Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0012739 (disseminated intravascular coagulation)
 8,673 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The procoagulant cellular activity (PCA) of intact and lysed leukaemic cells was evaluated at diagnosis in 23 patients with acute non-lymphoid leukaemia (ANLL). The leukaemic cells of all 13 patients having DIC feature (excess of fibrin monomers, serum FDP and plasma fibrino-peptide A) showed a significant (P less than 0.0001) increase of PCA, while a pattern similar to that of normal granulocytes and lymphomonocytes was observed in the remaining 10 patients without evidence of DIC. When the patients were subdivided according to the FAB cytological classification, features of DIC and increased PCA were demonstrated in 3/3 M3 patients, 5/6 M5 patients and only in 5/14 remaining patients. These findings indicate that in ANLL patients: (1) the increased PCA of leukaemic cells is closely related to the occurrence of DIC; (2) the increased PCA seems related to the differentiation line and maturation level of the leukaemic cells.
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PMID:Procoagulant cellular activity and disseminated intravascular coagulation in acute non-lymphoid leukaemia. 397 70

Sequential coagulation tests were carried out in 13 children with acute nonlymphoblastic leukemia (ANLL) treated with the German cooperative protocols BFM 78 and 82. The test program included a PTT, Quick's Prothrombin time, Thrombin time, Fibrinogen (Clauss method and RID), coagulation factors II, V, VII, AT III, antiplasmin, plasminogen and FDP. Severe coagulation changes could be demonstrated in ANLL patients with FAB type M2 (myeloblastic leukemia with maturation) and FAB type M5 (monocytic leukemia). Usually they were found already at the time of diagnosis and improved during induction therapy. A variety of coagulation changes were observed, resembling classical DIC, typical hyperfibrinolysis or atypical proteolysis. It is allowed to question the concept of DIC as the typical coagulation disturbance in children with ANLL.
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PMID:[Blood coagulation changes in children with acute myelogenous leukemia: thrombin effect or proteolysis]. 659 Sep 23

I studied the karyotype in 66 patients (38 children and 28 adults) with ANLL, who were diagnosed on the basis of the FAB classification. Clonal chromosome abnormalities were found in 45 of the 66 patients. Six patients had AML (M1), and 23 including 8 with t (8; 21), had AML (M2). All 10 patients but one with APL (M3) had t (15; 17). Twelve patients had AMMOL (M4); 7 of these had a normal karyotype, and another had t (11; 19). Fourteen patients had AMOL (M5); 4 of these had t (9; 11), and 4 others had 11q rearrangements not involving chromosome 9. Only one patient had EL (M6), whose karyotype was normal. As t (8; 21) and t (15; 17) are uniquely associated with AML (M2) and APL (M3), respectively, t (9; 11) is seen only kn AMOL The other 11q rearrangements are more common in AMOL, but are also seen in AMMOL. To examine correlation of karyotype with prognosis, the 66 patients were classified into 6 groups; 8 with t (8; 21), 9 with t (15; 17), 4 with t (9; 11), 5 with other 11q rearrangements, 19 with other abnormalities, and 21 with normal diploidy. All the patients with t (8; 21) entered remission, and their median survival (16 months) was longer than that of any other group of patients. The patients with normal diploidy, those with the 11q rearrangements, or those with other abnormalities had a little shorter survival than those with t (8; 21). All 9 patients with t (15; 17) had DIC, and 4 of these died during induction therapy. Their median survival was only 6 months. All patients with t (9; 11) died during induction therapy; 3 of these had extremely high WBC counts and DIC at diagnosis. The patients were also classified into 3 groups, i. e. NN (20 patients), AN (21 patients) and AA (19 patients), on the basis of the frequency of abnormal mitotic cells in the bone marrow. The NN patients or the AN patients had longer survival times than the AA patients (p less than 0.05). My study demonstrated that the karyotype is correlated with morphology of leukemic cells and with survival.
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PMID:[Chromosome aberrations and clinical features of childhood and adult acute nonlymphocytic leukemia]. 666 86

