Edit concept Question Editor Create issue ticket

Diffuse Pulmonary Lymphangiomatosis


Presentation

  • The patients comprised seven males and two females with a mean age at presentation of 10 years (age range, 1 month to 33 years). Six patients were younger than 10 years of age.[ncbi.nlm.nih.gov]
  • The authors present the case of a 48-year-old man with diffuse pulmonary lymphangiomatosis. This rare lymphatic disorder is characterized by proliferation of anastomosing lymphatic vessels varying in size.[ncbi.nlm.nih.gov]
  • The present article discusses clinical, radiographic and pathological features, and treatment options for DPL.[ncbi.nlm.nih.gov]
  • Here, we report a case involving a 52-year-old asymptomatic woman who presented with increased interstitial markings, as seen on a chest radiograph.[ncbi.nlm.nih.gov]
  • Despite the poor prognosis of the disease, 5 years after the treatment the patient has not presented new episodes of pleuropericardial effusions, nor has he required hospitalization for other symptoms, currently presenting only exertional dyspnea.[archbronconeumol.org]
Recurrent Pleural Effusion
  • Clinical presentations varies from mild wheezing and moderate cough to marked respiratory insufficiency due to progressive in infiltrative disease and recurrent pleural effusions ( 14 ).[atm.amegroups.com]
  • Palliative procedures include medical therapy with interferon-alpha, draining the pleural effusions, or performing pleurodesis for recurrent pleural effusions. 5 Acknowledgement The authors thank Dr. G. Brink for review of the manuscript.[ispub.com]
Recurrent Pleural Effusion
  • Clinical presentations varies from mild wheezing and moderate cough to marked respiratory insufficiency due to progressive in infiltrative disease and recurrent pleural effusions ( 14 ).[atm.amegroups.com]
  • Palliative procedures include medical therapy with interferon-alpha, draining the pleural effusions, or performing pleurodesis for recurrent pleural effusions. 5 Acknowledgement The authors thank Dr. G. Brink for review of the manuscript.[ispub.com]
Pleural Effusion
  • Nineteen patients had interlobular septal thickening, 18 had diffuse ground-glass opacities, 22 had pleural effusion, 14 had extrapleural soft-tissue thickening, 20 had pericardial effusion and thickened pericardium, 10 had multiple lymphadenopathy in[ncbi.nlm.nih.gov]
  • Seven of the eight had bilateral pleural effusions or smooth thickening of the pleura (or both), and two had regions of calcified pleural thickening.[ncbi.nlm.nih.gov]
  • effusion and a pericardial effusion.[unboundmedicine.com]
  • Definition / general Diffuse proliferation of abnormal, complex lymphatic channels (lymphangiomas) that may involve lung or other organs ( Lymphat Res Biol 2011;9:191 ) Pulmonary involvement often accompanied by chylous pleural effusions May be progressive[pathologyoutlines.com]
  • CT chest Described features include 4 increased interlobular septal thickening peribronchovascular thickening patchy ground glass opacities pleural thickening pleural effusion mediastinal soft tissue infiltration lymphangioleiomyomatosis Promoted articles[radiopaedia.org]
Dyspnea
  • He came to the emergency unit due to dyspnea with minimal exertion that had been evolving over the period of a month.[archbronconeumol.org]
  • Symptoms included "wheezing" or "asthma" (44%) and dyspnea (22%) present for 3 months to 20 years. Chest radiographs showed bilateral interstitial infiltrates, often greatest in the lower lobes.[ncbi.nlm.nih.gov]
  • ., ribs, vertebrae, humerus, femur), liver, and skin Presentation Dyspnea Cough Hemoptysis Chest pain Milky sputum Pleural effusions Mediastinal enlargement Laboratory Tests Pulmonary functional tests demonstrate Mixed pattern obstructive/restrictive[basicmedicalkey.com]
