Edit concept Question Editor Create issue ticket

Lymphomatoid Granulomatosis

LYG


Presentation

  • We present a case of lymphomatoid granulomatosis initially presenting with cutaneous lesions, with an accompanying review of the literature.[ncbi.nlm.nih.gov]
Fever
  • We report a 15-year-old boy presented with fever, dry cough and dyspnea from two months ago, after admission patient had nodular lesions on the left leg and hepatosplenomegaly.[ncbi.nlm.nih.gov]
Constitutional Symptom
  • symptoms or multiple organ involvement (2/3 die within a year of pulmonary disease and infection) May progress to EBV diffuse large B cell lymphoma Gross description Lung nodules up to 10 cm with central necrosis and cavitation 15% of patients with skin[pathologyoutlines.com]
  • Patients with WG can have similar cutaneous manifestations, also frequently present with constitutional symptoms, and a constellation of cutaneous, pulmonary, and renal disease.[dermatologyadvisor.com]
  • Constitutional symptoms, including weight loss, fatigue, sweating and chills, are typical ( 6 ).[spandidos-publications.com]
Chills
  • Alpha interferon can have flu-like side effects of headache, fever, chills, and body aches. EPOCH-R drugs can cause gastrointestinal problems, hair loss, and weakness. G-CSF can cause bone pain, body aches, and hair thinning.[clinicaltrials.gov]
  • Constitutional symptoms, including weight loss, fatigue, sweating and chills, are typical ( 6 ).[spandidos-publications.com]
  • He denied any fever, chills, sputum production, orthopnea or paroxysmal nocturnal dyspnea, anorexia, weight loss, recent or past exposure to tuberculosis, sick contacts, pets, recent travel or past exposure to cigarette smoke (active or passive), asbestos[jhoonline.biomedcentral.com]
  • Furthermore, the patient presented with serious systemic involvement characterized by fever, chills and rigors, hypoproteinemia, neutrophilia, and altered liver function findings.[docksci.com]
Axillary Mass
  • We report on a patient of rheumatoid arthritis (RA) who sequentially developed an axillary mass and a fatal interstitial pneumonia during a 2-year course of methotrexate (MTX) therapy.[ncbi.nlm.nih.gov]
  • Hideto Kameda Ayumi Okuyama Jun-ichi Tamaru Shinji Itoyama Atsushi Iizuka Tsutomu Takeuchi Case Report First Online: 03 January 2007 Abstract We report on a patient of rheumatoid arthritis (RA) who sequentially developed an axillary mass and a fatal interstitial[doi.org]
Inguinal Lymphadenopathy
  • CT scan revealed mediastinal (complex confluent subcarinal lymph node mass measuring 5.6 cm), aortopulmonary, paratracheal, right tracheobronchial, axillary, upper abdominal (1.5 cm), external ileac and right inguinal lymphadenopathy (largest 10.7 mm)[doi.org]
Generalized Lymphadenopathy
  • She in addition had generalized lymphadenopathy. Histopathological examination of the cutaneous lesions confirmed LG. Patient died despite therapy with cyclophosphamide and prednisolone.[bioline.org.br]
Cough
  • We report a 15-year-old boy presented with fever, dry cough and dyspnea from two months ago, after admission patient had nodular lesions on the left leg and hepatosplenomegaly.[ncbi.nlm.nih.gov]
  • It most often affects the lungs, causing chest pains, cough, and shortness of breath.[medical-dictionary.thefreedictionary.com]
  • Pulmonary Hypertension (see Pulmonary Hypertension , [[Pulmonary Hypertension]]): due to pulmonary vasculitis Pneumonia-like illness: presents with dyspnea/ cough/ sputum Endobronchial lesion: obstructive symptoms/ hemoptysis (may be massive) Pneumothorax[mdnxs.com]
  • Tony had a cold for 3 weeks, over Christmas, where he was coughing and sneezing, then came the pneumonia, otherwise he'd had no symptoms at all, but the cold and cough might have carried on, which after a couple of months, would have sent him then down[csn.cancer.org]
Dyspnea
