American journal of hematology
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Inhaled nitric oxide has been demonstrated to improve oxygenation in critically ill patients requiring mechanical ventilation. We therefore performed a retrospective review to determine the outcome of patients with hematological malignancies and acute respiratory failure who received inhaled nitric oxide (INO) in a multidisciplinary intensive care unit of a single tertiary referral medical center. Thirteen patients with hematological malignancies who required endotracheal intubation and mechanical ventilation and received INO for acute respiratory failure between January 1998 and December 2002 were identified. ⋯ Patients with hematological malignancies and acute respiratory failure to whom INO was administered had clinical deterioration since ICU admission. Despite a marked initial improvement in arterial oxygen tension, all patients ultimately died in the intensive care unit, 8 of them within 48 h of initiating INO. Therefore, despite initial improvement in oxygenation, we did not observe any survival benefit to INO in this setting.
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The histopathologic features characterizing the involvement of the bone marrow (BM) in systemic lupus erythematosus (SLE) have not been systematically analyzed to date. ⋯ BMB in patients with SLE and unexplained cytopenias presents a variety of histopathologic findings including BM necrosis, stromal alterations, hypocellularity, dyspoiesis, and distortion of normal BM architecture, characterized primarily by the presence of ALIP aggregates.
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Case Reports Comparative Study
Cerebrovascular disease associated with sickle cell pulmonary hypertension.
In patients with sickle cell disease, anemia is a recognized risk factor for stroke, death, and the development of pulmonary hypertension. We have proposed that hemolytic anemia results in endothelial dysfunction and vascular instability and can ultimately lead to a proliferative vasculopathy leading to pulmonary hypertension. Consistent with this mechanism of disease, we now report a case series of six patients with obliterative central nervous system vasculopathy who also have pulmonary hypertension and high hemolytic rate. ⋯ A review of the literature suggests that many clinical, epidemiological, and physiological features of the arteriopathy of pulmonary hypertension closely overlap with those of stroke in sickle cell disease, both known to involve proliferative vascular intimal and smooth muscle hypertrophy and thrombosis. These cases suggest that cerebrovascular disease and pulmonary hypertension in sickle cell disease share common mechanisms, in particular, reduced nitric oxide bioactivity associated with particularly high-grade hemolysis. Clinicians should suspect occult cerebrovascular disease in sickle cell patients with pulmonary hypertension.
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A 67-year-old man with acute myeloid leukemia (AML) was treated with low-dose decitabine. He achieved a complete remission (CR) after two cycles of therapy, and he remained in remission during 1 year of treatment. ⋯ He was retreated with decitabine and again achieved a CR, which has been maintained for 6 months. This case demonstrates that durable responses can occur upon retreatment with decitabine.
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Clinical Trial
Prolonged overall survival with second on-demand autologous transplant in multiple myeloma.
Between August 1993 and March 2003, 130 consecutive multiple myeloma (MM) patients eligible for high-dose treatment were offered a program including up-front autologous stem cell transplantation (ASCT) after conditioning with 200 mg/m(2) melphalan followed by a second ASCT in case of relapse or progression. A total of 107 (82%) patients completed the first ASCT. The best response obtained after ASCT was complete response (CR) 23%, very good partial response (VGPR) 28%, partial response (PR) 42%, and minimal response (MR) 7%. ⋯ Median OS and EFS after the second ASCT were 38.1 and 14.8 months. Treatment-related mortality was 1.9% after the first ASCT but no deaths occurred after the second. Our experience suggests that elective up-front single ASCT followed by second ASCT after relapse or progression is a safe and effective global strategy to treat MM patients.