  • One month later after his discharge, he complained of mild cough and dyspnea again. Follow-up chest CT revealed more extended crazy paving appearance on both lungs with recurred pleural effusion.[lungkorea.org]
  • […] liver Pathophysiology / etiology Progressive disease with recurrent effusions and respiratory failure secondary to infections and chylous accumulation Congenital, possibly from abnormal lymphatic development Clinical features Generally presents with dyspnea[pathologyoutlines.com]
Hemoptysis
  • ., ribs, vertebrae, humerus, femur), liver, and skin Presentation Dyspnea Cough Hemoptysis Chest pain Milky sputum Pleural effusions Mediastinal enlargement Laboratory Tests Pulmonary functional tests demonstrate Mixed pattern obstructive/restrictive[basicmedicalkey.com]
  • […] respiratory failure secondary to infections and chylous accumulation Congenital, possibly from abnormal lymphatic development Clinical features Generally presents with dyspnea, wheezing, cough and may be confused with asthma Occasionally with chyloptysis or hemoptysis[pathologyoutlines.com]
  • This 50-year-old nonsmoking Hispanic woman presented with a 20-year history of cough, hemoptysis, chyloptysis, and pleuritic chest pain.[experts.umich.edu]
  • In Kaposiform lymphangiomatosis, lymphatic spindle cells invade bronchoalveolar bundles and pulmonary septae, resulting in hemoptysis and hemothorax; thrombocytopenia and anemia may be associated with a positive disseminated intravascular coagulation[thoracic.org]
  • Pleural effusions are often chylous, the same with our case, and patients may have associated asthma syndrome, chyloptysis, hemoptysis or chylopericardium.[atm.amegroups.com]
Exertional Dyspnea
  • Despite the poor prognosis of the disease, 5 years after the treatment the patient has not presented new episodes of pleuropericardial effusions, nor has he required hospitalization for other symptoms, currently presenting only exertional dyspnea.[archbronconeumol.org]
  • Clinical presentation Pulmonary arterial hypertension manifests the following symptoms and signs: Symptoms Progressive onset of exertional dyspnea (60%) 14 Chest pain or discomfort (17%) Palpitations (5%) Dizziness and light-headedness.[mdedge.com]
Chest Pain
  • ., ribs, vertebrae, humerus, femur), liver, and skin Presentation Dyspnea Cough Hemoptysis Chest pain Milky sputum Pleural effusions Mediastinal enlargement Laboratory Tests Pulmonary functional tests demonstrate Mixed pattern obstructive/restrictive[basicmedicalkey.com]
  • Case presentation The patient was a 28-year-old female who had complained of chest pain and polypnea for one year.[atm.amegroups.com]
  • The complications of Diffuse Pulmonary Lymphangiomatosis may include the following: In general, lymphangiomatosis can affect any part of the body Severe breathing difficulties leading to respiratory failure resulting in death Severe chest pain Frequent[dovemed.com]
  • This 50-year-old nonsmoking Hispanic woman presented with a 20-year history of cough, hemoptysis, chyloptysis, and pleuritic chest pain.[experts.umich.edu]
  • Symptoms include chest pain and pressure, coughing up blood and shortness of breath. Talk with your doctor to find the best treatment if you or your child has been diagnosed with pulmonary lymphangiomatosis.[diseaseinfosearch.org]
Facial Edema
  • edema(1/18,5.56%) and bone or vertebral lesions(3/18,16.67%).Conclusion MSCT direct lymphangiography can clearly show the lymphatic abnormal return and the dilatation and proliferation of lymphatic channels by abnormal distribution of contrast agent,[en.cnki.com.cn]

