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Pulmonary Lymphomatoid Granulomatosis

Presentation

Pulmonary lymphomatoid granulomatosis is a rare diagnosis that frequently presents with a constellation of seemingly unrelated signs and symptoms. [ncbi.nlm.nih.gov]

Here, we present a case of low grade pulmonary LYG with multiple lung lesions and endobronchial mass which is a rare presentation. A 54-year-old female was admitted with a 1-month history of dyspnea, cough and pulmonary infiltrates. [synapse.koreamed.org]

Immune System

  • Splenomegaly

    A computerized tomography (CT) scan demonstrated bilateral pulmonary nodules and splenomegaly. [ncbi.nlm.nih.gov]

    The child redeveloped splenomegaly and fever, and then suffered an acute decompensation with hypoxemia, tachypnea, splenomegaly, and cardiac gallop. Open-lung biopsy revealed lymphomatoid granulomatosis. [baillement.com]

    Splenomegaly was observed and the SUVmax was 12.4 in the PET/CT image. The CT-guided transthoracic biopsy revealed inflammatory cells and large coagulated necrotic tissue. [spandidos-publications.com]

Entire Body System

  • Fever

    In a 49 year old woman presenting with fever, malaise and pulmonary masses the diagnosis of lymphomatoid granulomatosis was established histologically by open lung biopsy. [ncbi.nlm.nih.gov]

    CASE REPORT A 33-yr-old female was hospitalised with a 3-month history of cough, dyspnoea, fever and skin rash. [erj.ersjournals.com]

    Herein, we describe LYG in a 56-year-old renal allograft recipient 11 years after transplantation, on azathioprine and prednisolone maintenance immunosuppression, presenting to us with fever, weight loss, and nodular and patchy opacities in both lung [ingentaconnect.com]

  • Weight Loss

    Herein, we describe LYG in a 56-year-old renal allograft recipient 11 years after transplantation, on azathioprine and prednisolone maintenance immunosuppression, presenting to us with fever, weight loss, and nodular and patchy opacities in both lung [ingentaconnect.com]

    The symptoms associated with LYG depend on the site of disease involvement but mainly include cough, dyspnea or chest pain (in those with pulmonary involvement) and constitutional symptoms such as weight loss and fever. [orpha.net]

    The commonest presenting picture includes fever, cough, rash/nodules, malaise, weight loss, neurologic abnormalities, dyspnoea, and chest pain (Katzenstein et al. 1979 ). [springerplus.springeropen.com]

    We present a 60-year-old woman with worsening fatigue, night sweats, unintentional weight loss, and dyspnea of 2 weeks’ duration. [mdedge.com]

    Non-Hodgkin lymphoma can cause many symptoms, such as Swollen, painless lymph nodes in the neck, armpits or groin Unexplained weight loss Fever Soaking night sweats Coughing, trouble breathing or chest pain Weakness and tiredness that don't go away Pain [icdlist.com]

  • Disability

    […] for disability benefits. [disability-benefits-help.org]

    Please enable JS and disable any ad blocker [patientcareonline.com]

    E., Jan 1 2019, In : Research in Developmental Disabilities. 84, p. 43-56 14 p. [moh-it.pure.elsevier.com]

    After 34 days of antituberculosis medication, the patient showed a consciousness deterioration and became completely disabled. [journals.lww.com]

  • Swelling

    Non-Hodgkin lymphoma can cause many symptoms, such as Swollen, painless lymph nodes in the neck, armpits or groin Unexplained weight loss Fever Soaking night sweats Coughing, trouble breathing or chest pain Weakness and tiredness that don't go away Pain, swelling [icdlist.com]

    There was no evidence of rash, pallor, lymphadenopathy, clubbing, joint swelling or edema. Auscultation of chest revealed diminished breath sounds with bilateral velcro-like fine inspiratory crackles. [jhoonline.biomedcentral.com]

    MAR 2002; 23 (3) : 292-307 Mandel-L; SurattAnont-F Bilateral parotid swelling: A review ORAL-SURGERY-ORAL-MEDICINE-ORAL-PATHOLOGY-ORAL-RADIOLOGY-AND-ENDODONTICS. [malattierare.regione.veneto.it]

  • Pseudotumor

    Lmphoplasmacytic lymphoma, see WM Thyroid Lymphoma emedicine Inflammatory pseudotumor in lymph nodes pathologyoutlines.com Castleman's disease is associated with lymphoma: "Castleman's disease (CD) is a rare atypical lymphoproliferative disorder whose [lymphomation.org]

    The differential diagnosis includes tuberculosis, granulomatosis, lung cancer and inflammatory pseudotumors. The prognosis of LYG is extremely variable, ranging from extremely good in the cases of spontaneous resolution, to rapidly fatal. [spandidos-publications.com]

Respiratoric

  • Cough

    On clinical examination, this dog presented with nonproductive dry cough, serous nasal discharge, dyspnea, and lack of appetite. Radiography showed a consolidated lesion in the left cranial lung lobe. [ncbi.nlm.nih.gov]

