Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0023418 (leukemia)
 93,477 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Chlorambucil is useful in patients with rheumatoid arthritis (RA) refractory to other agents but there is concern about the risk of haematological malignancy with this agent. A retrospective survey was performed to assess the incidence of all types of malignancy in 39 patients treated with chlorambucil (mean daily dose 4.25 mg, mean duration of treatment 25 months). These patients were compared with 30 patients with RA who received contemporaneously, the purine analogues azathioprine or 6-mercaptopurine (mean dose 100 mg, mean duration of treatment 24 months). Eight patients treated with chlorambucil and one patient receiving purine analogues developed cutaneous malignancy (p = 0.03). In the chlorambucil-treated patients these were mostly multiple and recurrent. Three patients treated with chlorambucil developed myeloid leukaemia or a preleukaemic state, whilst no patient treated with purine analogues developed this complication. The use of chlorambucil in RA is associated with an increased risk of cutaneous as well as haematological oncogenesis.
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PMID:The oncogenicity of chlorambucil in rheumatoid arthritis. 342 70

Hematologic malignancy has rarely been reported in adults with sickle cell disease. We describe four sickle cell patients (two with hemoglobin SC, two with hemoglobin SS) who developed hematologic malignancy (acute myeloblastic leukemia, multiple myeloma, malignant histiocytosis, and Hodgkin's disease). Three of the cases represent the first adult association between SC or SS hemoglobinopathy and the particular malignancy involved. Sickle hemoglobin does not appear to exert a protective effect against childhood hematologic malignancies, suggesting that better survival in sickle cell disease may be accompanied by an increased incidence of hematologic neoplasms in adulthood. Karyotypic analysis revealed alterations of chromosome 5 in two sickle cell patients with leukemia, raising the possibility of a chromosomal link between the two diseases. Further epidemiologic and cytogenetic studies are needed to define the relationship between hematologic malignancy and sickle cell disease.
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PMID:Hematologic malignancy in sickle cell disease: report of four cases and review of the literature. 345 91

Cellular oncogenes have been localized at the breakpoints of characteristic chromosomal rearrangements occurring in certain hematologic malignancies. This has been reported to result in aberrant expression of the involved oncogenes. Over 90% of chronic myelogenous leukemia (CML) is characterized by a reciprocal translocation that brings c-abl from chromosome 9 to chromosome 22, and c-sis from chromosome 22 to chromosome 9. To investigate the possible role of these two oncogenes in the leukemic process, we studied their expression in a number of fresh samples obtained from patients with various forms of leukemia, by Northern blot analysis using c-onc probes. Seven of 24 samples obtained from patients with either CML or chronic myelomonocytic leukemia expressed a normal 4.0-kilobase (kb) c-sis transcript. C-sis expression was found only in the accelerated/blast phases but not in the chronic phase of CML. All of the CML Philadelphia chromosome-positive (Ph1+) samples expressed an aberrant 8-kb c-abl transcript. The expression of c-sis in
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PMID:C-sis and C-abl expression in chronic myelogenous leukemia and other hematologic malignancies. 345 50

Congenital monoblastic leukemia cutis is a rare disorder. We report an infant who developed infiltrative skin lesions by 2 weeks of age, which, when biopsied at 4 1/2 months of age revealed a monoblastic infiltrate. Blasts in the peripheral blood were not seen until 1 week before her death at 8 months of age. Chromosomal analyses of her bone marrow showed an abnormal clone of cells with a 46,XX,del(10)(p12) karyotype. Although chromosome 10 is rarely involved in hematologic malignancies, abnormalities of this chromosome within the region 10p11-10p13 have now been shown in four of 10 reported cases of congenital monoblastic leukemia.
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PMID:Congenital monoblastic leukemia cutis. A case report with chromosomal abnormality: del (10p). 346 18

Progress in the study, treatment and cure of hematologic malignancies in children is one of the most gratifying accounts in the entire history of cancer therapy. It is important to note that progress in treatment and cure have been the direct result of formal study of childhood cancers and the development and clinical trial of new treatments designed to improve the response and cure rate. In the experience of the Children's Cancer Study Group (CCSG), approximately half of the children with cancer which are registered have hematologic malignancies. By far the most significant of these is acute lymphoblastic leukemia (ALL), which accounts for 33% of all the cancers registered with the Group. Acute non-lymphoblastic types of leukemia have amounted to 6% of all cancers in our experience, non-Hodgkin's lymphoma for another 6% and Hodgkin's disease for approximately 5%. The cancer death rate in children under the age of fifteen years has declined very significantly in the United States since 1950. In 1955 the National Cancer Institute organized the first national cooperative groups in order to develop and test new treatments for acute lymphoblastic leukemia. The CCSG was the first of these. The success which has been achieved in treating acute leukemia is remarkable, particularly when one recalls that in 1950 the disease was 100% fatal. The death rate due to Hodgkin's disease has also shown a sharp decline, and more recently, deaths due to non-Hodgkin's lymphomas have also declined.
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PMID:[Progress in the study, treatment and cure of hematologic malignancies in children in North America]. 346 53