Workup

  • […] histological features of lung specimens from nine patients in whom no extrathoracic lesions were identified, which they termed "diffuse pulmonary lymphangiomatosis" (DPL). [4] Recognition of the disease requires a high index of suspicion and an extensive workup[en.wikipedia.org]
  • In this series, four patients with pericardial effusions were at first thought to have nonneoplastic disease, the workups of whom included two negative bloody pericardial fluid cytology exams, and two patients were initially treated with prednisone for[nature.com]
Pulmonary Infiltrate
  • The CT scans were analyzed for type of pulmonary infiltrative process and its distribution, presence or absence of pleural fluid or thickening, and presence or absence of thoracic lymphadenopathy.[ncbi.nlm.nih.gov]
  • Página 54 - Ward HN: Pulmonary infiltrates associated with leukoagglutinin transfusion reactions. Ann Intern Med 73:689-694. 1970. 234. Thompson JSA.[books.google.es]
Kerley B Lines
  • Key Facts Rare non-langerhans cell histiocytosis Pleural and perirenal soft tissue thickening Diffuse septal thickening Imaging Findings Chest radiograph Cardiomegaly, Kerley B lines, blunting costophrenic angles (mimic CHF) Sclerotic bone lesions CT[chestx-ray.com]
Ground Glass Appearance
  • glass appearance and interlobular septal thickening on follow-up chest CT.[lungkorea.org]
Human Herpesvirus 8
  • Kaposi’s sarcoma is a cancer caused by infection with human herpesvirus 8 (HHV8), also resulted in patches of abnormal tissue to grow under the skin, in the lining of the mouth, nose, and throat or in other organs.[atm.amegroups.com]
Pleural Effusion
  • Nineteen patients had interlobular septal thickening, 18 had diffuse ground-glass opacities, 22 had pleural effusion, 14 had extrapleural soft-tissue thickening, 20 had pericardial effusion and thickened pericardium, 10 had multiple lymphadenopathy in[ncbi.nlm.nih.gov]
  • Seven of the eight had bilateral pleural effusions or smooth thickening of the pleura (or both), and two had regions of calcified pleural thickening.[ncbi.nlm.nih.gov]
  • effusion and a pericardial effusion.[unboundmedicine.com]
  • Definition / general Diffuse proliferation of abnormal, complex lymphatic channels (lymphangiomas) that may involve lung or other organs ( Lymphat Res Biol 2011;9:191 ) Pulmonary involvement often accompanied by chylous pleural effusions May be progressive[pathologyoutlines.com]
  • CT chest Described features include 4 increased interlobular septal thickening peribronchovascular thickening patchy ground glass opacities pleural thickening pleural effusion mediastinal soft tissue infiltration lymphangioleiomyomatosis Promoted articles[radiopaedia.org]
Pleural Adhesions
  • Lungs were congested, edematous, of increased consistence, with bilateral pleural adhesions, pleural fibrinous reaction at the base of the lungs ( Figures 3C and 3D ) and a relevant bilateral pleural effusion.[shortnessofbreath.it]

Treatment

  • The present article discusses clinical, radiographic and pathological features, and treatment options for DPL.[ncbi.nlm.nih.gov]
  • Related Articles High hopes, realistic expectations drive bevacizumab treatment.[connection.ebscohost.com]
  • It may be erroneously diagnosed as asthma responding to bronchodilator treatment, which was the reason for the late diagnosis in our case, previously diagnosed with COPD with bronchodilator treatment.[archbronconeumol.org]
  • Although both effusions initially recurred and required subsequent drainage and treatment by colchicine, evolution at 19 months of diagnosis is favorable.[unboundmedicine.com]
  • Oral propranolol, which had been proposed as an alternative treatment for lymphatic malformation, was attempted to administrate at 0.5 mg/kg/day initially, then it was increased to 1.5 mg/kg/day. 5 months after treatment with propranolol, the patient[lungkorea.org]

Prognosis

  • This case illustrates the fact that the prognosis of diffuse pulmonary lymphangiomatosis in adults may be characterized by a less aggressive evolution and by a more favorable prognosis than that observed in children.[unboundmedicine.com]
  • Although it is a benign disease of unknown etiology and pathogeny, it presents a high rate of relapse and a poor prognosis.[archbronconeumol.org]
  • The prognosis for the patients with this disease limited to the thorax is guarded and progressive although some patients have realtively indolent course.[lymphsystemdisorders.blogspot.com]
  • Milky sputum Pleural effusions Mediastinal enlargement Laboratory Tests Pulmonary functional tests demonstrate Mixed pattern obstructive/restrictive Restrictive pattern only Natural History Progressive condition leading to end-stage respiratory failure Prognosis[basicmedicalkey.com]

