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Pulmonary Histiocytosis X

Pulmonary Eosinophilic Granuloma


Presentation

  • S100 protein was present in HX cells in 22 of the PHX cases; HLA-DR antigen was present in HX cells from 16 cases.[ncbi.nlm.nih.gov]
Anemia
  • […] can be treated with chemotherapy Quick Facts Letterer-Siwe Disease 10% of histiocytosis X Acute disseminated, fulminant form Age at onset Several weeks after birth to 2 years Pathology May be confused with leukemia Symptoms Hemorrhage, purpura Severe anemia[learningradiology.com]
  • Key terms Anemia — Abnormally low level of red blood cells in the blood. Biopsy — Surgical removal of tissue for examination.[medical-dictionary.thefreedictionary.com]
  • Anemia and destructive bony lesions are seen. Letterer-Siwe disease is rapidly fatal if untreated. With intensive chemotherapy, 5-year survival is about 50%.[histopathology-india.net]
  • Other complications may include: Pituitary gland problems Growth problems (including delayed growth) Anemia Lung failure Key points Langerhans cell histiocytosis is a rare condition.[stanfordchildrens.org]
  • 4201 4250 番を表示 (全 8148 件) 英文 和文 略語 high resolution computed tomography 高感度コンピューター断層撮影/高感度コンピューター断層撮影法 HRCT high risk infant ハイリスク児 high risk pregnancy ハイリスク妊娠 high-phosphatidylcholine hemolytic anemia 高ホスファチジルコリン溶血性貧血 higher brain function 高次脳機能 highly[jpeds.or.jp]
Falling
  • A severe obstructive defect was manifested by severely reduced rates of flow, a fall in the forced vital capacity from 2.6 L to 1.4 L, and a total lung capacity greater than 100 percent of the predicted normal value on three occasions.[ncbi.nlm.nih.gov]
Cough
  • It primarily affects young smokers and usually presents with symptoms of cough, dyspnea, and chest pain.[ncbi.nlm.nih.gov]
  • The patient had also dyspnea and non productive cough. The patient was heavy smoker for 6 years. General examination revealed that the patient had tachypnea but he was hemodynamically stable.[platform.almanhal.com]
  • Clinical Information: This 42 year old Caucasian male had a 2-year history of non-productive cough, dyspnea on exertion, and fatigue. He had four episodes of fever in the preceding 18 months. He had a 40 pack-year history of smoking.[ucdenver.edu]
  • Present to medical attention Incidental findings on CXR Spontaneous pneumothorax Respiratory or constitutional symptoms: Nonproductive cough (56 to 70 %) Dyspnea (40 to 87 %) Pleuritic chest pain (10 to 21 %) Fatigue (30 %) Weight loss (20 to 30 %) Fever[slideshare.net]
Dyspnea
  • It primarily affects young smokers and usually presents with symptoms of cough, dyspnea, and chest pain.[ncbi.nlm.nih.gov]
  • Dyspnea score Limitation of activity related to dyspnea was evaluated using the modified Medical Research Council (mMRC) scale. Chest CT scans Chest CT scans performed up to six months after CPET were analysed.[journals.plos.org]
  • The patient had also dyspnea and non productive cough. The patient was heavy smoker for 6 years. General examination revealed that the patient had tachypnea but he was hemodynamically stable.[platform.almanhal.com]
  • Clinical Information: This 42 year old Caucasian male had a 2-year history of non-productive cough, dyspnea on exertion, and fatigue. He had four episodes of fever in the preceding 18 months. He had a 40 pack-year history of smoking.[ucdenver.edu]
  • Present to medical attention Incidental findings on CXR Spontaneous pneumothorax Respiratory or constitutional symptoms: Nonproductive cough (56 to 70 %) Dyspnea (40 to 87 %) Pleuritic chest pain (10 to 21 %) Fatigue (30 %) Weight loss (20 to 30 %) Fever[slideshare.net]
Pulmonary Disorder
  • We therefore examined the frequency of CD-1-positive cells in a pool of patients with different pulmonary disorders, according to their smoking habits and diagnoses.[ncbi.nlm.nih.gov]
  • Eosinophilia in Pulmonary Disorders. Immunol Allergy Clin North Am . 2015 Aug. 35 (3):477-92. [Medline] .[emedicine.medscape.com]
  • Discussion PLCH is a smoking-related nodular-cystic pulmonary disorder, which occurs in young-medium-aged adults (20-40 years of age) with an equal gender distribution [ 2, 3 ].[scitechnol.com]
Exertional Dyspnea
  • A young male who currently smoked complained of a cough and exertional dyspnea, common clinical features of PLCH; however, the radiological findings did not support a diagnosis of PLCH.[kjim.org]
  • dyspnea typical ( Eur J Intern Med 2015;26:351 ) Rarely can present with hemoptysis Recurrent spontaneous pneumothorax in 15 - 25% of cases Pulmonary hypertension and vasculopathy frequent in advanced cases Diabetes insipidus in 15% of patients from[pathologyoutlines.com]
  • In half the cases, pulmonary involvement is revealed by non specific respiratory signs in smokers, dominated by non-productive cough, sometimes associated with exertional dyspnea.[pubs.sciepub.com]
Rales
  • A physical examination indicated that moist rales in bilateral lungs could be detected in 1 patient, and no complaint of hemoptysis or fever and no acropachia were found in all patients (Table 1).[journals.lww.com]
Heart Disease
  • Our report describes the occurrence of S. equinus endocarditis in a patient who had no history of pre-existing heart disease, but who showed evidence of a late-stage pulmonary histiocytosis X.[ncbi.nlm.nih.gov]
  • Hypertension due to Lung Disease and/or Hypoxia, Pulmonary Arterial Hypertension Associated with Congenital Heart Disease, World Health Organization Group 5 Pulmonary Hypertension, and more![books.google.de]
Left Ventricular Dysfunction
  • It is of note that 6 of the 8 subjects with a lower than 70% predicted oxygen pulse had performed echocardiography showing no sign of pulmonary hypertension (pulmonary artery systolic pressure lower than 40 mmHg) or left ventricular dysfunction (data[journals.plos.org]
Bone Pain
  • Symptoms are nonspecific h/o recurrent pneumothorax, diabetes insipidus, or bone pain can be helpful Current or past smoking history 11.[slideshare.net]
  • Adults may experience: • Bone pain; • Cough and shortness of breath; • Fever; • Frequent urination; • Weight loss; and • Rash. ICD-9: 202.5, 277.89 PROGRESSION LCH is relatively more common in children than in adults.[secure.ssa.gov]
  • Well-localised bone pain is a common presenting symptom, with pain during both activity and rest. Lesions are lytic. Bone pain usually correlates with a radiologically evident lesion but lesions can be painless.[patient.info]
  • Symptoms in adults may include: Bone pain Chest pain Cough Fever General discomfort, uneasiness, or ill feeling Increased amount of urine Rash Shortness of breath Thirst and increased drinking of fluids Weight loss There are no specific blood tests for[medlineplus.gov]
  • Bone pain due to bone cysts (18%), rash (13%), and polyuria due to central diabetes insipidus (5%) are the most common manifestations of extrapulmonary involvement and occur in up to 15% of patients, rarely being the presenting symptoms of PLCH.[msdmanuals.com]

