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)

During a 3-year period, 6 of 50 children with systemic malignacies developed neurologic complications such as hemiparesis, convulsions and loss of consciousness. The children consisted of 1 boy and 5 girls, from 3 to 12 years old, 3 with acute lymphoblastic leukemia and 3 with malignant lymphoma. Four patients received induction treatment that included intravenous administration of L-asparaginase and/or intrathecal administration of methotrexate. One patient received induction treatment and consolidation treatment that included intravenous administration of L-asparaginase. One patient received induction and consolidation treatment, and the protocol for peripheral blood stem cell transplantation. Laboratory examinations revealed coagulation dysfunction in 3 patients treated with L-asparaginase and 1 patient with disseminated intravascular coagulation (DIC). Magnetic resonance imaging (MRI) was performed on a 1.5-T unit, using spin-echo or fast spin-echo sequences. T1-weighted, T2-weighted, and proton density-weighted images were obtained in the axial and/or coronal plane (section thickness, 4 mm; inter-section gap, 2 mm). MRI was initially performed within 36 hours after the onset in all patients, and follow-up MRIs were performed for 6 months. MRI showed lesions involving the cortex and subcortex in 4 patients with coagulation dysfunction. In 2 of these 4 patients, Gd-enhanced T1-weighted images showed contrast enhancement in the surface of the gyrus, suggesting focal vascular stasis. Serial MRI revealed nearly complete resolution of the lesions. Symptoms were relieved in every case. The lesions on MRI were presumed to be due to venous thrombosis related to the coagulation dysfunction caused by L-asparaginase or DIC. On the other hand, in 2 patients with onset after intrathecal administration of high-dose methotrexate and cytarabine, MRI revealed multiple lesions involving the centrum semiovale and periventricular white matter. No Gd-enhancement of the lesion was detected. This MRI finding was consistent with leukoencephalopathy. As time passed, the symptoms improved completely, and the lesions became better demarcated. MRI is useful for differentiating lesions related to coagulation dysfunction from leukoencephalopathy.
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PMID:[MRI abnormalities of the brain in neurologic complications following treatment of cancer in children]. 875 96

The patient was 69-year-old male. He has a history of treatment for tuberculosis by artificial pneumothorax about 47 years ago. He was admitted an another hospital under the diagnosis of tuberculous pyothorax. He was transferred to our hospital because of chest pain and fever. Laboratory findings on the admission were as follows: ESR was 120 mm/hr, CRP was 20.22 mg/dl and other data were almost within normal limits. Chest X-ray showed a massive shadow in the right lower lung field, adjacent to the chest wall. Computed tomography (CT) showed tumor shadow with low density and invasions into the adjacent chest wall. Histological examination of surgically excised tumor biopsy revealed malignant lymphoma. The patient's condition improved and the size of tumor decreased temporarily by chemotherapy. Then, he began to complain of chest pain and high fever, and tumor in the chest wall invaded into the whole chest wall. He died of disseminated intravascular coagulation despite continuing chemotherapy. Postmortem examination revealed the following findings : the tumor existed mainly in the parietal pleura or the chest wall, adjacent to the lesion of pyothorax, and immunohistochemical examination showed that tumor was malignant lymphoma, diffuse, large B-cell type. Recent studies have shown a close association between EBV infection and pyothorax-associated lymphoma. We have to keep in mind the possible development of malignant lymphoma following tuberculous pyothorax, when we see patients complaining of fever or chest pain with tuberculous pyothorax.
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PMID:[A case of chronic tuberculous pyothorax associated malignant lymphoma]. 875 18

Acute promyelocytic leukemia (APL) is characterized by a unique hemorrhagic syndrome, disseminated intravascular coagulation, and the association with the specific (15;17 chi q22-23:q12-21) translocation, which disrupts the retinoic acid receptor alpha (RARA) and the promyelocytic leukemia (PML) genes. The t(15;17) leads to the formation of two reciprocal fusion genes, PML/RARA on chromosome 15 and RARA/PML on chromosome 17; it is responsible for the unique response of the disease to retinoic acid (ATRA) treatment. As was described for chronic myeloid leukemia and its associated t(9;22) [Philadelphia chromosome], variant translocations have been reported in APL, which are either complex translocations involving additional chromosome(s), or simple variant translocations involving only either one chromosome 15 or 17 and any of several chromosomes. Rearrangements of RARA and PML were documented in some of these variant translocations. In contrast, recent molecular analysis of APL cases with cytogenetically normal chromosomes 15 and 17 revealed the occurrence of submicroscopic translocations, leading to the formation of non reciprocal fusion genes, either PML/RARA or RARA/PML only. Detailed analysis of such cases may shed light on the mechanisms of translocation, on the selection of oncogenic products, and on the respective role(s) of the products of the translocation. Demonstration of the existence, in some APL-like leukemias, of masked translocations with involvement of PML and RARA, thus allows to (i) confirm the diagnosis of APL, (ii) adapt the treatment and (iii) monitor the residual disease. Finally APL-like leukemias were recently reported, with either a t(11;17) or t(5;17), resulting in the fusion of RARA to genes other than PML; these patients do not appear to respond to ATRA treatment. Altogether, these results emphasize the usefulness of a molecular definition of APL.
Leuk Lymphoma 1996 Jul
PMID:Variant and masked translocations in acute promyelocytic leukemia. 881 70