    It most often affects the lungs, causing chest pains, cough, and shortness of breath. [medical-dictionary.thefreedictionary.com]

    The symptoms associated with LYG depend on the site of disease involvement but mainly include cough, dyspnea or chest pain (in those with pulmonary involvement) and constitutional symptoms such as weight loss and fever. [orpha.net]

  • Rales

    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]

    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]

    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]

  • Dry Cough

    On clinical examination, this dog presented with nonproductive dry cough, serous nasal discharge, dyspnea, and lack of appetite. Radiography showed a consolidated lesion in the left cranial lung lobe. [ncbi.nlm.nih.gov]

    Conchillo Armend{\'a}riz and Francisco Vega Vazquez and {\'O}scar Beloqui Ruiz}, journal={Anales De Medicina Interna}, year={1998}, volume={15}, pages={487-489} } We present an AIDs patient who was admitted to our hospital because of dry cough, pleuritic [semanticscholar.org]

    Review of systems was positive only for a dry cough for two months. She denied fever, shortness of breath or weight loss. She was diagnosed with HIV 14 years prior but never sought medical care. [ijcasereportsandimages.com]

    Case report A 19-year-old male was presented to the pulmonary clinic of the People’s hospital of Shenzhen University (Shenzhen, China) with chills, a fever, mild intermittent dry coughing, night sweating and fatigue that had lasted 7 days. [spandidos-publications.com]

    Case Presentation A 66-year-old Caucasian man with no significant past medical history presented with flu-like symptoms, progressively worsening shortness of breath, difficulty in breathing (NYHA class III) and dry cough over the past 2 weeks prior to [jhoonline.biomedcentral.com]

  • Pulmonary Disorder

    […] lymphomatoid granulomatosis (PLG), which has been defined as a clinicopathological entity is a rare Epstein–Barr virus (EBV)-driven lymphoproliferative pulmonary disorder. [journals.lww.com]

    Pulmonary involvement is almost always present, while other common sites include central nervous system (CNS) and skin.[ 2 ] Currently, LYG is considered an EBV related B-lymphoproliferative disorder,[ 3 ] similar to other lymphoproliferative disorders [mjhid.org]

  • Respiratory Distress

    Kradin Springer Science & Business Media, 18.01.2014 - 243 Seiten Viruses that primarily target the lung are very significant causes of death and in the past decade have been responsible for major outbreaks of severe adult respiratory distress syndrome [books.google.de]

    Fever Cough, often with blood Nose bleeds, stuffy nose, pain, perforated nasal septum Weight loss Seizures Hypertension Breathing difficulties/respiratory distress Chest pain Diarrhea Muscle and joint pain Skin spots, nodules, and/or ulcers Other Signs [knowcancer.com]

    Other symptoms such as weight loss, sweating, acute respiratory distress may be present 1, 2, 5. Extrapulmonary site of involvement mainly affect the skin, nervous system, and kidneys 1. [synapse.koreamed.org]

    Upon presentation, the patient did not experience respiratory distress and the vital signs were as follows: Temperature, 40.2°C; heart rate, 110 beats/min; respiratory rate, 24 breaths/min; and blood pressure, 120/70 mmHg. [spandidos-publications.com]

Gastrointestinal

  • Constipation

    […] weakness High temperatures and excessive night sweats (may be recurrent) Frequent infections Anemia (low red blood cell count) Low lymphocyte count, established by a blood test Low blood pressure Back pain Swelling of the legs Abdominal pain and swelling; constipation [dovemed.com]

Skin

  • Cutaneous Manifestation

    […] of lymphomatoid granulomatosis cutaneous manifestations of lymphomatoid granulomatosis History and etymology It was Initially described by Leibow et al. in 1972 5 . [radiopaedia.org]

    Characteristic findings on physical examination Cutaneous lesions are the initial manifestation in up to one third of patients. [dermatologyadvisor.com]

    Books relating to lymphomatoid and brief extracts from same to provide context of its use in English literature. 1 Cutaneous Medicine: Cutaneous Manifestations of Systemic Disease Therapy Therapy of lymphomatoid papulosis is directed toward control of [educalingo.com]

  • Skin Disease

    France: IARC: Lyon; 2008 On DermNet NZ B cell lymphoma Infectious mononucleosis (EBV) Other websites Lymphomatoid Granulomatosis – Medscape Reference Books about skin diseases See the DermNet NZ bookstore. [dermnetnz.org]

  • Dermatitis

    Research output : Contribution to journal › Article Allergic contact dermatitis caused by clindamycin Veraldi, S., Guanziroli, E., Ferrucci, S. & Nazzaro, G., 2019, In : Contact Dermatitis. 80, 1, p. 68-69 Research output : Contribution to journal › Article [moh-it.pure.elsevier.com]

Psychiatrical

  • Suggestibility

    From the results ob-tained here, skin lesion was thought to be an involvement of PLG, which was suggestive of lympho-prolif erative disorder. [jstage.jst.go.jp]

