1/16. Fulminant parvovirus infection following erythropoietin treatment in a patient with acquired immunodeficiency syndrome.We report the case of a 41-year-old black man with acquired immunodeficiency syndrome who developed a severe chronic anemia due to parvovirus infection. Bone marrow biopsy revealed erythroid aplasia. The infectious nature of the anemia was not recognized, and the patient was treated with erythropoietin. The patient's reticulocyte response was inadequate, however, and he remained anemic. A second bone marrow biopsy showed erythroid hyperplasia and prominent intranuclear parvovirus inclusions within erythroid progenitors. erythropoietin was discontinued and was followed by a course of intravenous immunoglobulin, which resulted in rapid correction of anemia. To our knowledge, this is the first reported case of fulminant human parvovirus infection exacerbated by erythropoietin administration and documented by sequential bone marrow histologic examination. This case illustrates the critical importance of considering parvovirus in the etiology of chronic anemia with erythroid aplasia in immunocompromised patients.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
2/16. 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 = nature (Clic here for more details about this article) |
3/16. 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 = nature (Clic here for more details about this article) |
4/16. Limited heterogeneity of T cell receptor BV usage in aplastic anemia.Immune mediation of aplastic anemia (AA) has been inferred from clinical responsiveness to immunosuppressive therapies and a large body of circumstantial laboratory evidence. However, neither the immune response nor the nature of the antigens recognized has been well characterized. We established a large number of CD4 and CD8 T cell clones from a patient with AA and analyzed their T cell receptor (TCR) usage. Most CD4 clones displayed BV5, whereas most CD8 clones displayed BV13. We found sequence identity for complementarity determining region 3 (CDR3) among a majority of CD4 clones; the same sequence was present in marrow lymphocytes from four other patients with AA but was not detected in controls. The dominant CD4 clone showed a Th1 secretion pattern, lysed autologous CD34 cells, and inhibited their hematopoietic colony formation. In three of four patients, successful immunosuppressive treatment led to marked decrease in clones bearing the dominant CDR3 BV5 sequence. These results suggest surprisingly limited heterogeneity of the T cell repertoire in an individual patient and similarity at the molecular level of the likely pathological lymphocyte response among multiple patients with AA, consistent with recognition of limited numbers of antigens shared by individuals with the same HLA type in this disease.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
5/16. stem cell factor-induced bone marrow mast cell hyperplasia mimicking systemic mastocytosis (SM): histopathologic and morphologic evaluation with special reference to recently established SM-criteria.Although systemic mastocytosis (SM) is a well-defined hematologic neoplasm, it is sometimes difficult to discriminate between SM and a reactive mast cell (MC) hyperplasia. We describe a patient with aplastic anemia who was treated with recombinant stem cell factor (SCF). In response to SCF, the patient showed transient hematologic improvement and developed a marked increase in MC as well as a transient increase in serum tryptase. Histologic and immunohistochemical examination revealed a huge increase in MC in the bone marrow with focal infiltrates similar to SM. However, most of the SM-criteria were not met: First, MC showed normal cytomorphological characteristics without significant atypias (no cytoplasmic extensions, no oval nuclei, no hypogranulated cytoplasm). Furthermore, bone marrow MC were CD2- and CD25-negative and did not exhibit the C-KIT 2468 A-->T mutation (Asp-816-Val). After discontinuation of SCF the MC hyperplasia resolved confirming its reactive nature. Based on our case and similar cases mimicking mastocytosis, it seems of importance to apply recently established SM criteria in order to discriminate between reactive MC hyperplasia and true mastocytosis with certainty.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
6/16. Clonal remission in aplastic anemia after treatment with antithymocyte globulin.Aplastic anemia includes a group of disorders characterized by peripheral blood pancytopenia and marrow hypocellularity. The current report describes a patient who is an apparent constitutional mosaic and presented with marrow aplasia. Using cytogenetic analysis of bone marrow, skin, and peripheral T lymphocytes, we demonstrated the clonal nature of this patient's aplastic marrow, and, in addition, identify clonal evolution. The patient was treated with antithymocyte globulin (ATG) and achieved a complete remission, with disappearance of an abnormal evolved clone. This case illustrates that clonal cytogenetic abnormalities do not preclude a response to ATG and that aplastic anemia may be a nonmalignant clonal disorder with clonal evolution.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
7/16. Localized scleroderma-like lesions after bone marrow transplantation in man. A chronic graft versus host reaction.Localized scleroderma-like skin lesions which developed in two children, from 8 to 10 months after successful bone marrow transplantation for aplastic anaemia, showed histopathological features resembling those of scleroderma. This finding, like the animal models described in the literature, provides additional support for the auto-immune nature of scleroderma.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
8/16. Sweet's syndrome with acute leukemia.Sweet's syndrome presenting with malignancy or acute neutrophilic dermatosis is an unusual cutaneous disorder associated most commonly with acute myelogenous leukemia. Although other cancers may be linked to acute neutrophilic dermatosis, a large majority of patients have associated neoplasms of hematopoietic, plasma cell, or lymphoid nature. We report a patient with aplastic anemia who showed acute neutrophilic dermatosis and, ultimately, acute leukemia. The individual lesions responded to carbon dioxide laser surgery.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
9/16. Anabolic steroid therapy and intrahepatic cholangiocarcinoma.A 47-year-old man who had been treated with an anabolic steroid for refractory anemia developed an intrahepatic cholangiocarcinoma with metastases to abdominal lymph nodes and lungs. Microscopically, the tumor showed mucin production and was devoid of hepatocellular elements. Previous reports have suggested a possible relationship between anabolic steroid therapy and hepatocellular carcinoma. In many such cases, there are doubts about the histological diagnosis, malignant potential, and the nature of the association between the steroids and the tumors. The presence of distant metastases attests to the malignant nature of the tumor in the present case. Despite the apparent temporal eligibility of the steroid as an etiologic agent, a causative relationship between therapy and tumor is not established.- - - - - - - - - - ranking = 2keywords = nature (Clic here for more details about this article) |
10/16. heparin-like anticoagulant associated with systemic candidiasis.A 15 year old girl with aplastic anemia developed a heparin-like anticoagulant during the course of systemic candidiasis. This was initially detected in the laboratory by an elevation of the thrombin clotting time which corrected with toluidine blue but not by mixing with normal plasma. In vivo and in vitro the anticoagulant was remarkably resistant to neutralization by protamine sulfate. Nevertheless, its heparin-like nature was confirmed by its sensitivity to heparinase and its dependence on antithrombin iii.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
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