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

Systemic lymphosarcomas are common in all species of domestic mammal. A binomial classification of these tumours, based on both the anatomical form (i.e., distribution of lesions) and the type of cytology, is proposed. Mast cell tumours also are common, especially in the dog. The categories of lymphoid neoplasms described are: lymphosarcoma, lymphoid leukaemia, nodular lymphoid hyperplasia, tumours of the immunoglobulin-forming cells, and thymoma. The myeloid neoplasms described are: myeloid leukaemia, erythroleukaemia, acute erythraemia, polycythaemia vera, megakaryocytoid leukaemia, panmyelosis, myelosclerosis, and monocytoid leukaemia. Mast cell tumours are divided into mastocytoma and malignant mastocytosis.
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PMID:Neoplastic diseases of the haematopoietic and lymphoid tissues. 421 49

Using a pH-adjusted toluidine blue stain (pH range, 2.5-6.5), we sequentially examined the staining patterns of mast cells in tissue sections taken from patients with localized or reactive mast cell lesions and benign or malignant mastocytosis syndromes. Reactive or benign lesions stained well over the entire range of pHs and were separated from malignant mast cell proliferations which stained poorly and with greatest intensity in the less acidic range (pH greater than 3.5). Patients with disseminated mast cell lesions but without tumor masses or leukemia had a staining pattern between that of benign and malignant lesions. Basophils stained intensely at pH 2.5, and metachromasia rapidly diminished at higher pH. The use of the pH dependent toluidine blue stain may be an adjunct in recognizing patients with mast cell lesions, predicting their prognosis, and distinguishing basophils from mast cells.
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PMID:Benign and malignant mast cell proliferations. Diagnosis and separation using a pH-dependent toluidine blue stain in tissue section. 618 13

Six patients had hematologic malignancies and coincident urticaria pigmentosa, five with the disseminated maculopapular form and one with the plaque form. Two patients had the juvenile-onset variety; the remainder had the adult eruptive variety. None of the patients complained of symptoms that could be attributed to liberation of histamine. In the two patients with juvenile-onset urticaria pigmentosa, the hematologic malignancies developed at the age of 17 years; one had Hodgkin's disease, and the other had acute myelomonocytic leukemia. In three patients with adult eruptive urticaria pigmentosa, the cutaneous lesions developed within 12 months of the diagnoses of lymphocytic lymphoma (two patients) and evolving myelomonocytic leukemia (one patient). In the remaining patient, cutaneous lesions developed many years before chronic lymphocytic leukemia was diagnosed. None of the patients had systemic mastocytosis. Skin biopsy specimens from all six patients showed an increase in dermal and perivascular round cells, and mast cells were seen in specimens from five of the six patients. In patients who received cytotoxic drugs for the hematologic malignancy, there was no change in the urticaria pigmentosa.
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PMID:Hematologic malignancies occurring in patients with urticaria pigmentosa. 695 22

Osler's influence in haematology was twofold: as an original observer both in the laboratory and the ward, and his encouragement of the establishment of clinical laboratories with the consequent development of clinical and laboratory haematologists. In 1870, when Osler entered McGill Medical School at the age of 21, he was already an experienced microscopist from his school days, but now his interest shifted from pond life to parasites and clinical microscopy. His post-graduate year with Burdon-Sanderson was to have been a study of leucocyte function, but instead came his research on platelets, continued and expanded when he returned to Montreal in 1874, together with much of his laboratory haematology--his comprehensive studies of pernicious anaemia and work on leukaemia, Hodgkin's disease etc. The move to Philadelphia in 1884 saw the establishment of a clinical laboratory, work on malaria, arsenic in anaemia and the blood disease chapter for Pepper's System. At Baltimore he had a rewarding clinical microscopy department, distinct from Welch's Institute, and this is the period, continued at Oxford, of Osler's accounts of clinical syndromes--polycythaemia, telangiectasia, mastocytosis and 'splenic anaemia'.
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PMID:Osler's influence on haematology. 703 26