  • We report a 15-year-old boy presented with fever, dry cough and dyspnea from two months ago, after admission patient had nodular lesions on the left leg and hepatosplenomegaly.[ncbi.nlm.nih.gov]
  • […] have been reported in some cases (bronchioles lobar bronchi), may produce obstruction CBC: usually normal Dysproteinemia: usually absent Clinical Upper Airway Manifestations Pulmonary Manifestations Epidemiology: usually present Clinical Chest pain Dyspnea[mdnxs.com]
  • He had been examined because of cough, dyspnea, and a skin rash 16 years previously and was subsequently diagnosed with LYG.[jamanetwork.com]
  • We present a 60-year-old woman with worsening fatigue, night sweats, unintentional weight loss, and dyspnea of 2 weeks’ duration.[mdedge.com]
Pleural Effusion
  • As was the experience of others, the laboratory findings in our case were non-specific and although pleural effusion has been known to occur in LYG [12] , commoner causes of pleural effusion in our environment like pneumonia, empyema and tuberculosis[nigeriamedj.com]
  • Massive nodules, pleural effusions, pneumonitis, and cavitary nodules can be seen. Patients with hemoptysis are more likely to have cavitary nodules.[clinicaladvisor.com]
  • Patchy air space disease and pleural effusion have also been reported.[pulmonologyadvisor.com]
  • A small right pleural effusion and little pleural thickening/pleural reaction was reported in dependent parts of the left lower lobe (Figure 1 ). Figure 1 CT scan chest.[doi.org]
Pulmonary Disorder
  • disorders characterized by necrotizing, aseptic granulomas, usually with angiitis.[karger.com]
  • Cutaneous lesions may appear before the pulmonary disorder,[ 10 ] as in our case. Dermatologic manifestations are diverse, but most typically appear as nodules. The clinical pattern of the cutaneous lesions correlates with histological grade.[mjhid.org]
Rales
  • On lung auscultation, breath sounds were diminished over the lower left lung, and there were no crackles or rales on either side. A neurological examination revealed no focal defect.[spandidos-publications.com]
  • General physical examination revealed bilateral crackles and rales on both lung fields. There was no evidence of cardiac murmur, lymphadenopathy, hepatosplenomegaly, or skin lesion.[journals.lww.com]
  • This was slowly tapered, but on August 22, 1995, she suffered an acute decompensation with hypoxemia, tachypnea, poor color, splenomegaly, diffuse rales, and cardiac gallop.[baillement.com]
Right Upper Quadrant Pain
  • A twenty-six year old Caucasian woman with colonic Crohn disease on maintenance azathioprine therapy presented with right upper quadrant pain and fever. Diagnostic imaging revealed extensive liver, pulmonary and cerebral lesions.[ncbi.nlm.nih.gov]
  • Case presentation A 26-year-old Caucasian woman presented with three days of right upper quadrant pain, fever, and malaise.[doi.org]
Abdominal Bloating
  • Repeat MR imaging now demonstrated extensive T2 hyperintense lesions distributed throughout her liver and both kidneys (Figure 1 A).Over the next weeks in hospital, she experienced progressive abdominal bloating, malaise, dyspnea and night sweats without[doi.org]
Abdominal Tenderness
  • Physical examination revealed focal right upper quadrant abdominal tenderness but no palpable masses, organomegaly or signs of ascites.[doi.org]
Gingival Ulceration
  • A 65-year-old Caucasian man with a gingival ulceration underwent a biopsy. The histological pattern was compatible with a grade III lymphomatoid granulomatosis. The staging revealed a nodular lesion in the lower lobe of his right lung.[ncbi.nlm.nih.gov]
Hemianopsia
  • A 65-year-old man complained of visual disturbance and homonymous hemianopsia was designated. CT and MRI revealed an edematous, enhanced irregular and nodular lesion in the right occipital and parietal lobes.[ncbi.nlm.nih.gov]
