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]

• Pathologist

  Samples are collected for further examination by a pathologist Tissue biopsy from the affected lung: A biopsy of the tumor is performed and sent to a laboratory for a pathological examination. A pathologist examines the biopsy under a microscope. [dovemed.com]

  Up-to-date and richly detailed, Essentials of Anatomic Pathology, Third Edition offers both the pathologist-in-training and the practicing pathologist a concise summary of all the critical information needed to recognize, understand and interpret anatomic [books.google.com]

  In addition, pediatric pulmonologists are often very reluctant to perform lung biopsies due to a lack of a specialized pathologist. [paperity.org]

  In addition, consider consultation with the following specialists: Infectious disease specialist Immunologist Rheumatologist Transplantation specialist Pathologist: Consultation with a pathologist is recommended before tissue is obtained to ensure that [emedicine.medscape.com]

• Lymphedema

  […] syndrome – OMIM #267750, Urioste syndrome – OMIM %235255; • X-linked syndromes: Lymphedema hypoparathyroidism – OMIM 247410, Mandibulofacial dysostosis, lymphedema syndrome [ 41 ]. [ojrd.biomedcentral.com]

  […] retroperitoneal and splenic cystic lymphangioma(1/18,5.56%),cervical lymphatic vessels dilation(8/18,44.44%),thoracic duct outlet obstruction(8/18,44.44%),multiple small lymphadenopathy in neck(10/18,62.50%) and axilla(16/18,88.89%),lower extremity lymphedema [en.cnki.com.cn]

  Lymphedema: results from either dysplastic or obstructed collecting lymphatic vessels or non-functional peripheral lymphatic vessels. 14 Common Malformations Usually localized Can cause issues depending on where the lesions are located Can be macro or [slideplayer.com]

  Chylous pleural effusion associated with primary lymphedema and lymphangioma-like malformations. Chest. 1993; 103 :1916–1918. 21. Calabrese PR, Frank H, Taubin HL. [shortnessofbreath.it]

• 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]

• Malnutrition

  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]

• 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]

• 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]

• Exertional 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]

  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]

• Abdominal Pain

  When they do occur, symptoms include abdominal pain and/or distension; nausea, vomiting, diarrhea; decreased appetite and malnourishment. [en.wikipedia.org]

  Signs and Symptoms: Lungs – may cause chronic cough, wheezing, shortness of breath, collection of fluid (pleural effusions) around the lungs Heart – rapid heartbeat, chest pain Stomach, intestines – abdominal pain, nausea, vomiting, diarrhea Kidneys – [lymphedemapeople.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]

• Skin Lesion

  Diagnostic Criteria for Tuberous Sclerosis (2012) Major Criteria Minor Criteria Cortical tubers Non-renal hamartoma Subependymal nodules Confetti-like skin lesions Hypomelanocytic macule Multiple renal cysts (at least 3 or more and larger than 5 mm) Shagreen [medical-dictionary.thefreedictionary.com]

  Breast With an Apparent Etiology of Underlying Calcified Thrombosed Artery With Adjacent Fat Necrosis. ( 27097335 ) Strausburg M.B....Warren S. 2016 33 Successful treatment of bacillary angiomatosis with oral doxycycline in an HIV-infected child with skin [malacards.org]

  Late skin complaints were observed in nearly all survivors who had early skin lesions (131 out of 137; 95.62%). Seven out of 12 patients (58.33%) who did not have early skin lesions ultimately developed late skin complications. [science.gov]

  Airway infantile hemangioma can occur without skin lesions. It is important for an otolaryngologist to proactively assess lesions in this distribution before signs of stridor occur. [northshore.org]

• Fracture

  Rib or thoracic vertebral bone involvement may present as chronic pain or acute pain in cases of pathological fracture. Bone involvement in the young may result in shortening or deformity of the affected bone. [adc.bmj.com]

  Frequently asymptomatic, skeletal lymphangiomatosis may be discovered incidentally or when a pathological fracture occurs. Patients may experience pain of varying severity in areas around the effected bone. [en.wikipedia.org]

  Surgical intervention is necessary for certain pathological fractures and to correct symptomatic deformity. Fractures treated with non-surgical techniques can be expected to heal. [lymphedemapeople.com]

  Complications include fracture, poor healing and bone infection. [thoracic.org]

  Bone affection presents as limb swelling, bone pain, pathological fracture, and paraplegia on spinal involvement. Diagnosing lymphangiomatosis may be challenging due to its rarity, varied clinical, and imaging presentation. [ijamhrjournal.org]

• Aggressive Behavior

  One example of a benign entity that can occasionally exhibit aggressive behavior is lymphangiomatosis, a rare disorder of the lymphatic system characterized by the abnormal proliferation of lymphatic vessels, sometimes referred to as generalized lymphatic [link.springer.com]

[…] 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]