The syndrome of mastocytosis extends from cutaneous urticaria pigmentosa through systemic mastocytosis to the rarely occurring mast cell leukaemia. Our investigations with a large patient collective have shown that systemic forms of the disease occur more often than is generally supposed. In particular the incidence of bone marrow manifestations deserve more attention. The inflammatory-granulomatous findings in the bone marrow suggest an immunoactive component in the pathogenesis of this disease. The bone lesions that occur in about 50% of patients are probably the result of the common endosteal site of the mastocytosis granuloma. These lesions can be generalized (osteoporosis-osteosclerosis) or localized (osteolytic-osteosclerotic foci). In clinical practice bone biopsy and skeletal radiology complement one another; in addition to skin biopsy bone biopsy supplies the initial diagnosis of mastocytosis and documents systemic manifestation; the X-ray picture informs the clinician about the type and extent of the bone pathology.
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PMID:[Skin and bone findings in mastocytosis]. 715 88

Increased numbers of bone marrow mast cells were found in 45 (2.2%) of 2,000 bone marrow specimens obtained from patients who had hematologic disorders. Mast cells were most frequently seen in the marrows of patients who had preleukemic syndromes, lymphoproliferative disorders, and acute leukemia. The 16 patients who had preleukemic syndromes included those with refractory sideroblastic and megaloblastic anemia (with or without an excess of blasts), idiopathic pancytopenia or pure erythrocytic aplasia, paroxysmal nocturnal hemoglobinuria, idiopathic refractory neutropenia, agranulocytosis or thrombocytopenia, and persistent eosinophilia. Five of the seven patients who had acute leukemia had nonlymphoblastic leukemia; two had blastic crisis of chronic granulocytic leukemia. Of the 13 patients who had lymphoproliferative disorders, eight had chronic lymphocytic leukemia, three had macroglobulinemia, and two had non-Hodgkin's lymphoma. Three patients who had chronic renal failure associated with severe anemia and two who had chronic liver disease, splenomegaly, or hypersplenism were also encountered. In this study there appeared to be a consistent relationship between the presence of increased numbers of mast cells and the lymphocyte and plasma cell counts in the bone marrow. The significance of the presence of secondary mastocytosis in premalignant lesions, neoplasia, and, in particular, lympho- and myeloproliferative disorders, is still unclear.
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PMID:Increased bone marrow mast cells in preleukemic syndromes, acute leukemia, and lymphoproliferative disorders. 745 27

The phenotypic and biologic properties of malignant cells in a case of aggressive mastocytosis with multi-organ involvement, circulating mast cell precursors and absence of skin infiltrates were analyzed. Circulating mast cell precursors were detected by immunostaining using antibodies against mast cell tryptase as well as by electron microscopy. These progenitors were tryptase+/chymase- (MCT) and accounted for 10 to 20% of nucleated mononuclear blood cells (MNC). A subset of them contained metachromatic granules. As assessed by combined toluidine blue/immunofluorescence staining, the granulated mast cell precursors were found to express CD9 (P24), CD33 (gp67) and CD44 (Pgp-1), but not basophil-related markers (CD11b (C3biR), CDw17 (lactosylceramide), CD123 (il-3R alpha))or monocyte-related antigens (CD14, CD15). Expression of the mast cell growth factor (MGF) receptor, c-kit(CD117), was also demonstrable, whereas the skin mast cell marker C5aR (CD88) could not be detected on mast cell precursors. The ligand of c-kit, recombinant human (rh) stem cell factor (SCF = MGF), induced histamine release from circulating mast cell progenitors, whereas rhC5a, a potent skin mast cell-/basophil-agonist, was ineffective over the dose-range (10(-9) to 10(-7(M)) tested. Analysis of mast cell antigens in malignant mastocytosis or mast cell leukemias may be helpful to establish a diagnosis and to determine the phenotype of the clone.
Leukemia 1996 Jan
PMID:A case of malignant mastocytosis with circulating mast cell precursors: biologic and phenotypic characterization of the malignant clone. 855 22