    No significant bronchial obstruction was identified to suggest post-obstructive pneumonia and there was nothing in the history to suggest aspiration. The symptoms suggestive of lung abscess may not have been caused by infection. [thorax.bmj.com]

    S ubsequent reports suggested that some cases went on to develop T-cell lymphomas. [histopathology-india.net]

    Upper airway involvement such as sinusitis suggests Wegener granulomatosis. Medical renal disease is associated with Wegener granulomatosis and Churg-Strauss syndrome. [pubs.rsna.org]

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]

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]

X-Ray

  • Pulmonary Infiltrate

    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]

    Here, we present a case of low grade pulmonary LYG with multiple lung lesions and endobronchial mass which is a rare presentation. A 54-year-old female was admitted with a 1-month history of dyspnea, cough and pulmonary infiltrates. [synapse.koreamed.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]

    On hospital day-3, a biopsy of the pulmonary lesions via video-assisted thoracoscopy (VATS) was obtained. Pathology of the pulmonary nodule revealed an angiocentric infiltrate of lymphocytes with large areas of necrosis. [ijcasereportsandimages.com]

  • Pericardial Effusion

    A chest-CT revealed a left-sided pleural effusion, lobus inferior pulmonis atelectasis and small amounts of pericardial effusion. The patient received 2 l/min oxygen by nasal canula. [spandidos-publications.com]

Other Pathologies

  • 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]

Treatment

PLG is even harder to cure because of the lack of an established treatment strategy. [journals.lww.com]

The clinical response to treatment was very good, and a post-treatment CT chest shows complete resolution of lung changes. [springerplus.springeropen.com]

If the disease is localized, radiation treatment can be useful. Surgery and chemotherapy has helped some patients. Other Treatment Options Bone marrow transplants Interferon-alfa 2b treatments Antiviral therapy Rituximab [knowcancer.com]

Open lung biopsy and total excision is mandatory for the appropriate diagnosis and treatment. [ncbi.nlm.nih.gov]

Prognosis

Lymphoma diagnosed in this way was associated with a better prognosis than lymphoma diagnosed on the basis of conventional histopathologic findings. In three patients, solid tumors eventually developed. [ncbi.nlm.nih.gov]

Prognosis 64% mortality, median survival of 14 months; death is due to pulmonary destruction accompanied by sepsis; severe T-cell impairment may explain the malignant evolution of this condition. [medical-dictionary.thefreedictionary.com]

[…] confined to site of origin, the prognosis is usually excellent with appropriate therapy. [dovemed.com]

Prognosis: The prognosis is poor, the median survival being only fourteen months after diagnosis. [histopathology-india.net]

Etiology

Extensive work-up for neoplastic, infectious, inflammatory, and autoimmune etiologies needs to be done in such cases. A prompt diagnosis of LYG is necessary for optimal management and improved patient outcomes. [ncbi.nlm.nih.gov]

Lymphomatoid granulomatosis (LYG) is a rare tumor with unknown etiology. [spandidos-publications.com]

Differential diagnosis at this time was extensive with an infectious etiology considered most likely, but malignancy also a serious consideration. [ijcasereportsandimages.com]

Etiology: - L ymphomatoid granulomatosis is closely associated with EBV. - P atients may have underlying defects in their cell-mediated immunity. [histopathology-india.net]

The etiology is not known, but there is an association collagen vascular diseases, AIDS, and immunodeficiency. [icnet.uk]

Epidemiology

Abstract We reviewed the epidemiologic, laboratory, roentgenographic, pulmonary function, and survival data from 28 patients who had a histologic diagnosis of lymphomatoid granulomatosis (LG) with involvement of the lungs. [ncbi.nlm.nih.gov]

The epidemiologic, ultrastructural, immunologic, and clinicopathologic features of well-known viral pathogens and newer emergent infectious agents are discussed in detail. [books.google.de]

Methods Statistics Trigonometry Medical & Nursing Anatomy Anesthesiology Audiology Bacteriology Biochemistry Bioethics Biomedical Science Cardiology Cardiovascular Childbirth Chiropractic Dentistry Dermatology Diagnostic Imaging Drugs Endocrinology Epidemiology [brainscape.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]

While the epidemiology of neurological complications has changed considerably since the introduction of highly active antiretroviral therapy (HAART), a wide spectrum of neurological complications in HIV-infection individuals continues to be seen. [ijcasereportsandimages.com]

Pathophysiology

[…] infection and immunosuppression through immunosuppressive drugs (with case reports of methotrexate [6] [7] [8] [9] [10] [11] [12] [13] [14] and azathioprine [15] [16] ), infections such as HIV or chronic viral hepatitis or endogenous T cell defects. [17] Pathophysiology [en.wikipedia.org]

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]

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]

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]

Radiotherapy was delivered to the whole brain, however, this treatment did not prevent progression of the disease and the patient succumbed three months after initial presentation. [spandidos-publications.com]

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