrome nearly recovered. We recommend cGVHD-related glomerulonephritis should be considered in all patients with hypoalbuminemia following allogeneic HSCT, even if there is no other evidence of clinical GVHD.- - - - - - - - - - ranking = 0.15623672800899keywords = physical (Clic here for more details about this article) |
9/32. Aplastic crisis due to human parvovirus B19 infection in hereditary hemolytic anaemia.Specific anti-B19 IgM was demonstrated in sera from three children showing transient aplastic crisis. A two years-old boy living in Rio de Janeiro suffering from sickle-cell anaemia showed the crisis during August, 1990. Two siblings living in Santa Maria, RS, developed aplastic crisis during May, 1991, when they were also diagnosed for hereditary spherocytosis. For a third child from this same family, who first developed aplastic crisis no IgM anti-B19 was detected in her sera.- - - - - - - - - - ranking = 1keywords = suffering (Clic here for more details about this article) |
10/32. Late presentation of dyskeratosis congenita as apparently acquired aplastic anaemia due to mutations in telomerase rna.Aplastic anaemia in adults is usually acquired, but rarely constitutional types of bone marrow failure can occur late in life. We assessed two families with onset of pancytopenia in adults and detected two novel point mutations in the telomerase rna gene (TERC) in each family. This gene is abnormal in some kindreds with dyskeratosis congenita. Individuals in our families with mutated TERC did not have physical signs of dyskeratosis congenita, and their blood counts were nearly normal, but all had severely shortened telomeres, reduced haemopoietic function, and raised serum erythropoietin and thrombopoietin. bone marrow failure of variable severity due to dyskeratosis congenita, historically characterised by associated physical anomalies and early pancytopenia, may be present in otherwise phenotypically normal adults, and can masquerade as acquired aplastic anaemia.- - - - - - - - - - ranking = 0.31247345601798keywords = physical (Clic here for more details about this article) |
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