Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0010200 (cough)
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Retinoic acid syndrome (RAS) is the clinical syndrome that occurs after treatment of acute promyelocytic leukemia with all-trans-retinoic acid (ATRA). The patients experience fever, dyspnea, hypotension, respiratory distress, edema and weight gain. Chest x-ray will show pulmonary infiltrates and pleuropericardial effusion. The onset of this syndrome is usually 5-21 days after ATRA treatment when white blood cell counts are rising more than 10,000/cu.mm. The authors have reported a case of RAS. The patient was a 29-year-old man who had been working in a battery manufacturing factory for 7 years. He presented with easily bruising for one month. The initial blood test showed hematocrit of 36.2%, white blood cells count of 3,200/cu.mm with 28% neutrophils, 20% lymphocytes, 2% eosinophils and 50% promyelocytes and platelet of 20,000/cu.mm. Peripheral blood smear revealed numerous fragmented red blood cells. Bone marrow examination showed hypercellularity with abnormal promyelocytes of 95% and bone marrow cytogenetics was translocation of chromosome 15 and 17 [t (15;17)(q22;q12)]. The diagnosis was acute promyelocytic leukemia and the patient was treated with ATRA 45 mg/m2/day per oral starting on day 1 and intravenous idarubicin 10 mg/n2 on day 4, 5 and 6. On day 13, he had a body temperature of 39 degrees C and a dry cough. The white blood cells were rising to 7,400/cu.mm with 16% neutrophils. On day 18, he had oliguria, high grade fever, hypotension, cough with chest pain and white blood cells rose to 21,300/cu.mm with 65% neutrophils and rising of blood urea nitrogen and creatinine. Chest x-ray showed enlarged cardiac shadow with pleural effusion. Echocardiogram revealed moderate amount of pericardial effusion. The diagnosis of RAS was made and ATRA was withdrawn. Intravenous dexamethasone 4 mg every 6 hours and hemodialysis was started. The patient's symptoms improved dramatically and bone marrow examination was in complete remission. He was subsequently given cytarabine and idarubicin as consolidation. This patient had clinical manifestation consistent with RAS, which improved after prompt treatment.
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PMID:Successful treatment of retinoic acid syndrome with dexamethasone: a case report. 1685 73

Various acute pulmonary complications may occur in patients with hematologic malignancies because they are in an immunocompromised state due to systemic disease or to chemotherapy or hematopoietic stem cell transplantation. Pulmonary complications may arise from other treatment regimens, as well, or from direct pulmonary involvement in the malignant disease process. The differential diagnosis of pulmonary opacities in patients with hematologic malignancies is broad and includes both infectious and noninfectious causes. Pulmonary hemorrhage, edema, leukostasis, and pneumonia are well-known and common acute pulmonary complications. Less common complications are now encountered with increasing frequency because of the increasing complexity of therapeutic regimens for hematologic malignancies, which may include various drugs that are in clinical trials or were recently released to market. These once uncommon acute pulmonary complications include retinoic acid syndrome, tyrosine kinase inhibitor-induced pulmonary complications, engraftment syndrome, and hemophagocytic lymphohistiocytosis. It is often difficult to differentiate between these entities. However, the clinical setting and radiologic imaging findings may provide clues for interpreting imaging findings of abnormal pulmonary opacity in patients with a hematologic malignancy. Pulmonary hemorrhage is characterized by a sudden onset of symptoms and rapid progression of pulmonary imaging abnormalities and usually occurs in patients with impaired coagulation or a predisposition to bleed. Pulmonary edema should be considered when typical findings of hydrostatic pulmonary edema are seen. Pulmonary leukostasis develops in patients with hyperleukocytosis and leads to symptoms such as a cough, fever, and dyspnea. Various types of pneumonia may develop, depending on the degree and duration of immunosuppression in the patient. Retinoic acid syndrome, tyrosine kinase inhibitor-induced pulmonary complications, and engraftment syndrome occur after specific treatments, so a detailed medical history including recent or current treatments may be helpful for diagnosis. Accurate differentiation of these entities allows their appropriate management, with resultant decreases in morbidity and mortality.
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PMID:Acute pulmonary complications in patients with hematologic malignancies. 2531 Apr 29