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)

A retrospective study of 119 patients with acute promyelocytic leukemia (APL) treated with similar DNR containing regimens in reported. Antecedent of radiation exposure or cancer was found in 10 patients. At presentation hyperleucocytosis was rare (13/119 greater than 30 000/microliter); variant form was identified in 5 cases. Organomegaly was uncommon and severe metabolic abnormality was never noted at presentation. DIC was observed in 75% of pts; t (15;17) was confirmed in 25/30 pts. Complete remission (CR) rates have increased from 43% to 76% on account of improvement of supportive therapy with adequate DIC management. Addition of ARA C did not improve CR rates (72%). Surprisingly duration of CR seems related to maintenance therapy as 11/26 pts receiving 6 MP-MTX maintenance regimen were long-term survivors as compared to 1/34 comparable pts receiving cyclic monthly courses of chemotherapy.
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PMID:[Acute promyelocytic leukemia: retrospective study of 119 patients treated with daunorubicin]. 659 7

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

Procoagulant activity of gastric cancer tissues and leukocytes obtained from various types of leukemia have been studied with special reference to TTP. The following results were obtained. Homogenates of APL leukocytes and gastric cancer tissues contained strong procoagulant activities, most of which have been identified as TTP since the activities were neutralized by a specific antibody against purified human placenta TTP, inactivated by the removal of phospholipid with heptane-butanol mixture, and inactivated by the addition of phospholipase C. The delipidated homogenates regained procoagulant activities by relipidation procedures. These results also confirmed that TTP from APL leukocytes and gastric cancer tissues have the same lipoprotein properties as those of TTP in normal tissues. Though slight proteolytic activity and fibrinolytic activity were demonstrated in the homogenate of gastric cancer tissues, it was noted that the TTP activity was different from these two activities by partial purification of TTP from gastric cancer tissues. The TTP activity of 9 homogenates of gastric cancer tissues was 301 +/- 289 (mean +/- SD) units per mg protein, being higher in homogenates of mucinous adenocarcinoma and signet-ring cell carcinoma than in those of tubular and poorly differentiated adenocarcinoma. The mean TTP activity of leukocyte homogenates from 14 patients with APL and one out of 4 patients with CML in blastic crisis was 81 +/- 76 units/10(7) cells. The TTP activity of the homogenates of leukocytes from 7 out of 18 patients with AML and another patient with CML in blastic crisis ranged from one to six units/10(7) cells with a mean of 3.3 +/- 1.2. The TTP activity of leukocyte homogenates from the other 11 cases of AML, two cases of CML in blastic crisis, 6 cases of CML, and one case each of ALL and CLL were less than one unit/10(7) cells. In leukemic patients, all cases with a value of more than 202 for the product of units of TTP activity per 10(7) cells and differential count (%) of leukemic cells in the bone marrow smear (MU value) were accompanied by DIC. The MU value of leukemic patients correlated well to the plasma fibrinogen and serum FDP levels. All patients with a MU value of more than 277 died of DIC when a sufficient amount of heparin was not administered. On the other hand, no DIC developed in any of the patients with a MU value of less than 90.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:The role of tissue thromboplastin in the development of DIC accompanying neoplastic diseases. 666 48

A variant form of acute promyelocytic leukemia has been recently described, characterized by a particular morphological picture associated with severe disseminated intravascular coagulation, with hyperleukocytosis at onset in most cases, and often a rapidly fatal outcome. Reviewing our case series of the past 3 years, we have identified 2 cases which presented with this typical clinical and hematological picture, in which diagnoses have been made retrospectively. The possibility of diagnosing this variant at onset even only on the basis of cytomorphology is discussed as well as the advisability of studying a new therapeutic approach for cases of this type in a attempt to improve its otherwise very unfavorable prognosis.
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PMID:Atypical acute promyelocytic leukemia (M3 variant): description of two cases. 679 99