Workup

  • He was advised to stop smoking and put on inhalers by his GP who then referred this patient to one of the respiratory physician for further workup and management. Figure 1: Diffuse reticular and cystic changes typically sparing costophrenic angles.[chronic-obstructive-pulmonary-disease.imedpub.com]
Reticulonodular Pattern
  • Diagnosis was suspected when a reticulonodular pattern was detected by conventional X-ray or by computerized tomography of the lungs. Laboratory tests were altered nonspecifically, and lung function was impaired.[ncbi.nlm.nih.gov]
  • Imaging: His CXR shows a bilateral, predominantly mid- and upper-zone reticulonodular pattern with some small cystic areas in the upper lobes. There is mild bilateral hilar adenopathy.[ucdenver.edu]
  • Plain radiograph The earliest change is a diffuse bilateral symmetrical reticulonodular pattern with a predilection for the mid and upper zones. The ill-defined nodules range from 1-10 mm in size.[radiopaedia.org]
Pulmonary Infiltrate
  • Página 54 - Ward HN: Pulmonary infiltrates associated with leukoagglutinin transfusion reactions. Ann Intern Med 73:689-694. 1970. 234. Thompson JSA.[books.google.es]
Hypocapnia
  • Increased ventilation was not associated with hypocapnia and thus was appropriate to metabolic requests.[journals.plos.org]
Staphylococcus Aureus
  • Abstract The broncho-alveolar lavage from an 18-year-old girl with proven histiocytosis X of the lung has been cocultivated with Staphylococcus aureus.[ncbi.nlm.nih.gov]
Decreased Vital Capacity
  • Pulmonary function tests (PFT) show various abnormalities including reduction of the diffusing capacity of the lung for carbon monoxide (DL CO ), decreased vital capacity (VC), increased residual volume (RV) and airway obstruction [ 5 ].[journals.plos.org]

Treatment

  • Macrophage colony stimulating factor, c-fms and c-sis gene expression were reduced almost to normal after treatment.[ncbi.nlm.nih.gov]
  • One of the 7 patients declined all treatment, and another 2 patients later moved to another hospital to continue treatment.[journals.lww.com]

Prognosis

  • The three evolutionary patterns of improvement, stabilisation, and worsening are analysed with respect to the initial radiological features; one which carries a good prognosis is sparing of both costophrenic angles.[ncbi.nlm.nih.gov]
  • Langerhans cells Smoking-related lung disease May present with pneumothorax (17%) or develop recurrent pneumothoraces Upper and mid lung reticulonodular opacities, sparing costophrenic angles HRCT: irregular small nodules and bizarre shaped cysts Variable prognosis[chestx-ray.com]
  • […] as eosinophilic granuloma( tosis ) Proliferative disorder of the Langerhans cells Normally found in the skin (and a few other organs) and serve as antigen-presenting cells Rare diseases, affecting neonates up to adults 2:1 male to female predominance Prognosis[learningradiology.com]
  • Cystic bone lesions (4 to 20 %) localized pain or a pathologic bone fracture Diabetes insipidus (hypothalamic involvement) 15 % Worse prognosis. 10.[slideshare.net]
  • Neonates with LCH limited to the skin and mucous membranes have a good prognosis [ 15 ] .[patient.info]