Laboratory evidence of disseminated intravascular coagulation (DIC) and/or fibrinolysis is present in the majority of patients with acute promyelocytic leukemia (APL). Historically, early hemorrhagic death (EHD) occurred in 10% to 30% of patients treated with chemotherapy. All-trans retinoic acid (ATRA), a differentiating agent, has a CR rate above 80% in patients, with ATRA-associated leukocytosis. We studied thrombotic events in this population and compared it to patients treated with chemotherapy alone. The results of studies using ATRA in patients with APL were reviewed. Patients received ATRA 45-50 mg/m(2) orally in two divided doses daily until complete remission. In newly diagnosed patients, Idarubicin 12 mg/m(2)/day was given intravenously for 4 to 5 days beginning on the fifth day of ATRA therapy or when the white blood cell count (WBC) was over 10x 10(3)/mu l. Thrombotic complications were noted in 3 of 31 patients during induction. Two died from thrombotic events during therapy with multiple thromboses documented at autopsy. ATRA syndrome was suspected in 2 of the patients with thromboses and only 1 of the patients without thrombosis. In previous studies, 1 of 25 APL patients treated with chemotherapy alone had thrombotic events during therapy. In conclusion, treatment of APL with ATRA may decrease the incidence of hemorrhagic complications, but does not eliminate thrombosis. While thrombotic events were not significantly increased in patients treated with ATRA, they were more common in patients suspected of having ATRA syndrome.
Leuk Lymphoma 1996 Feb
PMID:Thrombosis in patients with acute promyelocytic leukemia treated with and without all-trans retinoic acid. 883 99

In order to establish the frequency and clinical complications of DIC during remission induction of untreated adults with acute lymphoblastic leukemia, we retrospectively reviewed the records of 125 consecutive patients treated with vincristine, doxorubicin, and dexamethasone but without L-asparaginase. DIC, defined as hypofibrinogenemia in the presence of elevated fibrin-fibrinogen degradation products, was detected at presentation in 10% of 99 and during remission induction in 67% of 58 patients who were screened for DIC. Elevated levels of D-dimers (DD) were seen in all eight patients with DIC in whom they were measured. All cases of DIC were diagnosed by the ninth day of induction and were associated with infection in 15 of 39 patients. DIC did not cause any deaths but was temporally associated with two thromboses and four hemorrhages in six of the 16 patients with fibrinogen levels < 100 mg/dl but with only one hemorrhage among 23 patients (4%) with fibrinogen levels > 100 mg/dl (P < 0.01). Heparin was not administered to any patient, whereas platelets were administered to all to maintain platelet counts > 20 x 10(9)/l. Fresh frozen plasma (FFP) and/or cryoprecipitate were administered 26 patients resulting in a contemporaneous correction of the coagulopathy and in control of hemorrhages and thromboses. We conclude that DIC is rare at presentation but common during induction of adult ALL and is frequently associated with clinical complications when fibrinogen levels are < 100 mg/dl. We recommend daily testing of fibrinogen, PT, and DD during the first 10 days of induction, and for the patients with DIC platelet transfusions to maintain counts > 20 x 10(9)/l, and when fibrinogen levels fall below 100 mg/dl transfusions of FFP and/or cryoprecipitate. Additional studies are needed to determine the optimal management of the DIC during remission induction of adult acute lymphoblastic leukemia.
Leuk Lymphoma 1996 Mar
PMID:Disseminated intravascular coagulation in adult acute lymphoblastic leukemia: frequent complications with fibrinogen levels less than 100 mg/dl. 890 74

Two new cases of t(8;16)(p11;p13) in acute nonlymphocytic leukemia (ANLL) are described. These two patients in addition to the 34 previously described, showed a striking association with myelomonocytic (M4) or monocytic (M5) leukemia, extramedullary infiltration, erythrophagocytosis and disseminated intravascular coagulation. One of our patients showed a TCRbeta gene rearrangement. Alltogether 36 cases of t(8;16) ANLL have been documented until today. We here review their clinical and cytogenetic features.
Leuk Lymphoma 1996 Mar
PMID:Translocation t(8;16)(p11;p13) in acute non-lymphocytic leukemia: report on two new cases and review of the literature. 890 81

