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1/25. Epithelioid hemangioendothelioma of the liver associated with thrombocytopenia and coagulopathy.

    Epithelioid hemangioendothelioma of the liver is a rare vascular neoplasm with intermediate malignant potential. The prognosis is highly unpredictable. We report the case of a 59-year-old woman who had the tumor radically resected, but multiple metastases of the liver developed associated with thrombocytopenia and consumption coagulopathy, as observed in kasabach-merritt syndrome. The patient did not respond to any treatment and the behavior of the tumor was very aggressive. The patient died 15 months after radical resection of the tumor.
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2/25. quinine-induced disseminated intravascular coagulation: case report and review of the literature.

    OBJECTIVE: To describe the clinical course of quinine-induced disseminated intravascular coagulation (DIC) and review all previous cases reported in the medical literature. DESIGN: Case report/literature review. SETTING: University teaching hospital medical ICU. patients: One patient in whom thrombocytopenia, coagulopathy, intravascular hemolysis, DIC, and acute renal failure temporally followed the ingestion of quinine. DATA SOURCES: We conducted a computerized free-text medline database search from 1969 to 2000 using the keywords quinine and thrombocytopenia, quinine and hemolytic-uremic syndrome, and quinine and disseminated intravascular coagulation. STUDY SELECTION: All reported cases and reviews of quinine-induced thrombocytopenia, hemolytic-uremic syndrome (HUS), and DIC were reviewed. DIC was distinguished from quinine-induced thrombocytopenia or quinine-induced HUS based on the presence of abnormal clotting times, elevated fibrin degradation products, and/or elevated D-dimer levels. DATA SYNTHESIS: Fifteen previous patients were found to meet the criteria for DIC temporally related to the recent ingestion of quinine. The clinical course and laboratory abnormalities documented for each case are reviewed. CONCLUSIONS: quinine-induced DIC is a distinct clinical entity, which may present as unexplained thrombocytopenia, coagulopathy, or renal failure. In susceptible patients, the immune response to quinine may result in the production of not only anti-platelet antibodies but also antibodies against leukocytes, erythrocytes, and endothelial cells. Furthermore, the varying patterns and specificities of antibody production in an individual patient may result in a spectrum of clinical disease from mild, transient thrombocytopenia to overt intravascular hemolysis, renal failure, coagulopathy, and DIC. Early recognition of quinine-induced DIC is paramount, as this diagnosis affords a better prognosis than other adult forms of HUS or DIC.
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ranking = 1
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3/25. endophthalmitis with klebsiella pneumoniae liver abscess.

    Endogenous endophthalmitis is a rare, but devastating complication of septicemia. The prognosis of maintaining visual acuity in patients with septic endophthalmitis is poor in spite of an early diagnosis and the timely start of conventional therapeutic procedures because the intravitreous drug concentration remains low after the systemic administration of antibiotics due to the blood-ocular barrier. We treated an elderly female patient with endogenous endophthalmitis complicated with disseminated intravascular coagulation associated with a klebsiella pneumoniae liver abscess. endophthalmitis developed rapidly and we thus had to perform an enucleation of both eyeballs even though we made an early diagnosis and performed liver abscess drainage as well as the prompt systemic and subconjunctival administration of antibiotics. Our experience in treating this case emphasizes the need to perform the timely intravitreous infusion of antibiotics with a support therapy consisting of the systemic and subconjunctival administration of antibiotics for endogenous endophthalmitis associated with a klebsiella pneumoniae liver abscess.
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keywords = prognosis
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4/25. Thoracic lymphangiomatosis in a child.

    An 8-year-old boy who presented with a mediastinal mass, pulmonary infiltrates, and disseminated intravascular coagulation was diagnosed with lymphangiomatosis. Despite medical management, he developed multiple organ failure and died. The authors discuss the diagnostic findings, medical management, and pathology and review 52 additional cases of thoracic lymphangiomatosis from the literature. patients presented with chylothorax (49%), a mass (47%), pulmonary infiltrates (45%), bone lesions (39%), splenic lesions (19%), cervical involvement (15%), disseminated intravascular coagulation (9%), and skin involvement (7%). Children (<16 years) had a worse prognosis than older patients (39% vs. 0% mortality). All patients who died had either parenchymal lung involvement or pleural effusion. Thoracic lymphangiomatosis should be included in the differential diagnosis of a mediastinal mass with pulmonary findings.
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keywords = prognosis
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5/25. A fetal case of transient abnormal myelopoiesis with severe liver failure in down syndrome: prognostic value of serum markers.

    The authors recently encountered a lethal case of down syndrome with transient abnormal myelopoiesis (TAM). Although the peripheral white blood cell count and blast cells had improved without specific treatment, the patient died of severe coagulopathy due to liver fibrosis when he was 5 years old. The prognosis of TAM with liver fibrosis was poor. The patient had high levels of N-terminal peptide of III procollagen, type IV collagen, and hyaluronic acid. These serum makers are noninvasive indicators of liver fibrosis and may be useful as prognostic indicators of TAM in down syndrome.
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ranking = 1
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6/25. Advanced gastric cancer and a concomitant pregnancy associated with disseminated intravascular coagulation.

