1/11. 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 = 1keywords = physical (Clic here for more details about this article) |
2/11. 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 = 1keywords = physical (Clic here for more details about this article) |
3/11. 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 = 1keywords = physical (Clic here for more details about this article) |
4/11. 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 = 2keywords = physical (Clic here for more details about this article) |
5/11. Periodontal treatment in severe aplastic anemia.BACKGROUND: Aplastic anemia (AA) is a rare hematologic disease characterized by hypo-cellular bone marrow. The clinical features include fatigue, increased bruising, and gingival bleeding caused by anemia, leukopenia, and thrombocytopenia. A patient with AA is at high risk for infection because of leukopenia. The risk of systemic infection is especially high in AA patients with severe local infections, including periodontitis. Accordingly, periodontal treatment should include antibiotic prophylaxis to reduce the risk of systemic infection. However, treatment of periodontitis in the AA patient is significantly complicated by the bleeding disorder. We present a case report of the successful periodontal treatment of an AA patient with spontaneous gingival bleeding. methods: The patient was closely monitored for platelet and neutrophil counts before every treatment. The patient's platelet count was always under 10,000/microl. Therefore, it was necessary to increase platelet counts to over 25,000/microl by transfusion, after which subgingival scaling with anesthesia was performed. When the neutrophil count was less than 2,000/microl, local minocycline chemotherapy was applied to the pockets. Periodontal infection was monitored by detection of bacterial dna and measurement of serum immunoglobulin (Ig) G titer against periodontal bacteria. RESULTS: Following the physical and chemical treatment, the gingival appearance improved dramatically and the spontaneous gingival bleeding disappeared. Moreover, the IgG titer against periodontal bacteria decreased to normal range and specific periodontal pathogens were no longer detectable in the tested pockets. CONCLUSION: We believe that the treatment strategy in the present report provides new sight into treatment planning for severely medically compromised patients.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
6/11. Aplastic crisis in sickle cell disorders: bone marrow necrosis and human parvovirus infection.Aplastic crisis in patients with sickle cell disease who develop a parvovirus infection may be associated with extensive bone marrow necrosis as well as acute selective erythroblastopenia. This illness may be manifested by pyrexia, lymphadenopathy, bone tenderness and significant hypoxemia with minimal roentgenographic findings in the lungs. It is uncertain whether the hypoxemia is caused by the effects of the viral infection on the lungs or is secondary to sickling of red blood cells in the pulmonary vasculature or both. The hypoxia may be sufficiently severe to require treatment with both oxygen and transfusion. The physical damage to the bone marrow associated with bone marrow necrosis may be more important than selective acute erythroblastopenia in inducing aplastic crisis in patients with sickle cell disorders. Studies of bone marrow biopsy specimens collected during parvovirus-associated aplastic crisis in patients with nonsickle cell hemolytic disorders would be helpful in determining the pathophysiology of parvovirus-associated disorders.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
7/11. Is bone marrow examination justified in idiopathic thrombocytopenic purpura?bone marrow examination is widely accepted among pediatric hematologists as a mandatory investigation in childhood idiopathic thrombocytopenic purpura (ITP). The aim of this procedure is to confirm the presence of megakaryocytes and to exclude other conditions, such as leukemia and aplastic anemia. To assess the need for bone marrow examination, we reviewed the charts of 127 children with presumed ITP and found that bone marrow examination led to a different diagnosis in five (3.9%) of them. All five patients had presented with clinical and/or laboratory features atypical of acute ITP; none had leukemia. The initial clinical and laboratory findings of 50 patients with aplastic anemia also were reviewed; all had features atypical of acute ITP. Proper history and physical examination as well as a complete blood cell count are reliable means of recognizing patients with typical vs atypical features of ITP. Bone marrow aspiration could be limited safely to those patients with atypical features of ITP or to patients being treated with corticosteroids.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
8/11. Bone marrow imaging with 59Fe.Commerically available collimators are not suitable for the visual demonstration of the kinetics of 59Fe, because of its physical properties. A locally designed collimator, that provides integral visual information of the whole body distribution of erythropoietic marrow, is discussed. Wholebody scans of 4 individuals are also included to demonstrate the capabilities of the collimator.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
9/11. Cyclic hematopoiesis: human cyclic neutropenia.Human cyclic neutropenia is a relatively rare disorder of unknown etiology. Study of patients and animals with the disorder has led to important information regarding the differentiation of blood cells and control mechanisms of hematopoietic regulation. It has a world-wide distribution, occurs in both sexes, and, in about one-fourth of the patients, a family history has been obtained. While usually benign, deaths from overwhelming infections occur. In addition to cycling of neutrophils, in the majority of cases the monocytes cycle and in about one-fifth of the cases eosinophils are elevated. In a small number of patients, cycling of platelets and reticulocytes occurs. Cycles of colony stimulating factor are present. Cycles of bone marrow cells are easily demonstrable. The recent transfer of human cyclic neutropenia following allogenic bone marrow grafting confirms the hypothesis that the disorder is of bone marrow origin. The following subjects are covered in this review article: A. Definition, history, and incidence; B. Etiology, geographic distribution, mode of transmission; C. Symptoms, physical signs, diagnosis, clinical course; D. Clinical laboratory studies; E. Experimental studies; F. prognosis; G. Treatment. It is felt that human cyclic neutropenia represents a heterogeneous group of disorders and that much remains to be learned about its cause(s).- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
10/11. Aplastic anemia associated with rubber cement used by a marathon runner.Marathon runners are subject to many unique physical problems, especially blistering of the feet. Once the feet have become blistered, some runners use rubber cement, which contains benzene as an impurity, to keep adhesive tape in place over the denuded areas while running. We report the case of a marathon runner who used rubber cement in this fashion for more than one year in whom aplastic anemia developed.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
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