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

From a series of consecutively studied spleens, perfusion-fixed and investigated according to a standardized procedure allowing morphometric investigations at the light and electron microscopic levels, 33 spleens causing splenomegaly syndrome (12 lymphoproliferative diseases, 10 hairy cell leukaemia, 11 myeloproliferative diseases) were compared with data in controls and autoimmune haemolytic anaemia and hereditary spherocytosis from previous studies. In splenomegaly syndrome, especially in hairy cell leukaemia and myeloproliferative diseases, less so in lymphoproliferative diseases, there is a disproportionate increase in the volume of pulp cords in the red pulp. Values for erythrocyte volume density are of the same order as splenic erythrocyte concentration determined by scintigraphic kinetic methods. Cases with complicating immunohaemolysis show a rather high proportion of erythrocyte profiles with nearly spheric shape as in autoimmune haemolytic anaemia and hereditary spherocytosis.
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PMID:Red pulp in splenomegaly syndrome: morphometric light and electron microscopy studies. 370 51

Thirty-four patients with a clinical diagnosis of plaque stage mycosis fungoides, poikiloderma atrophicans vasculare (poikiloderma) and parapsoriasis en plaques (parapsoriasis) were investigated for evidence of extracutaneous disease using a biochemical screen, blood films, bone marrow examination, chest radiograph, abdominal ultrasound, isotope liver scan, liver biopsy and lymphangiography. Skin biopsies were also taken from these patients and, in order to rule out non-specific histological change, from 29 controls with inflammatory skin disease; the slides were examined blind by two independent observers for evidence of mycosis fungoides or poikiloderma and graded accordingly. Both observers were able to differentiate significantly histological grades of mycosis fungoides corresponding to the clinical diagnosis although exact histological grades only corresponded on 35 per cent of slides. Features graded as classical or probable mycosis fungoides were found in between 22 and 67 per cent of patients with parapsoriasis and between 67 and 100 per cent of those with poikiloderma. We found no evidence of extracutaneous disease in any of our patients. Lymphangiograms were abnormal in 30 of 31 examinations but the changes were non-specific and did not correspond to disease type, duration or extent. Four patients had apparently unrelated co-existing disease including chronic lymphatic leukaemia in two, a monoclonal gammopathy and autoimmune haemolytic anaemia. This study shows that in its early stages mycosis fungoides is predominantly if not primarily a cutaneous disease. The findings also suggest that parapsoriasis and poikiloderma are part of the same disorder as mycosis fungoides or evolve into it. Aggressive treatment to prevent progression to mycosis fungoides plaque and tumour stage should therefore be tried. The findings support the idea that mycosis fungoides is a reactive rather than a neoplastic disorder.
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PMID:The nature of mycosis fungoides. 373 71

A patient whose initial hematologic evaluation suggested the diagnosis of a microangiopathic hemolytic anemia (MAHA) was further evaluated and found to have erythroleukemia (DiGuglielmo's syndrome). This prompted us to review retrospectively the peripheral blood morphology of 12 patients with erythroleukemia. Anisocytosis, poikilocytosis, macrocytosis, and nucleated red cells have been described in patients with erythroleukemia; however, changes characteristic of a microangiopathic hemolytic process (schistocytes) have not been previously described. Our patients with erythroleukemia had prominent helmet and fragmented red cells, as well as elliptocytosis. Six of our 12 patients with erythroleukemia did not have blasts on their peripheral smear, and platelets were decreased (platelet count ranged from 2 to 92 X 10(3)/microliter), resulting in changes similar to patients with MAHA due to thrombotic thrombocytopenic purpura (TTP), traumatic RBC lysis, and disseminated intravascular coagulation. Our data indicate the RBC changes characteristic of MAHA are commonly seen in erythroleukemia, and that as many as half of these patients may not have white cell changes suggestive of leukemia on the peripheral smear. Patients presenting with microangiopathic hemolytic anemia require a bone marrow examination to confirm or exclude a myelodysplastic syndrome.
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PMID:Schistocytes in erythroleukemia. 385 84

