Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019087 (hemorrhagic diathesis)
 678 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A study was conducted of 16 patients with acute promyelocytic leukemia. The most common oral manifestations were hemorrhagic diathesis and leukemic involvement. There was gingival bleeding, petechiae, and ecchymosis of the oral mucosa, massive infiltration-induced gingival swelling, ulcerative glossitis, swelling of the tonsils, and facial palsy. Inflammatory stomatitis also occurred. Patients with acute promyelocytic leukemia had a higher incidence and severity of oral bleeding than those with other types of acute leukemia. However, the oral findings in our series were not necessarily specific for acute promyelocytic leukemia because the same oral symptoms occur in other forms of acute leukemia.
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PMID:Oral manifestations of acute promyelocytic leukemia. 27 51

Seven patients affected by acute promyelocytic leukemia and presenting a hemorrhagic syndrome with hypofibrinogenemia have been treated with Daunorubicin and high doses of Glucocorticoids. In three out of the seven patients we also added an antiprotease agent (Trasylol, Bayer). Hemorrhagic diathesis has been controlled in all patients but one without massive platelet transfusions. A complete hematologic remission with prolonged survival was achieved in five patients.
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PMID:Disseminated intravascular coagulation in acute promyelocytic leukemia. Possibility of treatment with glucocorticoids at high doses. 28

A 60-year-old male with acute promyelocytic leukemia demonstrated bilateral diffuse air-space consolidation on chest X-ray. An autopsy on the next day revealed pure pulmonary alveolar hemorrhage without leukemic infiltration or inflammation. Disseminated intravascular coagulation was confirmed microscopically. In severe hemorrhagic diathesis and leukopenia, it is impossible to distinguish pulmonary hemorrhage from pneumonia by X-ray alone. Bronchalveolar lavage may be the only possible diagnostic approach.
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PMID:Diffuse pulmonary alveolar hemorrhage in acute promyelocytic leukemia. 163 46

The authors report four cases of intracranial hemorrhage associated with nontraumatic disseminated intravascular coagulation (DIC). Two cases demonstrated a sudden onset of intracerebral hemorrhage. The other two showed chronic subdural hematoma initially, followed by acute multiple intracranial hemorrhages or general hemorrhagic diathesis. The underlying disorders were glioblastoma multiforme, thoracoabdominal aortic aneurysm, acute promyelocytic leukemia, and stomach cancer associated with disseminated carcinomatosis of the bone marrow. All patients died eventually. When the underlying disorder has a rare incidence of DIC as in glioblastoma multiforme or thoracoabdominal aortic aneurysm, the possibility of DIC and the need for immediate initiation of replacement therapy should be recognized, although the mortality is very high because the underlying disorder cannot be eliminated quickly. When the underlying disorder has a high incidence of DIC as in acute promyelocytic leukemia or disseminated carcinomatosis of the bone marrow, it is mandatory to start replacement therapy and treatment for the underlying disorder simultaneously. DIC can be controlled when the treatment for the underlying disorder is effective.
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PMID:Intracranial hemorrhage associated with nontraumatic disseminated intravascular coagulation--report of four cases. 170 40

Acute promyelocytic leukemia (APL) is a rare subtype of acute myelogenous leukemia that is usually associated with a fatal hemorrhagic diathesis. All trans-retinoic acid (ATRA) is an active metabolite of vitamin A that differentiates the malignant cell clone, corrects the coagulopathy, and induces complete remission in the vast majority of patients with APL. Between June 1992 and September 1993, 8 patients with APL (4 previously untreated, 3 in first relapse and 1 in second relapse) received ATRA. Complete remission was achieved in 7 patients; in 5 with ATRA alone and in 2 with ATRA followed by cytotoxic chemotherapy due to the development of asymptomatic hyperleukocytosis. The earliest signs of response were the correction of the coagulopathy and an increase in the white blood cell count. Sequential morphological and immunophenotypical analyses of the bone marrow revealed differentiation of the malignant cell clone, in the absence of bone marrow hypoplasia. 4 of 5 patients treated only with ATRA until complete remission had late leukopenia. The most frequent adverse effects were dryness of skin and mucosae, hypertrigliceridemia and hypercholesterolemia, and a moderate increase in liver transaminases. An increase in the white blood cell count was common, and in two cases exceeded 35.0 x 10(9)/l. One of these patients developed multiple thrombosis of the extremities after cytotoxic chemotherapy. We frequently observed an increase in lactic dehydrogenase levels that was concomitant with the peak in the white blood cell count. The only patient on whom complete remission was not achieved was 60 years old, had chronic obstructive pulmonary disease, and died in the third week of therapy with a pulmonary distress syndrome.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Treatment of acute promyelocytic leukemia with trans-retinoic acid. Experience of the Santa Maria Hospital, Medical School of Lisbon]. 771 19