    Gastric cancer associated with pregnancy is extremely rare and the prognosis is generally grave. A 31-year-old Japanese women, 41 weeks pregnant, displayed disseminated intravascular coagulation (DIC), although clinical symptoms and diagnostic examinations did not indicate an obstetrical cause. She went into labor spontaneously and vaginally delivered a 3248-g normal female infant, after receiving a blood transfusion. On the day 5 postpartum, a gastroduodenal fiberscope examination indicated advanced gastric cancer. She was also diagnosed with bilateral chronic subdural hematoma and underwent an operation to allow drainage. It was not possible to treat her curatively, so she was treated conservatively for DIC. She died on day 13 postpartum. Necropsy of the iliac bone indicated bone marrow metastasis of adenocarcinoma. This is the first known case of a pregnant woman with DIC occurring as the first manifestation of advanced gastric cancer.
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ranking = 1
keywords = prognosis
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7/25. Primary antiphospholipid syndrome and chronic disseminated intravascular coagulation: the differences and the similarities.

    A middle aged woman with a medical history of recurrent spontaneous abortions and chronic leg ulcers presented with a pulmonary embolism and inferior vena caval thrombosis. Primary antiphospholipid syndrome (PAPS) was diagnosed by evidence of extremely high titers of anticardiolipin IgG and IgM, typical clinical features and the absence of other autoimmune diseases. Multiple coagulation parameters suggested chronic disseminated intravascular coagulation (DIC). It is important to distinguish PAPS from true chronic DIC as the underlying causes, treatment and prognosis differ greatly. In describing this case of PAPS presenting hematologically as a chronic DIC, we offer a discussion of the means to distinguish between these two coagulopathies and briefly discuss their treatments.
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ranking = 1
keywords = prognosis
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8/25. OPSI (overwhelming postsplenectomy infection) syndrome: a case report.

    Splenectomized patients are likely to suffer from severe infections, such as sepsis and meningitis. This syndrome is called overwhelming postsplenectomy infection (OPSI) in europe and America. We present an adult case of OPSI syndrome, which occurred as respiratory insufficiency, and thrombocytopenia. The course is rapid, the clinical symptoms are serious, and the prognosis is very poor. Clinical examination showed cyanosis, mandibular hypertonia, psychomotor anxiety and purpura. Laboratory findings were thrombocytopenia, leukocytosis, hypoglycemia and altered coagulation parameters. A chest X-ray showed right pulmonary aspecific thickening. The autopsy findings occurred as waterhouse-friderichsen syndrome.
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ranking = 1
keywords = prognosis
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9/25. Severe ocular involvement in disseminated intravascular coagulation complicating meningococcaemia.

    BACKGROUND: disseminated intravascular coagulation (DIC) is characterized by inappropriate widespread activation of coagulation leading to extensive microvascular thrombosis and haemorrhage. Ocular involvement typically manifests as fibrin-platelet clots in the choroidal vessels of the posterior pole with overlying serous retinal detachment and sparing of the retinal vessels. methods: Case report. RESULTS: An 18-year-old female with meningococcal septicaemia and DIC developed bilateral dense vitreous haemorrhage. At vitrectomy multiple areas of sub-internal limiting membrane haemorrhage were evacuated and silicone oil injected. The patient developed bilateral large macular holes with inferior tractional retinal detachment. Two years later, final visual acuity was 6/36 OD and count fingers OS. CONCLUSION: Ocular involvement in DIC can be more extensive than choroidal vascular occlusion alone. Previous reports suggest that DIC associated with sepsis (especially meningococcaemia) may present with more severe manifestations. This may be due to sub-clinical endophthalmitis or haemorrhagic ischemia. Visual prognosis can be poor.
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ranking = 1
keywords = prognosis
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10/25. "Children on the frontline against E. coli": typical hemolytic-uremic syndrome.

    A thirteen-month old infant presented with classical hemolytic-uremic syndrome (HUS), but with negative cultures for escherichia coli (E. coli) 0157:H7. HUS is commonly linked to infection with E. coli 0157:H7; however, traditional culture has demonstrated poor sensitivity. Pathogenesis of the organism in HUS involves the production of a Shiga-like toxin (STX), resulting in a triad of symptoms. An early and accurate differential diagnosis, based on patient presentation with acute renal failure, hemolytic anemia, and thrombocytopenia, is critical for supportive treatment and improved prognosis. Patient prognosis is related to the duration of renal failure and dialysis treatment. research is aimed at improved detection of E. coli 0157:H7 or the STX produced, and future vaccination to eliminate typical HUS.
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ranking = 2
keywords = prognosis
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