Seven hundred sixteen newly diagnosed patients were studied, to determine the clinical significance of chromosome analysis in ANLL. Karyotypes were classified in two ways. Cases were grouped into three categories, based on the presence of normal and abnormal metaphases. Cases were grouped into 12 categories based on more specific chromosome abnormalities (modified Chicago Classification). Both methods of classifying karyotypes, but especially the Chicago Classification, resulted in groups of patients with de novo ANLL with significantly different presenting clinical and hematologic features, including FAB type, leukocyte count, percent peripheral myeloblasts, platelet count, and DIC. Among patients with de novo ANLL, karyotypes, when classified according to the Chicago Classification, significantly correlated with frequency of initial complete remission and survival; the presence of normal and abnormal metaphases correlated with duration of first remission and survival. Among 305 intensively treated patients, the Chicago Classification also correlated with duration of first remission. Although both ways of classifying karyotype correlated with survival, even when other major risk factors in ANLL were considered, only the Chicago Classification was an independent prognostic factor among the intensively treated patients.
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PMID:Fourth International Workshop on Chromosomes in Leukemia 1982: Clinical significance of chromosomal abnormalities in acute nonlymphoblastic leukemia. 670 38

Sixteen children (ages 2--17) with acute promyelocytic leukemia (APL) were studied retrospectively. Diagnosis was based on clinical features and morphological criteria of the FAB classification. Bleeding diathesis was the predominant presenting symptom (greater than 85%), associated with laboratory findings of disseminated intravascular coagulation (DIC). Extramedullary manifestations included skin rash in six patients, gum infiltration in two, and meningeal leukemia in two. Induction therapy consisted of cytosine arabinoside and an anthracycline, with or without other agents. Prophylactic heparinization was given to 12 patients. Six patients (37.5%) failed to achieve remission and died, secondary to hemorrhage in three, and secondary to infection in the remaining three patients. The median duration of remission was 14 months, and the median survival for responders was 21 months. One meningeal leukemia preceded bone marrow relapse despite intermittent intrathecal chemotherapy for prophylaxis. Three patients remain in continuous complete remission 3 years after initial diagnosis. Although childhood APL shares many features of its adult counterpart, the high frequency of extramedullary manifestations and mortality secondary to neutropenia deserves separate attention.
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PMID:Acute promyelocytic leukemia in children. 693 7

Haemorrhagic diathesis is the commonest cause of morbidity and mortality in acute leukaemias (AL). It is most commonly due to thrombocytopenia resulting from bone marrow failure. However, in a significant number of cases, disseminated intravascular coagulation (DIC) plays an important part. Previously it was thought that this mechanism was mainly confined to acute promyelocytic leukaemia (APL), but recently it has also been reported to occur in other subtypes of acute leukaemia. We report the results of a study carried out to find the incidence of DIC in various types of AL at the time of first diagnosis and in the absence of other recognisable causes. DIC was observed in 14(13.4%) cases out of 104 cases of AL studied. Nine out of 49(18.4%) cases of AML and 5 out of 55(9.1%) cases of acute lymphoblastic leukaemia (ALL) showed coagulation abnormalities consistent with DIC. Out of the 9 cases of AML showing DIC, 63 (66.67%) belonged to APL (FAB ME) subtype. Three (60%) out of 5 cases of ALL with DIC had T-cell immunophenotype. The results indicate that DIC may also occur in types of AL other than APL, particularly in T-ALL, and should be looked for.
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PMID:Disseminated intravascular coagulation in acute leukaemias at first diagnosis. 762 93

Perloza beaded cellulose was functionalised by a cyanoethylation/reduction procedure to give aminopropyl Perloza. Fmoc-amino acids were anchored to aminopropyl Perloza beaded cellulose via the TFA labile 4-oxymethylphenoxyacetyl (HMPA) linker. Using Fmoc-aminoacyl-4-oxymethylphenoxyacetyl-2,4-dichloro-phenyl esters, all 20 amino acids were anchored at substitution levels ranging from 0.37 to 0.65 mmol/g. Fmoc-amino acids were also anchored using the peptide-amide linker 4-[(R,S)-1-[1-(9H-fluoren-9-yl)-methoxycarbonylamino - (2',4'-dimethoxybenzyl]phenoxyacetic acid. The Fmoc-aminoacyl resins were used for SPPS using Fmoc chemistry. SPPS was carried out using either an LKB Biolynx 4175 low-pressure pumped column continuous-flow peptide synthesiser or an ABI 430A automated vortexing batchwise instrument. Comparison of peptides made using each synthesiser showed little difference in quality of the crude peptides. Different Fmoc-amino acid activation methods (DIC/HOBt/DMF, HBTU, DIC/HOBt/DCM) were found to be equally useful with Perloza. Peptides were cleaved using TFA plus scavengers; however, the TFA-swollen resin was not readily separated from the TFA/peptide solution by simple filtration. Therefore alternative cleavage workup procedures were used with Perloza. Peptides were purified by HPLC and characterised by HPLC and amino acid analysis, and in some cases by FAB-MS. Successful syntheses ranged from 5 to 34 amino acids in length. Some of the peptides were also synthesized using a polystyrene support and standardised (ABI Fastmoc) SPPS protocols. The crude cleaved peptides from each synthesis were compared by HPLC analysis. The overall aim of our work with Perloza is synthesis of resin-bound peptide ligands for affinity chromatography and antibody generation.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Fmoc SPPS using Perloza beaded cellulose. 792 85