Schistosomes are eliminated from laboratory rats around 28 days post-infection, whilst they are still resident within the hepatic portal distributaries of the liver. We have previously shown that their presence in this location is accompanied by an intense mastocytosis. We have investigated the potential relationships between IgE responses, the allergenicity of schistosome antigens, mast cell responsiveness, and worm elimination. Total and specific IgE were measured using an ELISA and a functional assay based on 3H serotonin release from activated rat basophilic leukemia cells (RBL-SRA), respectively. Both assays revealed that infected rats produced elevated IgE titres relative to naive animals. At days 28 and 35, mixed-sex infections stimulated a higher total IgE than male-only infections. IgE was affinity purified from rat infection serum and used to probe a fractionated soluble worm antigen preparation (SWAP) by Western blotting. Two allergenic products were detected of M(r) 67 and 36-38 kDa, the former having the same molecular weight as a previously identified secretory protein. IgE from mixed-sex schistosome infections bound strongly to the 36-38 kDa molecule, compared to the relatively weak binding exhibited by IgE from male-only infection serum. Since eggs were not recovered from the infected rats, this reactivity was attributed to the greater release of allergens from female worms. Results from the RBL-SRA showed that female SWAP was a more effective trigger of mast cell degranulation in vitro, for equal amounts of protein. This enhanced allergenicity was ascribed to the relative abundance of carbohydrate moieties. Our results support a role for IgE, and mast cell degranulation in the elimination of a primary schistosome burden from rats.
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PMID:Elimination of a primary schistosome infection from rats coincides with elevated IgE titres and mast cell degranulation. 907 11

Four patients with systemic mastocytosis, two men and two women, are presented. Three of them (patients 1, 2, and 4) developed portal hypertension and ascites without histological evidence of cirrhosis in liver biopsy. The remaining patient (patient 3) had severe bone lesions with multiple vertebral fractures. None of the patients had skin or lymph node involvement. Two patients (patients 1 and 2) died 12 and 9 months after diagnosis with acute nonlymphocytic leukemia and overt mastocytic leukemia, respectively, while the other two (patients 3 and 4) are alive 58 and 14 months after diagnosis. Treatment with hydroxyurea or cytosine arabinoside had not any beneficial effect in two patients, while a substantial amelioration of back pain had been obtained by local irradiation and recombinant human interferon-alpha-2b administration in one patient (patient 3). All patients had laboratory findings compatible with autoimmune cholangitis. We concluded that systemic mastocytosis is a rare cause of noncirrhotic portal hypertension often simulating autoimmune cholangitis and leading to the erroneous diagnosis of liver cirrhosis. Diagnosis is based on the presence of mast cells in Giemsa-stained liver histological sections, and it may be confirmed by immunohistochemical detection of tryptase in the cytoplasm of these abnormally proliferating cells.
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PMID:Systemic mastocytosis: a rare cause of noncirrhotic portal hypertension simulating autoimmune cholangitis--report of four cases. 944 86

The stem cell factor (SCF)c-kit receptor interaction plays a critical role in the development and survival of mast cells. Several studies have also associated c-kit receptor mutations with the human diseases, mastocytosis and piebaldism. Overexpression of c-kit has been reported to be associated with myeloproliferative disorders and myelodysplastic syndromes. Using peripheral blood mononuclear cells (PBMCs) from 11 patients with indolent mastocytosis (category I), mastocytosis with an associated hematologic disorder (category II), or aggressive mastocytosis (category III); a patient with CMML unassociated with mastocytosis, and PBMCs from 13 normal subjects, we examined the level of expression of c-kit mRNA along with other c-kit isoforms to determine if overexpression of the c-kit receptor was associated with mastocytosis. Using quantitative competitive PCR, c-kit mRNA levels on average were found to be statistically elevated in the five patients with mastocytosis with an associated hematologic disorder and in the patient with aggressive mastocytosis as compared with controls, but not elevated in patients with indolent mastocytosis. The relative mRNA expression for the two c-kit isoforms was not significantly different in the mastocytosis patients compared with controls. This demonstration of the overexpression of c-kit mRNA in mastocytosis, and particularly those patients with clinical evidence of myelodysplastic syndrome, adds evidence to support the conclusion that mastocytosis, at least in some patients, is a feature of myelodysplasia; and suggests that determination of c-kit mRNA expression in PBMCs may provide an additional approach to assessing prognosis.
Leukemia 1998 Feb
PMID:Elevated expression of the proto-oncogene c-kit in patients with mastocytosis. 951 79


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