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Query: UMLS:C0019079 (hemoptysis)
 6,129 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We report on two cases of pulmonary lymphangiomyomatosis, one of which was diagnosed by chance. Retroperitoneal lymphomas were found during a routine "check-up" and biopsy proved a lymphangiomyomatosis (LAM). High resolution CT of the lung was typical for pulmonary LAM. The other patient was examined because of a progressive dyspnea and hemoptysis. We compared the high-resolution CT of the early manifestation of LAM with that of a progressive disease. In both, the results of the high-resolution CT were pathognomonic.
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PMID:[High-resolution CT in the early and late form of lymphangioleiomyomatosis]. 788 33

A 22-year-old woman presented with left pelvic pain and mass. Ultrasonography confirmed a multilocular left adnexal mass containing cysts of varying sizes. The patient had no pulmonary symptoms at the time of presentation. The mass was surgically excised and pathologic diagnosis of lymphangioleiomyomatosis (LAM) was made. Subsequently, she developed hemoptysis and pleural effusion. High-resolution computed tomography of the chest showed findings consistent with LAM. Early diagnosis and treatment for LAM was possible in this atypical case.
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PMID:Pelvic lymphangioleiomyomatosis. Atypical precursor to pulmonary disease. 792 9

The diagnosis of lymphangioleiomyomatosis was established in a 35-year-old woman with hemoptysis, mild cough, and dyspnea based on histologic review of results of a transbronchial biopsy correlated with high-resolution computed tomographic scan findings. A chest x-ray film revealed diffuse interstitial lung disease, and a high-resolution computed tomographic scan showed diffuse cystic changes throughout both lungs. The transbronchial biopsy specimen revealed cystic changes and a patchy, sometimes nodular proliferation of smooth muscle that focally expanded the interstitium suggestive of lymphangioleiomyomatosis. The smooth-muscle nature of the cells was confirmed by positive immunohistochemical stains for actin and desmin; positive staining for HMB-45 was also observed. Although the diagnosis of lymphangioleiomyomatosis usually requires an open lung biopsy, this case shows that rarely, in the appropriate clinical setting, the diagnosis may be rendered based on results of a transbronchial biopsy in conjunction with findings from ancillary immunohistochemical studies and high-resolution computed tomography.
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PMID:Pulmonary lymphangioleiomyomatosis. Diagnosis based on results of transbronchial biopsy and immunohistochemical studies and correlation with high-resolution computed tomography findings. 806 Feb 40

A case of pulmonary lymphangiomyomatosis in a 28-year-old woman with dyspnea, hemoptysis, chylothorax and irregular menstruation as presenting symptoms was reported. Chest CT scan showed the presence of pleural effusion on right side and a honeycomb pattern throughout the lung parenchyma bilaterally. The diagnosis was confirmed histologically by transbronchial lung biopsy. Medroxyprogesterone was administered; improvement in arterial blood gas analysis and reduction of the amount of pleural effusion were noted after one month. The etiology, clinical manifestations, pathological characteristics and treatment of this disease were discussed.
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PMID:[Pulmonary lymphangiomyomatosis: report of a case]. 826 59

Lymphangiomyomatosis (LAM) is a progressive disease afflicting women of childbearing age. LAM remains a rare disease, unfamiliar to many clinicians. It usually poses a diagnostic dilemma for the primary physician, possibly resulting in a delayed or missed diagnosis, unnecessary operative intervention, and inappropriate therapy. LAM is characterized microscopically by abnormal smooth muscle proliferation causing gradual obstruction of small airways, lymphatics, and vasculature. The proliferation eventually results in a characteristic clinical syndrome of progressive dyspnea, recurrent pneumothorax, chylous effusion, and hemoptysis. Despite a variety of treatment regimens developed since the first description of LAM, patient survival has not improved appreciably. Most patients die within 10 years of the time of diagnosis. This report presents a patient with LAM and a review of the literature.
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PMID:Lymphangioleiomyomatosis. 832 71

A 34 year old women was admitted to the hospital with a 9 year history of intermittent haemoptysis associated with increasing breathlessness. A working diagnosis of lymphangioleiomyomatosis was made, based on clinical, radiological and histological findings. Three years later, the patient was admitted to hospital with worsening haemoptysis, which rapidly progressed and resulted in death from massive pulmonary haemorrhage. Postmortem examination and histology revealed findings consistent with multiple mesenchymal cystic hamartomas of the lungs. This is a rare condition which has previously been described as having a good prognosis. This case is the first fatality resulting directly from the disease to be reported.
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PMID:Fatal haemorrhage from mesenchymal cystic hamartoma of the lung. 866 15