Unilateral Proptosis
  • A 1-year-old girl with unilateral proptosis was found to have primary orbital lymphomatoid granulomatosis - a condition rarely occurring in children.[ncbi.nlm.nih.gov]
Night Sweats
  • We present a 60-year-old woman with worsening fatigue, night sweats, unintentional weight loss, and dyspnea of 2 weeks' duration.[ncbi.nlm.nih.gov]
  • We present a 60-year-old woman with worsening fatigue, night sweats, unintentional weight loss, and dyspnea of 2 weeks’ duration.[mdedge.com]
  • After clinical improvement, the patient was discharged from hospital with planned repeat MR imaging in one to two months.Six weeks later, she developed progressive abdominal pain, night sweats, and non-positional presyncopal symptoms.[doi.org]
  • sweats, 10% weight loss A absent B present Extranodal sites are also designated M marrow L lung H liver P pleura O bone D skin and subcutaneous tissue Although originally designed for Hodgkin lymphoma, the Ann Arbor System is also used for non-Hodgkin[surgpathcriteria.stanford.edu]
  • What to be alert for in the history Lymphomatoid granulomatosis (LYG) should be considered when a patient presents with multiple papules, plaques or nodules, constitutional symptoms (fever, fatigue, night sweats, weight loss) and pulmonary symptoms (dyspnea[dermatologyadvisor.com]
Cutaneous Manifestation
  • manifestations of lymphomatoid granulomatosis History and etymology It was Initially described by Leibow et al. in 1972 5 .[radiopaedia.org]
  • Cutaneous manifestations of lymphomatoid granulomatosis: Report of 44 cases and a review of the literature. Arch Dermatol 1981 ; 117: 196 – 202. Google Scholar Crossref Medline 6. Drasga, RE, Williams, SD, Wills, ER, Roth, LM, Einhorn, LH.[journals.sagepub.com]
  • Patients with WG can have similar cutaneous manifestations, also frequently present with constitutional symptoms, and a constellation of cutaneous, pulmonary, and renal disease.[dermatologyadvisor.com]
Sparse Hair
  • Abstract Cartilage-hair hypoplasia (CHH) is an autosomal recessive chondrodysplasia characterized by short-stature, sparse hair and impaired cellular immunity.[ncbi.nlm.nih.gov]
Hair Hypoplasia
  • Abstract Cartilage-hair hypoplasia (CHH) is an autosomal recessive chondrodysplasia characterized by short-stature, sparse hair and impaired cellular immunity.[ncbi.nlm.nih.gov]
Swelling of the Scrotum
  • A 55-year-old gentleman with confirmed lymphomatoid granulomatosis on lung biopsy was noted to have a swelling in his scrotum.[ncbi.nlm.nih.gov]
Testicular Swelling
  • This resulted in resolution of the testicular swelling and his other symptoms. Prognosis with lymphomatoid granulomatosis depends mainly on grade.[ncbi.nlm.nih.gov]
Peripheral Neuropathy
  • Abstract A case of lymphomatoid granulomatosis of the lung is described in which the presenting features were a skin eruption and peripheral neuropathy.[ncbi.nlm.nih.gov]
  • Patients may be asymptomatic at the time of diagnosis, but up to 60% can present non-specific symptoms such as cough, fever, rash, subcutaneous nodules, asthenia and anorexia, dyspnea, chest pain, ataxia or peripheral neuropathy.[archbronconeumol.org]
  • A 48-year-old woman with peripheral neuropathy, hypercalcemia, and pulmonary infiltrates. Chest 1998 ; 114 : 1205 –1209. Lymphomatoid granulomatosis. In : Travis WD, Colby TV, Corrin B, Shimosato Y, Brambilla E.[erj.ersjournals.com]
Aphasia
  • Then he manifested neurologic signs such as seizure, aphasia and right-sided hemiplegia. Chest X-ray and CT scan revealed bilateral pulmonary nodules predominantly in lower lobes and peripheral lung fields. Laboratory exams showed pancytopenia.[ncbi.nlm.nih.gov]
Limb Weakness
  • A 35 year-old homosexual man presented with a 5-week history of headaches followed by a 3-week history of horizontal diplopia, limb weakness and right 6th cranial nerve palsy.[ncbi.nlm.nih.gov]

