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Query: UMLS:C0023418 (leukemia)
 93,477 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 16-year old patient with Fanconi's anemia developed pre-leukemia and pulmonary infiltration which was found upon autopsy to be pulmonary alveolar proteinosis. The question whether the pulmonary alveolar proteinosis was due to the marked leukopenia and an eventual defective phagocytic ability of the leukocytes is raised.
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PMID:Pulmonary alveolar proteinosis associated with fanconi's anemia. 51 42

Two brothers developed acute leukemia, one at the age of 7 months and the other at the age of 14 months. Both suffered from a staturoponderal retardation and the same malformation syndrome. The karyotype carried out only on the second child revealed breaks and chromatid changes. A diagnosis of Fanconi's anaemia can be discarbed since no blood cytopenia preceded the leukemia. Finally, the diagnosis of Bloom's syndrome prevailed despite the absence of telangiectatic erythema and the atypical chromosomal anomalies.
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PMID:[Bloom's syndrome. Discussion of the diagnosis concerning two cases of terminal leukemia in a sibship (author's transl)]. 59 25

The article comprises the literature data on a new group of enzymopathies in man associated with the defect of the cell reparative mechanisms, which in the norm restore damages of DNA induced by factors of a different character. Special attention is paid to molecular processes observed in the hereditary disease in man--xeroderma characterized by a high sensitivity of the patient to ultraviolet irradiation and by a high incidence of cancer of the skin. Experimental evidences are presented testifying to an elevated sensitivity of cells of such patients to carcinogens, some viruses, and illustrating peculiar features of formation of structural mutations of chromosomes induced by physical, chemical and biological agents. Defects of individual enzymes of reparation in progeria, Fanconi's anemia and some other human diseases are described. The author recommends to simulate defects of reparative enzymes on diploid human cells infected with the virus of leukemia.
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PMID:[Nature of diverse molecular diseases in man]. 101 91

When the causes of death were determined in 18 relations of a child with Fanconi's anaemia 10 deaths were found to be due to carcinoma of various organs. Cases of osteogenic sarcoma, leukaemia, and Marfan's syndrome were also discovered among relatives. The family was from a remote community in the hebrides and there was considerable intermarriage. Suggestive evidence of heterozygosity was found by chromosome analysis.
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PMID:Familial cancer on a Scottish island. 106 48

Three patients with Fanconi's anemia were analyzed for chromosome breaks. T and B cells were separated and grown in tissue culture with PHA and pokeweed antigen to ascertain the rates of breakage in these lymphocytic subpopulations. It has been found that there is no statistically significant difference in breakage rates in T and B lymphocytes. It is postulated that both T and B cells could be involved in the development of leukemia in Fanconi's anemia patients, assuming that chromosome breaks constitute a factor predisposing to the development of malignancy.
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PMID:Chromosomal breaks in T and B lymphocytes in Fanconi's anemia. 108 38

Corticosteroids were used to treat two children with presumed idiopathic thrombocytopenic purpura and one with juvenile rheumatoid arthritis without examination of the bone marrow. Of the two with presumed idiopathic thrombocytopenic purpura, one had Fanconi's anaemia and the other may have had aplastic anaemia. The third child had acute lymphoblastic leukaemia. The diagnosis of Fanconi's anaemia was delayed. A diagnostic and therapeutic dilemma was caused in the second case. In the third, delayed diagnosis and, perhaps, compromised outlook resulted. These three cases re-emphasize the well aired caveats about the diagnosis of idiopathic thrombocytopenic purpura and juvenile rheumatoid arthritis and provide further support for the arguments of those who believe that if corticosteroids are to be used to treat such children, their bone marrow should be examined first.
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PMID:Bone marrow examination before steroids in thrombocytopenic purpura or arthritis. 129 Aug 52

