1/223. The significance of lymphocytosis in congenital hypoplastic anemia.Two infants with congenital hypoplastic anemia had an unusual number of lymphocytes in their peripheral blood and in the bone marrow. This caused an erroneous diagnosis of acute lymphocytic leukemia to be made in the first case and inappropriate therapy to be administered for three months. The second of these cases provided an unusual opportunity to study human erythrocyte precursor dynamics. Serial bone marrow aspirates, obtained after institution of treatment with a corticosteroid, revealed an initial increase in labeled lymphocytes, a concomitant decrease in their number, and the subsequent appearance of erythroid elements. These findings suggest that cells classified morphologically as lymphocytes may serve as erythroid precursors in human beings.- - - - - - - - - - ranking = 1keywords = leukemia (Clic here for more details about this article) |
2/223. incontinentia pigmenti (Bloch-Sulzberger syndrome) associated with acute granulocytic leukemia.incontinentia pigmenti, a syndrome of developmental defects, was found to be associated with acute granulocytic leukemia in a 4-month-old black girl. She has been responsive to antileukemic drugs, and remission has been induced without difficulty after each relapse. Although survival time was expected to be very short because of her age, race, type of leukemia, and initial peripheral leukocyte count at time of diagnosis, she is still alive 35 months after diagnosis of leukemia. One may speculate that the prolonged survival time might be related to the unknown genetic defect.- - - - - - - - - - ranking = 7keywords = leukemia (Clic here for more details about this article) |
3/223. Pneumomediastinum, subcutaneous emphysema, and pulmonary fibrosis in a patient with idiopathic pneumonia syndrome after bone marrow transplantation.An adolescent female underwent bone marrow transplantation for relapsed leukemia and developed acute and chronic graft-versus-host disease and idiopathic pneumonia syndrome. Her lung disease responded to large doses of methylprednisolone but evolved to pulmonary fibrosis and pneumomediastinum and subcutaneous emphysema in the convalescent period. Pulmonary function tests revealed a restrictive pattern. Pneumomediastinum and subcutaneous emphysema are complications not only of obstructive but also of restrictive lung disease and vary with respect to time of onset.- - - - - - - - - - ranking = 1keywords = leukemia (Clic here for more details about this article) |
4/223. Erythroleukemia-like syndrome due to busulfan toxicity in polycythemia vera.Over a 19-year period, a patient with polycythemia vera who had undergone a splenectomy received six courses of busulfan for recurrent thrombocytosis. The total dose of busulfan given for the sixth course was greater than that used for the previous ones. Severe pancytopenia followed, which persisted for 4 months. During this period there was marked erythroid hyperplasia in the bone marrow with striking dyserythropoiesis; PAS-positive red cell precursors, as well as moderate numbers of circulating normoblasts and evidence of chronic and acute hemolysis, were present. All of these findings reverted to normal without therapy, and the polycythemic state eventually recurred. These events are interpreted as an unusual marrow reaction following busulfan overdosage rather than a transient erythroleukemia.- - - - - - - - - - ranking = 5keywords = leukemia (Clic here for more details about this article) |
5/223. 17p- syndrome arising from a novel dicentric translocation in a patient with acute myeloid leukemia.The cytogenetic contribution to the poor prognosis when myelodysplastic syndrome (MDS) progresses to acute myeloid leukemia (AML) is not well understood. We present a 66-year-old male who had thrombocytopenia with dysplastic features in peripheral blood neutrophils (hypogranular, hyposegmented neutrophils) comprising the pelger-huet anomaly, increased blasts in the marrow, and markers consistent with AML. Diagnostic marrow cytogenetics showed a complex karyotype including del(5q), a novel unbalanced dicentric translocation, t(17;20), resulting in both del(20q) and del(17p). fluorescence in situ hybridization (with probe TP53) showed deletion of 17p13 on the dicentric chromosome, completing the criteria for the 17p- syndrome. fluorescence in situ hybridization with probes for two tumor suppressor genes on chromosome 5q also showed deletion (CSF1R [at 5(q33.2-q33.4) and EGR-1 [5(q31-q32)]). Remission was difficult to achieve and cytogenetic relapse occurred 6 months postdiagnosis, and clinical relapse approximately one month later. Our case provides a novel mechanism for the 17p- syndrome, and highlights the difficulty of attributing prognostic significance to a particular cytogenetic abnormality in AML.- - - - - - - - - - ranking = 5.0784995009467keywords = leukemia, myeloid leukemia (Clic here for more details about this article) |