A 1-year-old boy with Wiskott-Aldrich Syndrome (WAS) who developed malignant lymphoma is described. He showed various complications such as atypical lymphocytosis, disseminated intravascular coagulation (DIC), intracranial hemorrhage, macroamylasemia, and monoclonal gammopathy (immunoglobulin A kappa chain). Epstein-Barr virus (EBV) DNA was detected in the tumor tissue, and the monoclonality of B cells from the tumor tissue was established. EBV-associated lymphoma is frequently observed in immunocompromised patients including those with WAS. The development of macroamylasemia, which is rare in childhood, is discussed in relation to lymphoma and monoclonal gammopathy. This case is unique in that the EBV-associated malignant lymphoma developed at an early age and was accompanied by macroamylasemia.
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PMID:Epstein-Barr virus-associated malignant lymphoma with macroamylasemia and monoclonal gammopathy in a patient with Wiskott-Aldrich syndrome. 902 18

We report the case of a 50-year-old female with malignant lymphoma presenting hemophagocytic syndrome and liver failure. She developed high fever, marked jaundice, and progressive liver failure, followed by evidence of disseminated intravascular coagulation (DIC). The course was complicated by severe hepatitis and the patient died six days after admission. Pathological diagnosis on autopsy specimens of the lung hilar lymph nodes was non-Hodgkin's T cell lymphoma, of the diffuse small cell type. Histopathologic examination of the liver demonstrated diffuse liver cell destruction with prominent T lymphocyte infiltration in the portal and periportal area. In addition to marked lymphoma cell infiltration, hemophagocytosis by prominent infiltrative macrophages was observed in various organs, such as the liver and bone marrow, indicating the hemophagocytic syndrome. The hemophagocytic syndrome characterized in the present case may have been responsible for the extremely rapid and fulminant course.
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PMID:A case of malignant lymphoma with hemophagocytic syndrome presenting as hepatic failure. 915 62

The promyelocytic blast crisis is a rare form of transformation during the evolution of chronic myeloid leukaemia (CML). We report a case of promyelocytic blast crisis with t(15;17) in addition to t(9;22). The morphology and immunophenotype of the blasts were similar to those seen in acute promyelocytic leukaemia (APL). The t(15;17) was confirmed by FISH. The patient had evidence of coagulopathy with clinical and laboratory findings of disseminated intravascular coagulation (DIC). This report highlights the importance of correlating the results of multiple diagnostic methods in order to establish a correct diagnosis of the promyelocytic blast crisis of CML.
Leuk Lymphoma 1998 Sep
PMID:Promyelocytic blast crisis of chronic myelogenous leukaemia with translocations (9;22) and (15;17). 972 Jul 33

A case of nasal type natural killer (NK)/T cell lymphoma of the subcutis showing clinical and morphological features that resemble subcutaneous panniculitis-like T cell lymphoma (SPTCL) is presented. A 73-year-old man presented with swelling of the left arm and was diagnosed with panniculitis by a dermatologist. It was concluded from a skin biopsy specimen that the patient had non-Hodgkin's lymphoma of the large cell, NK/T cell type because the neoplastic cells showed polyclonal CD3 immunoreactivity. Treatment with interferon-gamma was initiated, but the patient died of disseminated intravascular coagulation and multiple organ failure 2 months after the initial symptoms appeared. However, involvement of additional organs by the lymphoma was not apparent clinically. An autopsy was not performed. A routinely stained section of the biopsy skin specimen revealed massive necrosis of the subcutaneous fat, karyorrhexis admixed with reactive histiocytes, and large atypical lymphoid cells. Immunoreactivity for polyclonal CD3 was present in the perinuclear region, but absent in the neoplastic cell membranes. CD56, CD45RO (UCHL-1), CD43 (MT1), CD45 (leukocyte common antigen), and the cytotoxic molecules perforin, granzyme B and TIA-1 were positive, but CD20 (L26), CD4, CD8, and betaF1 were negative. Epstein-Barr virus (EBV) mRNA was detected in the nuclei of neoplastic cells by in situ hybridization. Subcutaneous panniculitis-like T cell lymphoma is reported to be an EBV-negative, clonal T cell neoplasm. Although this case showed clinical and morphological features that resembled SPTCL, perinuclear polyclonal CD3 staining and membranous CD56 reactivity seen in neoplastic cells were suggestive of NK cells. Furthermore, the neoplastic cells were positive for EBV. This case is considered to be a NK/T cell lymphoma of the subcutis resembling SPTCL. It is believed that it is important to recognize such a tumor because patients may undergo a fulminant clinical course, despite the tumor being localized in the subcutaneous adipose tissue.
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PMID:A case of natural killer/T cell lymphoma of the subcutis resembling subcutaneous panniculitis-like T cell lymphoma. 1033 81


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