Three patients with acute leukemia, disseminated intravascular coagulation, and a specific acquired chromosome abnormality [t (15;17)] were found by transmission electron microscopy to have the typical distribution of granules seen in promyelocytes. However, the average granule sizes were 120, 170, and 180 nm, respectively, for the three patients, significantly less than the 250-nm resolution of light microscopy. We regard the leukemia in these three patients as comprising a distinct clinical, ultrastructural, and cytogenetic entity that we have chosen to call "microgranular" acute promyelocytic leukemia.
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PMID:"Microgranular" acute promyelocytic leukemia: a distinct clinical, ultrastructural, and cytogenetic entity. 692 5

Fifteen patients with acute promyelocytic leukemia (APL) were treated with anthracycline agents alone or combined with arabinosylcytosine (Ara C). Disseminated intravascular coagulation (DIC) was managed individually based on the presence of clinical as well as laboratory evidence of DIC. Ten patients received heparin. Eleven patients (73%) achieved complete remission (CR) but there were two early deaths from infection. The median duration of CR was 232 days (range, 41-780 days). Six patients were studied during ten relapses. In all cases, a morphologic picture more typical of acute myelocytic leukemia was seen during relapse but the DIC was similar in severity to that seen initially. Fever, without obvious infection and possibly due to the leukemic process, was a common presenting feature. Regenerating bone marrow aspirates in many patients who subsequently achieved CR without further treatment showed an inordinate number of promyelocytes, and this finding should not be immediately interpreted as disease resistant to therapy.
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PMID:Acute promyelocytic leukemia--clinical management of 15 patients. 693 77

Disseminated intravascular coagulation is relatively common as initial clinical presentation of certain myeloblastic leukemias, particularly acute promyelocytic leukemia, occurring less frequently in association with acute non-myeloid leukemias. The occurrence of such coagulopathy as presenting feature of a lymphoblastic leukemia is exceptional as demonstrated by the scarcity of reports in the literature and by the authors experience, having encountered only one case out of 86 consecutive patients with acute lymphoblastic leukemia. The laboratory findings in this case are commented upon, and particular emphasis is made on the measurement of coagulation factors II, V, VII-X, and VIII, the study of fibrin formation and fibrinolysis, and the changes in these parameters brought about by therapy with heparin, coagulation factors (fibrinogen, platelets), and cytostatic drugs. The coagulation abnormalities were corrected once the blast population was destroyed by chemotherapy, with the exception of persisting fibrinopenia secondary to the corticosteroid treatment included in the chemotherapy protocol. Disseminated intravascular coagulation was manifested in this case by gingivorrhagia, ecchymosis and hematuria. The probable etiological role of lymphoblasts in the development of disseminated intravascular coagulation is discussed.
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PMID:[Disseminated intravascular coagulation as the presenting feature of lymphoblastic leukemia. Study of a case]. 693 72

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

Three cases of a newly recognized "microgranular" variant of acute promyelocytic leukemia (FAB M3) are described. This poor-prognosis variant is easily confused with myelomonocytic (M4) or monocytic (M5) leukemia, but is associated with disseminated intravascular coagulation as is "hypergranular" M3. Such patients may be inappropriately treated unless the promyelocytic nature of the leukemia is recognized.
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PMID:Morphology and cytochemistry of "microgranular" acute promyelocytic leukemia (FAB M3m). 693 28

A 29-year-old female with acute promyelocytic leukemia presented with severe liver dysfunction and disseminated intravascular coagulation. Her condition rapidly deteriorated and she expired before any antileukemic therapy could be administered. At autopsy, she was found to have massive thrombosis of hepatic veins and their tributaries. Microscopic picture of liver was consistent with acute Budd-Chiari Syndrome, presumably due to the coexisting acute promyelocytic leukemia.
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PMID:Case report: hepatic vein thrombosis (acute Budd-Chiari syndrome) in acute leukemia. 694 90


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