6/223. Acute myeloid leukemia in TAR syndrome.TAR syndrome is a rare inherited autosomal recessive disorder with a mortality rate of 30-40% mainly as a result of haemorrhage, in the first year of life. Most of the infants recover from the effects of thrombocytopenia and associated haematological complications with the modern medical care. Very rarely, the outcome is fatal, with the occurrence of acute leukemias.- - - - - - - - - - ranking = 5.0627996007574keywords = leukemia, myeloid leukemia (Clic here for more details about this article) |
7/223. A novel syndrome of radiation-associated acute myeloid leukemia involving AML1 gene translocations.AML1 is a transcriptional activator that is essential for normal hematopoietic development. It is the most frequent target for translocations in acute leukemia. We recently identified 3 patients in whom pancytopenia developed almost 50 years after high-level radiation exposure from nuclear explosions during or after world war ii. In all 3 patients, acute myeloid leukemia (AML) eventually developed that had similar characteristics and clinical courses. cytogenetics from the 3 patients revealed a t(1;21)(p36;q22), a t(18;21)(q21;q22), and a t(19;21)(q13.4;q22). By fluorescent in situ hybridization (FISH), all 3 translocations disrupted the AML1 gene. Two of these AML1 translocations, the t(18;21) and the t(19;21), have not been reported previously. It is possible that the AML1 gene is a target for radiation-induced AML. (blood. 2000;95:4011-4013)- - - - - - - - - - ranking = 6.0784995009467keywords = leukemia, myeloid leukemia (Clic here for more details about this article) |
8/223. Somnolence syndrome in a child following 1200-cGy total body irradiation in an unrelated bone marrow transplantation.Neurological complications may occur following intensive chemotherapy and hematopoietic cell transplantation. Postirradiation somnolence syndrome has been observed in children with acute lymphoblastic leukemia who received central nervous system preventive therapy with 1800-2400 cGy cranial irradiation. The authors report a 16-year-old boy with chronic myelogenous leukemia in chronic phase, who developed symptoms compatible with the somnolence syndrome (SS) 6 weeks following HLA-matched unrelated bone marrow transplantation (BMT). The preparative regimen consisted of 1200 cGy total body irradiation (TBI), cytosine arabinoside and cyclophosphamide. The patient developed lethargy and low-grade fever, with intermittent rhythmical delta activity in electroencephalograph. He recovered spontaneously without specific therapy 3 weeks after developing symptoms. This is the first report describing that as low as 1200 cGy TBI can induce SS in a child. After allogeneic BMT, some patients develop neurological symptoms. The authors suggest that somnolence syndrome should be included in differential diagnosis in these patients.- - - - - - - - - - ranking = 2.0816438470425keywords = leukemia, myelogenous, myelogenous leukemia (Clic here for more details about this article) |
9/223. Diffuse alveolar hemorrhage in acute promyelocytic leukemia patients treated with ATRA--a manifestation of the basic disease or the treatment.All-trans-retinoic acid (ATRA) is considered the recommended induction treatment for acute promyelocytic leukemia. In the pre-ATRA era pulmonary bleeding was a common cause of death in these patients, mostly due to disseminated intravascular coagulation which was further exacerbated by the administration of chemotherapy. Although ATRA syndrome, the most serious adverse effect of ATRA treatment, involves the lungs, pulmonary hemorrhage has only rarely been reported as a manifestation of ATRA syndrome. Here we describe 2 patients who developed diffuse alveolar hemorrhage during treatment with ATRA. The possible mechanisms of pulmonary bleeding in these cases are discussed.- - - - - - - - - - ranking = 5keywords = leukemia (Clic here for more details about this article) |
10/223. Acute lymphoblastic leukemia in one of two siblings with alstrom syndrome.alstrom syndrome is a rare autosomal recessive disease; less than 60 cases have been reported. No Chinese patient with this disease has been reported previously in the literature. Here, we describe an 11-year-old Chinese boy with this condition. His elder sister also had alstrom syndrome, and his father had non-insulin-dependent diabetes mellitus. Both siblings had degenerative retinopathy, obesity, mental retardation, perceptive hearing loss, short stature, non-insulin-dependent diabetes mellitus, nephropathy, hyperlipidemia, acanthosis nigricans, and hepatic dysfunction. The boy also developed acute lymphoblastic leukemia, which was confirmed by cytochemistry and immunophenotyping findings. He received chemotherapy and radiotherapy for the malignancy. The present case suggests that acute lymphoblastic leukemia may be coincident with or may be a previously undescribed systemic manifestation of alstrom syndrome.- - - - - - - - - - ranking = 6keywords = leukemia (Clic here for more details about this article) |
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