Several different structural chromosome aberrations have been observed in human neoplasias. In this report we describe the isochromosomes found in nine patients with hematologic malignancies: five with leukemia, one with sideroblastic anemia, and three with malignant lymphomas. The isochromosomes i(7q), i(11q), i(17q), and i(21q) were detected in these patients. We suggest that the presence of isochromosomes permits us to speak of a gene-dosage effect and that this mechanism may play a role in malignant transformation.
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PMID:Presence of isochromosomes in hematologic diseases. 346 31

The Seattle Marrow Transplant Team treated about 130 patients (age 4-68 yr) for hematologic cancer with supralethal chemoradiotherapy and bone marrow transplantation (BMT) from the normal genetically identical twin. The procedure was well tolerated. The principal problem was tumor resistance. Nevertheless, BMT for acute leukemia in relapse still cured about 20% of the patients. Moreover, BMT performed while in complete remission cured about 50% of patients with acute lymphocytic leukemia or acute nonlymphocytic leukemia. Sixteen patients received transplantation in the chronic phase of Ph1+ chronic granulocytic leukemia (CGL). All showed disappearance of all Ph1+ cells. Two died of pneumonitis. Of the 14 who are alive, 3 continue to have CGL 37-76 months after BMT and 11 remain in complete hematologic and cytogenetic remission without any Ph1+ metaphases at 31-108 months (median = 68) after BMT. Thus the Ph1-positive clone can be ablated and blast crisis prevented. BMT in the accelerated or blastic phase was far less effective. Syngeneic BMT also benefited or cured patients with lymphoma, hairy-cell leukemia, and multiple myeloma. Therefore, BMT should be considered for every patient who has a hematologic cancer and an identical twin.
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PMID:Identical-twin (syngeneic) marrow transplantation for hematologic cancers. 352 68

During the past 10 years, 86 patients 30 to 54 years of age with hematologic malignancies were prepared with high-dose radiochemotherapy and received histocompatible bone marrow grafts. Thirty-four of these patients are surviving for 4 months to 9 years (median, 26 months) following marrow transplantation and 32 of them are in continuing complete remission (CR). Disease-free survival is 44% for 37 patients who were in first remission of acute leukemia or in the chronic phase of chronic granulocytic leukemia (CGL), 23% for 39 patients whose leukemia had relapsed at least once before transplantation or who had advanced stages of CGL, and 60% for ten patients who had hematologic malignancies other than leukemia. The median age of the surviving 34 patients is 36 years (range, 30 to 43 years). The incidence of moderate to severe acute graft-v-host disease (GVHD) was 48% and of chronic GVHD, 26%. The major causes of failure were interstitial pneumonia in 31 patients (24 of whom had antecedent acute GVHD) and recurrent leukemia in 12 patients (11 of whom had either never entered a CR or had relapsed at least once with acute leukemia or had progressive CGL before transplantation). Our data warrant further prospective studies in patients with hematologic malignancies who are older than 30 years.
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PMID:Bone marrow transplantation for hematologic malignancies in patients aged 30 years or older. 353 23

The immunocompromised patient, with or without superimposed granulocytopenia, provides a wide range of life-threatening challenges for the primary medical and nursing care management group. In the context of haematological malignancy, particularly leukaemia and bone marrow transplantation, special expertise needs to be developed which includes competence in many aspects of general medical, nursing, and intensive care techniques. Clearly, the range of essential knowledge extends to other disciplines including respiratory, cardiac, and renal medicine, with important contributions from microbiology, virology, dietetics and parenteral nutrition. The increasing cure rate achieved in haematological malignancy and the ever-widening indications for bone marrow transplantation require a dedicated and experienced group, able to handle all challenges. Integral to managing the complete patient, both through the acute phase and into periods of recovery and long-term rehabilitation, is inclusion of the paramedical specialists--social workers, occupational therapists, physical medicine physicians and physiotherapists. Proper balance in the cost-effective use of expertise from each of these groups and the provision of a suitable physical facility with protected environment and intensive care facilities are the cornerstones on which individual patient survival and ultimately increase in cure rates depend.
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PMID:The immunocompromised host. 355 Nov 28

Splenectomy was performed on 47 patients with massive splenomegaly (spleen weight greater than 1.5 kg). With one exception, all patients had a haematological malignancy. The indications for splenectomy were for the diagnosis of unknown cause (n = 5), for the relief of pain (n = 8), as the initial treatment of hairy cell or prolymphocytic leukaemia (n = 8), and for the correction of a haematological cytopenia (n = 26). Splenectomy was completely successful in relieving pain and in establishing a diagnosis, and provided effective palliation in patients with hairy cell and prolymphocytic leukaemia. An immediate correction of a haematological cytopenia was achieved in 24 patients, and the correction was maintained for over 1 year in 11 patients. There were no postoperative deaths and morbidity was acceptably low. We conclude that patients with massive splenomegaly can derive considerable benefit from splenectomy and that massive splenomegaly should not be regarded as a contra-indication to splenectomy.
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PMID:Splenectomy for massive splenomegaly. 359 18


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