A review is presented of the clinical and morphological manifestations of lymphangioleiomyomatosis (LAM), a systemic disorder of unknown etiology that affects women. The clinical features include dyspnea, hemoptysis, recurrent pneumothorax, chylothorax, and chylous ascites. It is characterized by: 1) proliferation of abnormal smooth muscle cells (LAM cells) in pulmonary interstitium and along the axial lymphatics of the thorax and abdomen; 2) thin-walled pulmonary cysts, and 3) a high incidence of angiomyolipomas. The pulmonary cystic lesions have a characteristic appearance on high resolution computed tomography. The most specific method for diagnosing LAM is lung biopsy to demonstrate the presence of LAM cells, either by their characteristic histological appearance or by specific immunostaining with HMB-45 antibody. LAM cells differ in several important respects from the types of smooth muscle cells normally present in lung. Their reactivity with HMB-45 antibody is localized in stage I and stage II melanosomes. LAM cells show additional evidence of incomplete melanogenesis, and the significance of these observations remains to be determined. Two types of LAM cells are recognized: 1) small, spindle-shaped cells that are centrally located in the LAM nodules and are highly immunoreactive for matrix metalloproteinase-2 (MMP-2), its activating enzyme (MT-1-MMP), and proliferating cell nuclear antigen (PCNA), and 2) large, epithelioid cells that are distributed along the periphery of the nodules and show a high degree of immunoreactivity with HMB-45 antibody and with antibodies against estrogen and progesterone receptors. Types of treatment used for LAM include oophorectomy, administration of Lupron or progesterone and in very severe cases, pulmonary transplantation (following the onset of respiratory insufficiency, not relieved by O(2)).
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PMID:Lymphangioleiomyomatosis (LAM): a review of clinical and morphological features. 1103 60

Pulmonary lymphangioleiomyomatosis (LAM) is a rare, serious, chronic disease whose etiology is unknown and which affects young women almost exclusively. It produces typical clinical and radiological characteristics. Diagnosis is confirmed on finding histological evidence of irregular proliferation of the smooth muscle cells of the lungs. Treatment is not usually effective and there is rapid worsening of the pulmonary function leading to serious respiratory failure which often results in the patients death. We present two cases of LAM in women, one of whom was 33 and the other 38. In the first case the patient had all the pulmonary findings described in the literature, while the second also had bilateral renal angiomyolipomas. We discuss the histological, radiological and clinical characteristics and evolution of both cases, as well as the treatment given. We also review the literature. It is concluded that LAM should be suspected in young women who have dyspnea, cough and/or hemoptysis and an interstitial radiological pattern, especially when associated with a pleural effusion or pneumothorax.
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PMID:[Pulmonary lymphangioleiomyomatosis. Description and course of 2 cases]. 1132 35

Lymphangioleiomyomatosis is a rare and complicated disorder that affects the young, almost exclusively women. It may be associated with the tuberous sclerosis complex, which includes renal angiolipoma, chylothorax and lymph node myomatosis. Its clinical pulmonary manifestations vary from simple cough to the development of recurrent pneumothoraces, hemoptysis, and even complicated pleural effusions. Progressive dyspnea develops as the disease evolves. Eventually most patients require lung transplantation. This wide array of symptoms and signs makes the differential diagnosis extensive, and the clinician must be familiar with this disorder to arrive promptly to the correct diagnosis. We report a case of a 36-year-old woman with a long history of recurrent pleuritic chest pain with associated dyspnea before being diagnosed with lymphangioleiomyomatosis. A review of the literature pertinent to this case is provided.
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PMID:Lymphangioleiomyomatosis: an unusual cause of dyspnea in young women. 1201 58

A 29-year-old woman was admitted to our hospital with a 7-day history of elevated temperature to 39.5 degrees C associated with headache and nausea. She had been diagnosed with tuberous sclerosis complex 10 years earlier. Her unconsciousness progressed, and she was diagnosed as having aseptic meningoencephalitis. The next day, she had a generalized seizure with severe hemoptysis, and she suddenly fell into severe respiratory failure (PaO2/FiO2 = 76.9). Transbronchial lung biopsy revealed the findings of lymphangioleiomyomatosis. It was suggested that neurogenic pulmonary edema accompanied with venous flow obstruction by lymphangioleiomyomatosis lesions resulted in diffuse pulmonary hemorrhage with resultant gross hemoptysis accelerating to severe hypoxemia.
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PMID:Acute respiratory failure with gross hemoptysis in a patient with lymphangioleiomyomatosis as part of tuberous sclerosis complex. 1546 81


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