Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0010200 (cough)
 23,843 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A woman with scleroderma and classic polyarteritis nodosa (PAN) who developed idiopathic myelofibrosis (IM) is reported. The patient presented with a one-year history of weakness, polyarthritis, Raynaud phenomenon, dry cough, and epigastralgia. The diagnosis of scleroderma with visceral involvement was made and treatment with prednisone subsequently started, with good clinical response. Six years later, fever, weight loss, livedo reticularis, and dysesthesias developed. Electromyographic studies were consistent with sensory neuropathy and a sural nerve biopsy yielded the diagnosis of PAN. The patient received cyclophosphamide plus prednisone with a favorable response, but 11 years later she was admitted because of weakness, constitutional symptoms, and abdominal pain due to spleen infarcts. Marked anemia, with aniso-poikilocytosis, tear-drop cells, immature myeloid precursors in the peripheral blood, and an increased serum LDH, was observed and the diagnosis of IM established by bone marrow biopsy. This case represents a new association between IM and an autoimmune disease and supports the hypothesis of an immune basis of IM in some patients.
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PMID:Idiopathic myelofibrosis associated with classic polyarteritis nodosa. 1268 29

A 62-year-old female presented with a 1-month history of irritating cough and increasing dyspnea. A chronic idiopathic myelofibrosis had been diagnosed 5 years ago. CT of the chest and abdomen showed bilateral pleural effusions with a thickened pleura, nodular infiltrations in both lungs, enlarged intraabdominal lymph nodes and splenomegaly. Pleuroscopy (medical thoracoscopy) on the left side revealed dense tumorous nodules mainly on the posterior chest wall pleura, but also on the diaphragm and the lung. Biopsies taken from the chest wall pleura revealed extramedullary hematopoiesis (EMH) with abnormal megakaryocytes as well as myeloid and erythroid precursors. After unsuccessful tetracycline pleurodesis, talcum slurry was instilled via the chest tube without recurrence of the pleural effusion. Furthermore, treatment with hydroxyurea was started, and the disease regressed and then remained stable over the next 24 months. In conclusion, the pleuropulmonary findings were caused by EMH due to chronic idiopathic myelofibrosis. The definite diagnosis was established by pleuroscopy followed by successful pleurodesis with talc slurry, after tetracycline pleurodesis had failed.
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PMID:A 62-year-old woman with bilateral pleural effusions and pulmonary infiltrates caused by extramedullary hematopoiesis. 1867 16

The Myeloproliferative Neoplasm Symptom Assessment Form (MPN-SAF) is a validated quality of life (QoL) instrument. In our Swedish cohort of 114 patients the symptomatic burden was found to be severe, with fatigue reported in 88% of the patients and reduced QoL in the majority of patients. Patients with primary myelofibrosis had the highest scores, low QoL, for most MPN-SAF items, compared to patients with polycythemia vera and essential thrombocythemia. Higher age showed significant associations with the BFI (Brief Fatigue Inventory) score, early satiety, concentration problems, dizziness, insomnia, cough and weight loss. Blood values, disease duration and myelosuppressive treatment did not significantly associate with any of the MPN-SAF items, with the exception of higher hemoglobin, which correlated with sad mood. Male patients with MPN scored significantly higher as regards sexual problems and weight loss compared to female patients. Overall, the MPN-SAF was found to be a valid instrument for assessing symptomatic burden among this population.
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PMID:Association between quality of life and clinical parameters in patients with myeloproliferative neoplasms. 2188 29

The quality of life (QoL) at the time of diagnosis of myeloproliferative neoplasm (MPN) has, to date, not been studied. One hundred and seventy-nine patients with MPN: 80 with essential thrombocythemia (ET), 73 with polycythemia vera (PV), 22 with primary myelofibrosis (PMF) and four with MPN undifferentiated, were included in this study. European Organisation for Research and Treatment of Cancer Quality of Life Questionnaire Core 30 (EORTC-QLQC30) and the MPN-Symptom Assessment Form (MPN-SAF) were used to evaluate QoL. Fatigue was the most reported symptom in these patients. Patients with PV reported significantly higher mean scores for inactivity, dizziness, cough, itching, depression and lower total QoL compared to patients with ET. Patients with PV had significantly more headache and itching compared to patients with PMF. When the newly diagnosed patients with MPN were compared with a cohort of patients with MPN with mean disease duration of 7.8 years, the differences were most striking for patients with PMF, with significantly more fatigue, abdominal discomfort, concentration problems, insomnia, fever, weight loss and lower overall QoL developed over time.
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PMID:Patients with polycythemia vera have worst impairment of quality of life among patients with newly diagnosed myeloproliferative neoplasms. 2339 6