The behavior of transplanted large granular lymphocyte leukemia was studied by isogeneic inoculation of 55 young F344 rats. A high percentage of transplants were successful, showing a dose-dependent latent phase followed by weight loss, exponential increase of tumor cells, and widespread infiltration. Serial passage of tumor cells appeared to increase malignancy by decreasing longevity. Grossly, there was splenomegaly, variable lymphadenopathy, and petechial hemorrhages on the lungs, lymph nodes, and brain. Diffuse infiltration of tumor cells caused morphologic changes identical with spontaneous large granular lymphocyte leukemia including splenic lymphoid depletion, hepatocellular necrosis, tumor cell erythrophagocytosis, and femoral medullary endosteal bone proliferation. Clinicopathologic features were also similar to spontaneous large granular lymphocyte leukemia. There was immune-mediated hemolytic anemia and thrombocytopenia, which coincided with development of leukemia, as well as hyperbilirubinemia and elevated serum enzymes, indicative of liver disease. It was concluded that isogeneic transplantation of large granular lymphocyte leukemia is not only highly successful but reproduces the pattern and clinical syndrome of the spontaneous tumor including the important paraneoplastic syndrome of hemolytic anemia and thrombocytopenia.
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PMID:Behavior of transplanted large granular lymphocyte leukemia in Fischer 344 rats. 402 45

Marrow transplantation is effective treatment for a number of haematological diseases in patients under the age of 50 who have an HLA-identical sibling donor. It is generally successful when used early in the treatment of aplastic anaemia. It is the only treatment that offers long-term disease-free survival for patients with acute leukaemia who have relapsed at least once, with 10-30 per cent apparent cures. Although still somewhat controversial, it appears also to be the treatment of choice for patients with acute non-lymphoblastic leukaemia in first chemotherapy induced remission and for those with chronic myelogenous leukaemia in the chronic phase since approximately 50-60 per cent of these patients are surviving after marrow transplantation in complete remission, apparently cured. Marrow grafting is the only effective treatment for many patients with inherited immunological-deficiency diseases and certain genetic storage diseases. It is being explored for the therapy of patients with lymphoma, Hodgkin's disease, multiple myeloma, small-cell lung cancer, testicular cancer, ovarian cancer and genetic disorders of haematopoiesis. Cures of congenital Fanconi anaemia, Blackfan-Diamond anaemia, osteopetrosis, and paroxysmal nocturnal haemoglobinuria have been achieved by marrow grafting. Genetic disorders associated with haemolytic anaemia and cyclic neutropenia have been cured by marrow grafting in animals. Target disorders for marrow transplantation in humans are thalassaemia major and sickle cell disease, and, indeed, a first successful transplant for treatment of thalassaemia major has recently been described (Thomas et al, 1982). Marrow transplantation has been limited by the fact that many patients do not have HLA-identical siblings and very few have monozygotic twins. The Seattle team has now explored the use of less well-matched family member donors in more than 80 patients with leukaemia. These donors share one HLA haplotype genetically with the patient and are phenotypically identical at two of the three major HLA loci on the other HLA haplotype (Clift et al, 1979). Overall, the post-transplant survival appears more a reflection of the type and stage of the leukaemia than of the marrow donor. Patients with leukaemia grafted in relapse have a projected survival of 20-30 per cent and those transplanted in remission of 50 per cent. The incidence and severity of GVHD may not be significantly different from that of patients given HLA-identical sibling marrow grafts.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:Application of bone marrow transplantation in leukaemia and aplastic anaemia. 635 79

Complete hemograms were evaluated for 57 rats with mononuclear cell leukemia and compared to hemograms obtained from 52 age- and sex-matched nonleukemic rats. All leukemic rats had marked hemolytic anemia and associated spherocytosis, reticulocytosis, anisocytosis, and polychromasia. The anemia varied with the stage of illness and was more severe in rts with advanced leukemia. Death appeared to be related to anemia. There was a marked neutrophilia with left shift, mild lymphopenia, and moderate to severe thrombocytopenia. Atypical mononuclear cells were detected in circulation in all but three rats. Total white blood cell counts ranged from 5.0-370 x 10(3) cells/ml. There was an increase in erythrocyte osmotic fragility with separation into two distinct populations of erythrocytes. Eight of nine rats were Coombs' positive indicating an immune-mediated pathogenesis for the anemia. Hemostasis tests revealed a markedly prolonged prothrombin time, hypofibrinogenemia, slightly increased to normal partial thromboplastin time, and undetected fibrin degradation products. These findings suggest significant liver disease associated with the leukemia.
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PMID:Pathology of the mononuclear cell leukemia of Fischer rats. II. Hematology. 664 39