Cryopreserved human umbilical-cord (HUC) blood is an alternative to bone marrow as a source of haemopoietic "stem" cells for HLA-identical transplantation of children with leukaemia or Fanconi's anaemia. We have studied the in-vitro growth potential of HUC blood in clonogenic assays and in longterm haemopoietic cultures. Clonogenic assays showed that HUC blood produced as many haemopoietic-cell colonies as normal adult bone marrow and a higher proportion of primitive-cell colonies. In longterm culture on preformed irradiated marrow stroma, both progenitor-cell production and lifespan of cultures were significantly greater in HUC blood than in normal bone marrow (p = 0.0007). Our findings indicate that the quality and quantity of HUC-blood-derived haemopoietic "stem" cells are better than those of normal bone marrow. Therefore, single HUC-blood donations are probably sufficient for adults requiring transplantation for leukaemia and other haemopoietic disorders. Banking of HLA-typed HUC blood to facilitate transplantation of patients who lack a family donor should be considered.
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PMID:Growth of human umbilical-cord blood in longterm haemopoietic cultures. 136 Jan 7

Complete or partial monosomy 7 is a recurring cytogenetic abnormality in acute myelogenous leukemia (AML) and myeloproliferative syndromes (MPS) and is particularly common in patients with Fanconi's anemia and in secondary AML. A familial form of monosomy 7 has been recognized in which two or more siblings develop MPS or AML before age 20. We tested the hypothesis that a recessive cancer susceptibility locus on chromosome 7 was important in the pathogenesis of leukemia in familial monosomy 7 by determining the parental origins of the chromosome 7 retained in the bone marrows of three pairs of affected siblings. We found no overlapping region where all three pairs retained DNA derived from the same paternal or maternal chromosome. These data suggest that inactivation of a single allele of a putative tumor-suppressor gene may be sufficient to contribute to leukemic transformation in familial monosomy 7.
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PMID:Evidence implicating heterozygous deletion of chromosome 7 in the pathogenesis of familial leukemia associated with monosomy 7. 135 90

In this article, the acid-base disturbances encountered in hematologic diseases are discussed. Occurrence of lactic acidosis (LA) without obvious clinical tissue hypoxia has been reported in patients with leukemia and lymphoma. Most of the patients with LA had liver involvement and clinical evidence of impaired hepatic function, suggesting that both increased production and decreased lactate metabolism are necessary for the development of LA in leukemia and lymphoma. Acute tumor lysis syndrome consists of hyperuricemia, hyperpotassemia, and hyperphosphatemia with hypocalcemia following neoplastic cell lysis, particularly in lymphoproliferative disorders. In patients with multiple myeloma (MM), proximal renal tubular acidosis (Fanconi syndrome) associated with Bence Jones proteinuria has been reported. In addition, MM is one of the first conditions recognized to be associated with lower anion gap.
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PMID:[Acid-base disturbances in hematologic diseases]. 143 14

Fanconi's anemia is an autosomal recessive disorder with a high incidence (greater than 90%) of aplastic anemia and a premalignant component with a greater than 10% risk of leukemia or solid tumors. The diagnosis of Fanconi's anemia depends on increased chromosomal breakage in lymphocytes following treatment with a DNA cross-linking agent; patients have been identified who are clinically well and whose physical appearance is normal. Although bone marrow or cord blood transplants can be curative, treatment for the aplastic anemia usually depends on androgens. Close to 20 patients with Fanconi's anemia have delivered normal babies, and the mothers' hematologic status was not significantly adversely affected by the pregnancy. A few patients have clonal cytogenetic abnormalities in their bone marrow that do not necessarily indicate leukemic transformation, but further follow-up is important. Studies of in vitro erythropoiesis indicate a correlation between the clinical hematologic status and the presence of erythroid progenitors in the blood or bone marrow. Certain hematopoietic growth factors do increase growth in vitro, suggesting that new types of therapy may become available. Not every patient has a poor prognosis. There are now many adults with Fanconi's anemia, some with families of their own.
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PMID:Fanconi's anemia. Current concepts. 153 Jan 23


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