Workup

  • Broad infectious workup was negative. Given the time course of presentation and anterior accentuation of the lung infiltrates, the greatest clinical concern was radiation pneumonitis followed by drug toxicity.[mdedge.com]
  • Extensive infectious disease workup included negative lyme serology, cryptococcal antigen; HSV, acid fast stain and rapid plasma regain (RPR).[medcraveonline.com]
  • Additional workup showed negative antinuclear antibodies and antineutrophil cytoplasmic antibodies (ANCA). Two skin biopsies were performed, showing a dense T-cell lymphoid infiltrate, EBV-ISH negative.[mjhid.org]
Multiple Pulmonary Nodules
  • Lymphomatoid granulomatosis (LYG) is a rare lung disorder diagnosed by radiological imaging of multiple pulmonary nodules and occasionally induced by methotrexate (MTX) use. To date, the treatment of LYG has not been standardized.[ncbi.nlm.nih.gov]
  • Vahid B, Salerno DA, Marik PE: Lymphomatoid granulomatosis: a rare cause of multiple pulmonary nodules. Respir Care 2008;53:1227–1229.[karger.com]
  • pulmonary nodules Skin involved in nearly half of cases Dermal and subcutaneous nodules show similar features to lung May show nonspecific plaque like dermal infiltrate CNS and kidney also frequently involved High grade lesions with preponderance of[surgpathcriteria.stanford.edu]
  • LYG usually presents as multiple pulmonary nodules on chest radiograph with rapid progression and excavation. Clinically, the most frequent signs are cough, dyspnoea and chest pain. Fever, malaise and weight loss are also commonly observed.[erj.ersjournals.com]
Pulmonary Infiltrate
  • We report the case of a 23-year-old man clinically presented with fever, sweating, and physical intolerance, and bilateral pulmonary infiltrates of nodular type and destructive changes on the chest X-ray, previously treated with antituberculotics for[ncbi.nlm.nih.gov]
  • infiltrates, and serologic evidence of Histoplasma infection Lymphohistiocytic infiltrate with parenchymal necrosis and vasculitis (differential diagnosis with LYG grade 1) Small necrotizing granulomas; granulocytes( ) GMS( ), EBER( ) Chronic form and[basicmedicalkey.com]
  • Given the persistence of the nodular pulmonary infiltrates, emperic itraconazole was started. Over the next several weeks, continued improvement and resolution of the pulmonary infiltrates was noted (Fig 2).[baillement.com]
  • A 48-year-old woman with peripheral neuropathy, hypercalcemia, and pulmonary infiltrates. Chest 1998 ; 114 : 1205 –1209. Lymphomatoid granulomatosis. In : Travis WD, Colby TV, Corrin B, Shimosato Y, Brambilla E.[erj.ersjournals.com]
Bilateral Pulmonary Infiltrate
  • We report the case of a 23-year-old man clinically presented with fever, sweating, and physical intolerance, and bilateral pulmonary infiltrates of nodular type and destructive changes on the chest X-ray, previously treated with antituberculotics for[ncbi.nlm.nih.gov]
  • A crucial factor contributing to the frequent delay in diagnosis of LYG is its ability to mimic various infectious diseases, vasculitides, and malignancies. [5] [10] Bilateral pulmonary infiltrates or nodules are usually seen.[ijcasereportsandimages.com]
Kerley A Lines
  • Chest x-ray revealed increased coarse, linear shadowing in the mid- and lower zones, prominent hila, bilateral Kerley B lines, and a pleural reaction on the left. Nodular shadows were present bilaterally, the largest measuring 18 mm.[doi.org]
Kerley B Lines
  • Chest x-ray revealed increased coarse, linear shadowing in the mid- and lower zones, prominent hila, bilateral Kerley B lines, and a pleural reaction on the left. Nodular shadows were present bilaterally, the largest measuring 18 mm.[doi.org]
Granulomatous Tissue
  • Kradin-RL; Mark-EJ; Belley-G; Norris-PJ; Ton-F A 48-year-old man with a cough and bloody sputum - Wegener's granulomatosis, with microabscesses, capillaritis, granulomatous vasculitis, diffuse granulomatous tissue, and palisading granuloma.[malattierare.regione.veneto.it]
Pleural Effusion
  • As was the experience of others, the laboratory findings in our case were non-specific and although pleural effusion has been known to occur in LYG [12] , commoner causes of pleural effusion in our environment like pneumonia, empyema and tuberculosis[nigeriamedj.com]
  • Massive nodules, pleural effusions, pneumonitis, and cavitary nodules can be seen. Patients with hemoptysis are more likely to have cavitary nodules.[clinicaladvisor.com]
  • Patchy air space disease and pleural effusion have also been reported.[pulmonologyadvisor.com]
  • A small right pleural effusion and little pleural thickening/pleural reaction was reported in dependent parts of the left lower lobe (Figure 1 ). Figure 1 CT scan chest.[doi.org]

