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]

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]

The present article discusses clinical, radiographic and pathological features, and treatment options for DPL. [mayoclinic.pure.elsevier.com]

In this paper, We would like to present an unusual case of Diffuse pulmonary lymphangiomatosis involving a 28-year-old young female, who presented with progressive chest pain and polypnea of one year duration, and CT showed much left hydrothorax. [atm.amegroups.com]

• Recurrent Pleural Effusion

  […] and aspiration with pleurodesis for patients with recurrent pleural effusion. [adc.bmj.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]

  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]

  Recurrent pleural effusions may require pleural fluid drainage or pleurodesis. [ijri.org]

  Other surgical procedures have included parietal pleurectomy, pleurodesis and ligation of the thoracic duct to reduce recurrent pleural effusion [ 71 – 74 ]. [err.ersjournals.com]

• Pleural Effusion

  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]

  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]

  effusion and a pericardial effusion. [unboundmedicine.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 References Promoted [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]

  Age Mean at presentation: 8.5 years May manifest at birth and rarely in adults Gender No predilection Site Localized to lung or chest Widespread distribution involving spleen, bones (e.g., ribs, vertebrae, humerus, femur), liver, and skin Presentation Dyspnea [basicmedicalkey.com]

  They present with chylous effusion and dyspnea. A restrictive pattern is noted in pulmonary function tests. [ispub.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]

• Hemoptysis

  This 50-year-old nonsmoking Hispanic woman presented with a 20-year history of cough, hemoptysis, chyloptysis, and pleuritic chest pain. [experts.umich.edu]

  […] presentation: 8.5 years May manifest at birth and rarely in adults Gender No predilection Site Localized to lung or chest Widespread distribution involving spleen, bones (e.g., ribs, vertebrae, humerus, femur), liver, and skin Presentation Dyspnea Cough Hemoptysis [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]

  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]

  Tazelaar Mayo Clinic Arizona Click on each slide thumbnail image for an enlarged view History A 42 year old man presented with hemoptysis. [uscapknowledgehub.org]

• Respiratory Insufficiency

  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]

  DPL, especially in paediatric populations, can often have a progressive evolution to respiratory insufficiency and death [ 15 ]. [err.ersjournals.com]

  Justin Besancon L, Pequignot H, Galey JJ, Renault P, Even P: Acquired diffuse pulmonary lymphangiectasis with respiratory insufficiency and chylothorax. Sem Hop 1963;39:1179-1190. Pachter MR, Lattes R: Mesenchymal tumors of the mediastinum. III. [karger.com]

• Pulmonary Disorder

  Diffuse pulmonary lymphangiomatosis is a rare pulmonary disorder affecting the lymphatic channels from the mediastinum to the pleura. The disease usually occurs in children and young adults and frequently ends with death due to progressive course. [ncbi.nlm.nih.gov]

  Diffuse pulmonary lymphangiomatosis is a rare pulmonary disorder affecting the lymphatic channels from the mediastinum to the pleura. [ispub.com]

• Chest Pain

  This 50-year-old nonsmoking Hispanic woman presented with a 20-year history of cough, hemoptysis, chyloptysis, and pleuritic chest pain. [experts.umich.edu]

  Case presentation The patient was a 28-year-old female who had complained of chest pain and polypnea for one year. [atm.amegroups.com]

  Widespread distribution involving spleen, bones (e.g., ribs, vertebrae, humerus, femur), liver, and skin Presentation Dyspnea Cough Hemoptysis Chest pain Milky sputum Pleural effusions Mediastinal enlargement Laboratory Tests Pulmonary functional tests [basicmedicalkey.com]

  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]

  He tolerated treatment well and showed an excellent symptomatic response to therapy with a significant reduction in his dyspneic episodes, chest and body pain. [ijamhrjournal.org]

• Osteoporosis

  Diagnosis Serum alkaline phosphatase may be elevated in GSD, but serum testing is not helpful in making the diagnosis, which is suspected by x-ray, CT or MRI demonstrating bone findings that initially appear similar to osteoporosis. [thoracic.org]