Etiology

  • Discussion: The combination of nodules and multiple, irregular-shaped, thin-walled cysts is highly suggestive of Pulmonary Histiocytosis X(also known as Pulmonary Eosinophilic Granulmona.This condition of unknown etiology is uncommon, occurring predominantly[ucdenver.edu]
  • Pulmonary Langerhans Cell Histiocytosis (PLCH) Eosinophilic Granuloma of the Lung Pulmonary Langerhans Cell Granulomatosis Pulmonary Histiocytosis X Uncommon ILD Young adults Caucasians Equal gender distribution Smoke is an etiologic factor The duration[slideshare.net]
  • Signs and symptoms, pathology, pathogenesis, etiology, course and prognosis, and treatment are discussed. Evidence is preponderantly in favor of the etiology in all cases being nematode larvae invasions of the liver and other viscera.[pediatrics.aappublications.org]
  • The etiology of PLCH is unknown, but the disease occurs almost exclusively in whites 20 to 40 yr of age who smoke. Men and women are affected equally.[msdmanuals.com]
  • Chronic Eosinophilic Leukemia (CEL) Clinical signs fever fatigue cough angioedema myalgia pruritus, diarrhoea sometimes neurological, pulmonar an rheumatic symptoms Etiology The BCR-ABL1 gene or the PDGFRA, PDGFRB rebuild is absent.[atlases.muni.cz]

Epidemiology

  • OBJECTIVE: To define the clinico-epidemiological features of pulmonary histiocytosis X in Japan. METHODS: A nationwide survey was carried out in 1997 using two questionnaires.[ncbi.nlm.nih.gov]
  • 抄録 Objective To define the clinico-epidemiological features of pulmonary histiocytosis X in Japan. Methods A nationwide survey was carried out in 1997 using two questionnaires.[jstage.jst.go.jp]
  • Articles include: Epidemiology of Pulmonary Arterial Hypertension, Pathology of Pulmonary Hypertension, Genetics of Pulmonary Arterial Hypertension, Diagnosis of Pulmonary Arterial Hypertension, Pulmonary Hypertension Owing to Left Heart Disease, Pulmonary[books.google.de]
  • Epidemiology Frequency United States Pulmonary Langerhans cell histiocytosis X (PLCH) is a rare disorder and the true prevalence is unknown.[emedicine.medscape.com]
  • […] chronic progressive variant with onset prior to five years of age classic triad i ncludes skull lesions diabetes insipidus exophthalmus Eosinophilic granuloma extraskeletal generally limited to lung best prognosis and sometimes regresses spontaneously Epidemiology[medbullets.com]
Sex distribution
Age distribution

Pathophysiology

  • The pathophysiology of PH in pulmonary LCH is likely to be multifactorial, with contributions from chronic hypoxaemia, abnormal pulmonary mechanics and vascular remodelling.[erj.ersjournals.com]
  • Pathophysiology may involve recruitment and proliferation of Langerhans cells in response to cytokines and growth factors secreted by alveolar macrophages in response to cigarette smoke.[msdmanuals.com]
  • The pathophysiologic mechanisms that limit exercise capacity have been poorly investigated and consequently, the effect of symptomatic treatments is unknown.[journals.plos.org]
  • - 50 years More common in Caucasian than African Americans or Asians Sites 50% of cases only involve lung 20% of those with multicentric disease (i.e. bone, skin, lymph nodes, spleen, CNS, pituitary, rarely thyroid and thymus) have lung involvement Pathophysiology[pathologyoutlines.com]
  • Although nearly a century has passed since histiocytic disorders were recognized, their pathophysiology has started to be elucidated with the application of molecular analyses.[disabled-world.com]

Prevention

  • Pulmonary Langerhans cell histiocytosis X is a rare disease and may present by spontaneous pneumothorax, so, high index of suspicion is required in heavy smoker young males to diagnose the condition and to perform pleurodesis preventing recurrence of[platform.almanhal.com]
  • , and Complications Prognosis depends on specific type and severity of disease 50% of pulmonary histiocytosis cases are fatal young children, especially infants, usually die of disease Prevention there are no known preventive measures for childhood disease[medbullets.com]
  • Can Langerhans cell histiocytosis be prevented? Because the cause of Langerhans cell histiocytosis is unknown, there is no known way to prevent the condition. Having a family history of thyroid disease may increase the risk.[stanfordchildrens.org]
  • Prevention Because the cause of Langerhans' cell histiocytosis is not known, there is no way to prevent it.[drugs.com]
  • Investigations for pulmonary hypertension should be done early to prevent unnecessary delay in management.[chronic-obstructive-pulmonary-disease.imedpub.com]

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