Etiology

  • ETIOLOGY/PATHOGENESIS Developmental Anomaly Congenital disease due to abnormal lymphatic system development CLINICAL ISSUES Epidemiology Age Mean at presentation: 8.5 years May manifest at birth and rarely in adults Gender No predilection Site Localized[basicmedicalkey.com]
  • […] if proliferation is focal Epidemiology Rare condition, typically presents in children and young adults No sex predilection Sites Localized to lung/chest or can also involve multiple organs, including bone, spleen, soft tissue, liver Pathophysiology / etiology[pathologyoutlines.com]
  • Although it is a benign disease of unknown etiology and pathogeny, it presents a high rate of relapse and a poor prognosis.[archbronconeumol.org]
  • Pulmonary lymphangioleiomyomatosis (LAM) is a benign, uncommon disorder of unknown etiology affecting women of child-bearing age ( 7 ).[atm.amegroups.com]

Epidemiology

  • ETIOLOGY/PATHOGENESIS Developmental Anomaly Congenital disease due to abnormal lymphatic system development CLINICAL ISSUES Epidemiology Age Mean at presentation: 8.5 years May manifest at birth and rarely in adults Gender No predilection Site Localized[basicmedicalkey.com]
  • […] lymphangiomas) that may involve lung or other organs ( Lymphat Res Biol 2011;9:191 ) Pulmonary involvement often accompanied by chylous pleural effusions May be progressive and fatal from respiratory failure Terminology Lymphangioma if proliferation is focal Epidemiology[pathologyoutlines.com]
  • Epidemiology Lymphangiomatosis may present at any age but is predominantly a condition of children and young adults, with no gender predilection 8. Clinical features Patients present with "asthma", dyspnea or haemoptysis 8 ,12.[e-immunohistochemistry.info]
  • Epidemiology [ edit ] Lymphangiomatosis can occur at any age, but the incidence is highest in children and teenagers.[en.wikipedia.org]
  • Schneble L, Murer L, Carraro A, Pape L, Kranz B, Oh J, Zirngibl M, Dello Strologo L, Buscher A, Weber LT, Awan A, Pohl M, Bald M, Printza N, Rusai K, Peruzzi L, Topaloglu R, Fichtner A, Krupka K, Koster L, Bruckner T, Schnitzler P, Hirsch HH, Tonshoff B: Epidemiology[forschdb.verwaltung.uni-freiburg.de]
Sex distribution
Age distribution

Pathophysiology

  • Terminology Lymphangioma if proliferation is focal Epidemiology Rare condition, typically presents in children and young adults No sex predilection Sites Localized to lung/chest or can also involve multiple organs, including bone, spleen, soft tissue, liver Pathophysiology[pathologyoutlines.com]
  • […] microcystic) 21 Evaluation CT MRI Lymphangiograms Pulmonary Function Tests 22 Treatment Treat the anomaly: medicine, intervention, surgery Pulmonary medications Respiratory therapy Physical therapy Lung transplant 23 Questions to be answered What is the pathophysiology[slideplayer.com]
  • Pathogenesis and pathophysiology Pulmonary hypertension can be caused by narrowing of the precapillary vessels (arteries and arterioles), loss of vascular surface area, or passive back pressure from the postcapillary vessels [see Table 2].[what-when-how.com]

Prevention

  • Lotery, Andrew; MacEwen, Carrie // BMJ: British Medical Journal;11/22/2014, Vol. 349 Issue 7894, pg6887 The authors stress the need for the British government to act to remove the hurdles from preventing the use of bevacizumab for the treatment of age[connection.ebscohost.com]
  • In the case of recurring chylothorax, drainage and pleurodesis are indicated, with the option of thoracic duct ligation 3 to prevent complications such as malnutrition, pulmonary fibrosis and deteriorated respiratory function.[archbronconeumol.org]
  • This procedure thereby obliterates the space between the pleura and prevents the reaccumulation of fluid.[lymphedemapeople.com]
  • There are currently no standardized or optimized methods of casualty management; current treatment strategy consists of symptomatic management and is designed to relieve symptoms, prevent infections, and promote healing.[science.gov]

Ask Question

5000 Characters left Format the text using: # Heading, **bold**, _italic_. HTML code is not allowed.
By publishing this question you agree to the TOS and Privacy policy.
• Use a precise title for your question.
• Ask a specific question and provide age, sex, symptoms, type and duration of treatment.
• Respect your own and other people's privacy, never post full names or contact information.
• Inappropriate questions will be deleted.
• In urgent cases contact a physician, visit a hospital or call an emergency service!