The syndrome of vitreous hemorrhage in association with any form of intracranial bleeding is known as Terson's syndrome. Acute promyelocytic leukemia (APL) constitutes 5% to 15% of cases of acute nonlymphocytic leukemias, in which hemorrhagic diathesis often occurs and results in a rapid fatal outcome. In this report we describe a patient with APL who developed cerebral bleeding in association with bilateral subhyaloid and vitreous hemorrhages consistent with Terson's syndrome while she was on all-trans retinoic acid induction therapy.
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PMID:Terson's syndrome in a patient with acute promyelocytic leukemia on all-trans retinoic acid treatment. 815 57

Acute promyelocytic leukemia (APL) is a rare subtype of acute myelogenous leukemia. It is frequently associated with a life-threatening hemorrhagic diathesis, often aggravated by induction cytotoxic chemotherapy. Patients with APL have bone marrow infiltration by abnormal promyelocytes, usually with prominent cytoplasmic granulation. These patients have a unique cytogenetic abnormality, a balanced reciprocal translocation between the long arms of chromosomes 15 and 17. The nuclear retinoic acid receptor alpha gene, on chromosome 17, is translocated to the PML gene region, on chromosome 15, resulting in the synthesis of two fusion messenger ribonucleic acids, PML/RAR-alpha and RAR-alpha/PML, easily detected by the reverse transcriptase polymerase chain reaction. This assay is extremely useful in the diagnosis and detection of minimal residual disease in APL patients. All trans-retinoic acid (ATR) differentiates the malignant cell clone and corrects the coagulopathy associated with this disease. The most important adverse effect is a respiratory distress syndrome, treatable with steroids, if detected at its onset. ATR yields durable remissions in patients with APL, after consolidation with cytotoxic chemotherapy.
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PMID:[Acute promyelocytic leukemia. The therapeutic advances]. 828 19

Acute promyelocytic leukaemia (APL) is characterised by a life-threatening hemorrhagic diathesis which is attributed to a DIC-like coagulopathy. This report describes the problems of childbirth in two patients with untreated APL. It is concluded that caesarean section can be performed without major complications. A prerequisite is an active treatment of the coagulopathy and a close collaboration between the obstetrician and the haematologist.
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PMID:Caesarean section in previously untreated acute promyelocytic leukaemia. Report of two patients. 1081 77

The bleeding syndrome of acute promyelocytic leukemia (APL) is complex and consists of disseminated intravascular coagulation (DIC) and hyperfibrinolysis. Elastase, derived from malignant promyelocytes, is believed to mediate the fibrinogeno- and fibrinolysis by aspecific proteolysis. In this study we measured the role of elastase in fifteen patients with APL by using an assay for elastase degraded fibrin(ogen) and the results were compared with those obtained in patients with sepsis induced DIC. High levels of elastase were observed in sepsis and APL. The levels of fibrinogen and fibrin degradation products were significantly higher in APL patients compared to patients with sepsis induced DIC. Nevertheless, the level of elastase degraded fibrin(ogen) was higher in the sepsis group (635.3 ng/ml, compared to 144.3 ng/ml in APL; p <0.0001). So, the enormous increase in fibrin and fibrinogen degradation products in APL cannot be explained by elastase activity. This study suggests a minor role for elastase mediated proteolysis in the hemorrhagic diathesis in APL patients.
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PMID:Elastase mediated fibrinolysis in acute promyelocytic leukemia. 1089 47

The most impressive clinical feature of acute promyelocytic leukemia (APL) at diagnosis is the presence in 80% to 90% of patients of a severe hemorrhagic syndrome. Recent data favor a fibrinolytic/proteolytic process rather than a disseminated intravascular coagulation as the mechanism mainly responsible for the hemorrhagic diathesis in APL. Morphologically, two main cytologic variants have been Identified: the classical hypergranular APL (M3), which represents the great majority of all APL, and the microgranular variant (M3v), which accounts for about 15% to 20% of all APL. A rare basophilic variant has also been described. With regard to prognosis, it has markedly changed from that of a rapidly fatal acute leukemia to that of a highly curable disease. This revolutionary progress was mainly due to the introduction during the 1990s of all-trans retinoic acid (ATRA) for the treatment of this disease. After the introduction of ATRA, in addition to clinical features such as hyperleukocytosis (white blood cell count > 10 x 10(9)/L) or thrombocytopenia (platelet count < 10 x 10(3)/L) at presentation, immunophenotype markers and polymerase chain reaction status for promyelocytic leukemia/retinoic acid receptor-alpha during follow-up also had an impact on prognosis.
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PMID:Acute promyelocytic leukemia: clinical and morphologic features and prognostic factors. 1117 35


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