Acute promyelocytic leukemia represents 5-10% of acute myeloid leukemia cases (AML) recorded in the literature, occurring more frequently in young adults. It has a special clinical and biological behaviour when compared to the other forms of AML, being characterized by a particular morphology of blast cells (M3 in FAB classification), translocation of chromosomes 15;17, and disseminated intravascular coagulation at diagnosis or after the onset of chemotherapy. Within this AML subgroup there are 2 morphological subsets called the hypergranular promyelocytic leukemia and the hypogranular or variant form. We have studied clinical and laboratory aspects of 19 cases of AML M3 out of 217 AML cases, and observed a high incidence of failure to recognize the M3 variant form, although its diagnosis has been mainly based on cytomorphology. Only 4 out of 8 cases of the variant form received in our laboratory were correctly diagnosed, being the other 4 cases wrongly identified as the myelomonocytic subset of AML (M4). Immunophenotyping with monoclonal antibodies using CD2 and CD7 as T cell markers, CD10 and CD19 as B cell markers and CD33, CD13, CD14, CD15 and anti MPO as myeloid markers is a complementary diagnostic tool that permits solving difficult cases. It is important to classify AML correctly because of the special therapeutic and prognostic features of AML M3, which differently from other AML forms, has been successfully treated with cellular differentiating agents.
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PMID:[M3 variant leukemia: clinical and diagnostic features]. 816 87

Congenital leukaemia is a condition occurring very rarely. In a recent review in 1993, 175 cases are reported, 25-30% of them being well documented as leukaemia cutis. We reported a new case of congenital leukaemia diagnosed as an acute non lymphoblastic leukaemia M4 (FAB) and diagnosed at birth. It involves a newborn female at 42 weeks of gestational age. The most relevant clinical features were hepatomegaly and cutaneous petechial lesions along with a generalized distribution of nodules. From the blood peripheral count, leukocytosis is observed (177 x 10(9)/L) with 48% blasts of myeloid immunophenotype. The coagulation studies were consistent with a disseminated intravascular coagulation syndrome. A biopsy carried out on a cutaneous nodule, revealed diffuse dermoepidermic infiltration by immature cells of myeloid lineage, with cellularity and count similar to that of bone marrow and peripheral blood. The karyotype in the peripheral blood was normal. Infectious and immune causes were excluded as well as constitutional illnesses associated with unstable haematopoiesis. The family rejected treatment with chemotherapy and the baby died on day 53 of life due to progressive leukocytosis and concurrent infection. Our case, like 80% of the cases reported, is of myeloid origin and confirms the fatal evolution of untreated congenital leukaemia.
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PMID:[A new case of congenital leukemia with leukemia cutis]. 885 Feb 37

We describe an extremely rare case of granulocytic sarcoma of the porta hepatis causing obstructive jaundice. The patient was an 84-year-old man admitted because of obstructive jaundice. Ultrasonography (US) and computed tomography (CT) scanning of the abdomen disclosed a mass about 2.5 cm in diameter near the neck of the gallbladder, and thickening of the gallbladder wall. Based on these findings, gallbladder carcinoma was suspected. After endoscopic retrograde biliary drainage (ERBD) was performed, the jaundice resolved. However, blast cells were detected in the peripheral blood 51 days after admission, and laboratory studies disclosed acute myelocytic leukemia (AML: French-American-British [FAB] type M0). We treated him conservatively, with antibiotics and ERBD but he died of disseminated intravascular coagulation. Autopsy showed that the suspected gallbladder carcinoma was actually a granulocytic sarcoma arising in association with AML and causing obstructive jaundice. The largest tumor involved the porta hepatis. It should be kept in mind that granuloctyic sarcoma is a possible cause of obstructive jaundice, even in patients with no evidence of AML.
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PMID:An autopsy case of granulocytic sarcoma of the porta hepatis causing obstructive jaundice. 965 26


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