Treatment

  • A review of the literature regarding aetiology, clinical features, diagnosis and treatment options is presented.[ncbi.nlm.nih.gov]
  • The disease typically always relapse after successful treatment due to inability of the immune system and current viral drugs to eliminate an EBV-infection.[en.wikipedia.org]
  • The clinical response to treatment was very good, and a post-treatment CT chest shows complete resolution of lung changes.[doi.org]

Prognosis

  • The prognosis for LYG has been reported to be poor, and no satisfactory treatment has been established.[ncbi.nlm.nih.gov]

Etiology

  • No infectious etiology was identified. Following nondiagnostic lung and liver biopsies, the largest pulmonary mass was resected. The histopathologic findings were diagnostic of lymphomatoid granulomatosis. There was no residual B-ALL.[ncbi.nlm.nih.gov]
  • respiratory etiology (hemoptysis)/ progressive NHL References xxx[mdnxs.com]
  • ABSTRACT Lymphomatoid granulomatosis, currently called as extranodal angiocentric and angiodestructive immunoproliferative disorder, is a rare entity of unclear etiology. It involves most frequently lungs, central nervous system and skin.[scirp.org]
  • Lymphomatoid granulomatosis (LYG) is a rare tumor with unknown etiology.[spandidos-publications.com]

Epidemiology

  • Epidemiology First described by Liebow in 1972 Mean age: 48 y/o (range: 7-85 y/o) Predisposed groups: M F ( 2:1) Associated with: Lymphoma, Sjogren’s, chronic viral hepatitis, RA, renal transplant Physiology Pulmonary Vasculitis Angiocentric and angio-destructive[mdnxs.com]
  • […] immune surveillance Relationship between LYG and post-transplant lymphoproliferative disorders is unclear Clinical Associations Congenital and acquired immunodeficiencies Wiskott-Aldrich syndrome HIV infection High-dose chemotherapy CLINICAL ISSUES Epidemiology[basicmedicalkey.com]
  • Epidemiology This is a rare condition, usually presenting in adults 9, but also seen in children with immunodeficiency. The male to female ratio is 2:1 6,9.[e-immunohistochemistry.info]
  • Her main area of interest is cancer epidemiology, with a particular focus on melanoma and other skin cancers. Nagwa M. Elwan is Professor of Dermatology at the Faculty of Medicine, Tanta University, Egypt.[books.google.es]
Sex distribution
Age distribution

Pathophysiology

  • Its pathophysiology is unclear in numerous respects, thus making it difficult to diagnose and treat. We recently encountered a case of LYG that was followed clinically and histologically for 14 months.[ncbi.nlm.nih.gov]
  • Etiology Pathophysiology LYG is an EBV-associated lymphoproliferative disorder. LYG may be more common in patients with immunodeficiency due to incomplete clearance of EBV and the resulting clonal proliferation of atypical EBV-infected B-cells.[dermatologyadvisor.com]
  • Gain fresh perspectives and up-to-date insights from the world’s leading authorities on the pathophysiology, diagnosis, and management of stroke.[books.google.ro]

Prevention

  • Prevention Little can be done to prevent this disease; however, avoiding the Epstein-Barr Virus (human herpesvirus 4) is one preventative measure.[knowcancer.com]
  • Since virtually all patients who did not have complete remission went on to have malignant lymphoma, early recognition and prompt treatment during the lymphomatoid-granulomatosis phase of disease may not only lead to complete remissions but also prevent[nejm.org]
  • How can Lymphomatoid Granulomatosis be Prevented? Presently, the cause of Lymphomatoid Granulomatosis is unknown; hence, there are no known methods to prevent its occurrence.[dovemed.com]

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!