• Low Back Pain

  back pain (Diagn Interv Radiol 2007; 13: 144-148) CT measurement of trunk muscle areas in patients with chronic low back pain (Diagn Interv Radiol ; : 144-148) Head and Neck Imaging - Original Article Incidental findings in the maxillofacial region detected [dirjournal.org]

• Hematuria

  Abdominal involvement manifests as pain, distension, vomiting, diarrhea, or hematuria if kidneys are affected. Bone affection presents as limb swelling, bone pain, pathological fracture, and paraplegia on spinal involvement. [ijamhrjournal.org]

[…] 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]

Further workup for PHACE may be needed on the basis of central nervous system (CNS) findings. [northshore.org]

• 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]

  Sometimes massive pleural effusion and progressive pulmonary infiltration cause respiratory failure [ 15 ]. [err.ersjournals.com]

• 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]

  His chest radiograph at presentation showed mediastinal widening, bilateral pleural effusions, and Kerley B lines. [ijri.org]

  Clin Radiol. 2002; 57:8–17. 4.14 Septal (Kerley B) lines Pulmonary lobules are the smallest lung units bounded by interlobular septa. Each lobule is supplied by a lobular airway and arterial branch. [clinicalgate.com]

• X-Ray Abnormal

  […] of tissue well before the abnormalities become detectable on the chest x-ray or CT scan. [anyflip.com]

• Decreased Lung Compliance

  Decreased lung compliance increases the work of breathing and energy expenditure. Energy supplementation should be undertaken with consideration to the added difficulty in handling high carbohydrate loads with chronic lung disease. [emedicine.medscape.com]

• Pleural Effusion

  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]

  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]

  effusion and a pericardial effusion. [unboundmedicine.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 References Promoted [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]

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]

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]

Although both effusions initially recurred and required subsequent drainage and treatment by colchicine, evolution at 19 months of diagnosis is favorable. [unboundmedicine.com]

The present article discusses clinical, radiographic and pathological features, and treatment options for DPL. [mayoclinic.pure.elsevier.com]

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]

Non-neoplastic diseases are presented according to epidemiology, genetics, clinical manifestations, radiographic findings, pathology, cytology, laboratory findings, pathogenesis, differential diagnosis, prognosis and natural history. [books.google.com]

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]

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 the etiology of most cases of DAD cannot be determined histologically, the cause of the damage can usually be obtained from... [brainscape.com]

Imagining studies showed interstitial lung disease of unknown etiology. The lung tissue biopsy confirmed that the diagnosis was diffuse pulmonary lymphangiomatosis. [ispub.com]

Sarcoidosis Sarcoidosis is a systemic noncaseating granulomatous disease of unknown etiology that affects the mediastinum, lungs, and other organs to varying degrees. [radiologykey.com]

On this page: Article: Epidemiology Radiographic features See also References There is no recognized gender predilection. It typically manifests in children and young adults. [radiopaedia.org]

Non-neoplastic diseases are presented according to epidemiology, genetics, clinical manifestations, radiographic findings, pathology, cytology, laboratory findings, pathogenesis, differential diagnosis, prognosis and natural history. [books.google.com]

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]

This thoroughly reworked edition includes new classification schemes and the latest understanding of the pathophysiology and molecular aspects of a wide range of diseases. [books.google.com]

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]

Large-vessel in situ thromboses Pulmonary emboli result in two main pathophysiologic consequences Occlusion of the large pulmonary arteries is almost always caused by... [brainscape.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]

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]

Early diagnosis of diffuse pulmonary lymphangiomatosis is difficult to achieve, but it allows the application of therapies that prevent disease progression, reducing morbidity and mortality. [pubmed.ncbi.nlm.nih.gov]

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]

Radiation therapy to the brain may also help treat or prevent metastasis. [anyflip.com]