Paroxysmal nocturnal hemoglobinuria is characterized by chronic hemolytic anemia, leukopenia, and thrombocytopenia. The increased hemolysis and hemoglobinuria associated with sleep have been observed so frequently that these features have been incorporated into the syndrome's name. Infections, especially of the respiratory and urinary system, can cause hemolytic episodes. Patients with paroxysmal nocturnal hemoglobinuria have increased susceptibility to infections. Some PNH patients are leukopenic, but many are not. It has been reported that leukocyte alkaline phosphatase of granulocytes in patients with PNH is low. As Hartmann and Kohlhouse point out, "The principles of treating infection in PNH seem no different than the therapy of infections in any group. "If major surgery is indicated, preparation should include saline-washed red cells, which would increase the patient's number of circulating normal red blood cells, if necessary. The prognosis is variable. A small percentage of patients with PNH develop leukemia. However, in at least half of all patients, the number of complement sensitive cells decreases, and many of these patients live a fairly normal life.
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PMID:Paroxysmal nocturnal hemoglobinuria: report of case with odontogenic infection. 693 60

Chromosomes of bone marrow from 28 patients with acute nonlymphocytic leukemia (ANLL) (26 with AML, 2 with AMMoL), 19 of whom had chromosome abnormalities, were studied; 11 cases exhibited previously unreported karyotypic abnormalities. The marrows of two cases had 8-21 translocations associated with an iso-X chromosome in the female patient and with 9q13- and a missing Y in the male patient. Usually, AML patients with a 8-21 translocation have been considered to have a good prognosis; however, our cases had rather short survival times. Therefore, the prognosis of AML with an 8-21 translocation but associated with other abnormalities is still not clear. Centromere spreading (CS), which was originally reported in marrow cells of megaloblastic anemia (B12 and folic acid deficiency), was detected in leukemic cells, disappeared during remission, and reappeared on relapse. These findings suggest that CS may be a new type of abnormality in AML. In two patients with atypical hypoplastic anemia and hemolytic anemia, chromosome abnormalities were detected at the anemic stage. One case with CS was associated with atypical hypoplastic anemia and developed AML after 1 year; the other with 48,XY,+i(1q),+3,/12 and -14 had hemolytic anemia and developed AMMoL 3 weeks later. Interestingly, identical clones were detected both before and after the clinical diagnosis of leukemia. These cases strongly support the concept that some chromosome abnormalities precede the clinical manifestations of leukemia. The present study also revealed that lymphocytes in ANLL respond poorly to PHA in the presence of high numbers of blasts but do respond well to mitogens during remission. Therefore, the response of lymphocytes to PHA may serve as one criterion for determining remission.
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PMID:Diagnostic and prognostic significance of chromosome abnormalities in marrow and mitogen response of lymphocytes of acute nonlymphocytic leukemia. 695 Aug 4

Hematologic, serum chemical, and histopathologic studies were performed on 17 aged Fischer 344 rats with mononuclear leukemia. Twelve of the rats had leukemic hemograms, while five had nonleukemic or marginally abnormal differential leukocyte counts. Hematologic findings revealed that all rats were profoundly anemic. Serum chemistry studies confirmed the occurrence of icterus observed clinically, a finding consistent with hemolytic anemia. Alanine aminotransferase and serum alkaline phosphatase values were elevated.
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PMID:Hematologic and serum chemical characteristics of mononuclear leukemia in Fischer 344 rats. 709 30

221 patients were treated by splenectomy for various hematologic disorders, including immunologic thrombocytopenia (80 cases), hereditary spherocytosis (15 cases), immune hemolytic anemia (25 cases), chronic lymphatic leukemia (27 cases), lymphosarcoma (27 cases), myelofibrosis (11 cases) and pancytopenia/aplastic anemia (14 cases). There were 8 postoperative deaths (3.6%), and 49 patients (22%) had postoperative complications, mainly infections and bleeding. Complications were more frequent in cases of massive splenomegaly (greater than or equal to 1 000 g) (65 cases), severe thrombocytopenia (less than 20 X 10(6)/1) (51 cases), and leukemia. Late postsplenectomy fulminant infection was seen in 8 patients (3.6%) for a mean follow up of 5.3 years. We conclude that splenectomy is sufficiently well tolerated even by severely ill patients to make it applicable as a therapeutic modality in the various hematologic disorders presently studied.
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PMID:Complications after therapeutic splenectomy for hematologic disease in adults. 722 97


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