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]
- Hodgkin Lymphoma
Lymphoma Also called: Non-Hodgkin lymphoma Lymphoma is a cancer of a part of the immune system called the lymph system. There are many types of lymphoma. One type is Hodgkin disease. The rest are called non-Hodgkin lymphomas. [icdlist.com]
Kim JH, Lee SH, Park J, Kim HY, Lee S, Park JH, et al. (2004) Primary pulmonary non Hodgkin’s lymphoma. [link.springer.com]
B-cell lymphoproliferative diseases, Hodgkin’s lymphoma (HL), and T-cell lymphomas. [lymphomation.org]
lymphoma, the Ann Arbor System is also used for non-Hodgkin lymphomas. [surgpathcriteria.stanford.edu]
- Inflammation
Fifteen patients had Wegener's granulomatosis characterized by necrotizing granulomatous inflammation of lung, frequent upper airway and renal involvement, and responsiveness to cyclophosphamide. [annals.org]
Fiberoptic bronchoscopy was performed in 74 patients, and it was macroscopically abnormal in 55 percent (showing bronchial inflammation or stenosis or both or isolated hemorrhage). Six patients presented with alveolar hemorrhagic syndrome. [ncbi.nlm.nih.gov]
Pathologic examination of these entities demonstrates vasculitis, granulomatous inflammation, and parenchymal necrosis. [pubs.rsna.org]
[…] hybridization, CD3 + T cells in background Molecular / cytogenetics description B cells are clonal (IgH rearrangements), but T cells are not Differential diagnosis Inflammatory lesions (neutrophils, less prominent lymphocytic infiltrate), granulomatous inflammation [pathologyoutlines.com]
- Pathologist
A pathologist examines the biopsy under a microscope. After putting together clinical findings, special studies on tissues (if needed) and with microscope findings, the pathologist arrives at a definitive diagnosis. [dovemed.com]
Am J Dermatopathol ... 10 Inflammatory Dermatopathology: A Pathologist's Survival Guide The current WHO-EORTC classification includes lymphomatoid papulosis as a CD30+ lymphoproliferative disorder along with cutaneous anaplastic large cell lymphoma. [educalingo.com]
Grade II/III LYG likely raise the suspicion of a malignant lymphoproliferative disease even in the hands of general pathologist. [shortnessofbreath.it]
Lymphomatoid granulomatosis: a practical review for pathologists dealing with this rare pulmonary lymphoproliferative process. Pathologica 2013;105:111–116. 9. Campo E, Swerdlow SH, Harris NL, Pileri S, Stein H, Jaffe ES. [synapse.koreamed.org]
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]
Skin
- Sweating
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]
[…] involved IIIE if extralymphatic site involved Stage IV Diffuse or disseminated involvement of one or more extralymphatic organs or tissues, with or without associated lymph node involvement Systemic Symptoms in 6 months preceding admission Fever, night sweats [surgpathcriteria.stanford.edu]
Constitutional symptoms, including weight loss, fatigue, sweating and chills, are typical ( 6 ). [spandidos-publications.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]
Psychiatrical
- Aggressive Behavior
Both the CNS cases were Grade 3 correlating with its aggressive behavior. Two of the three Grade 3 lesions were in young patients. Fifty percent of the young patients had Grade 3 lesions. The patient with CVID had Grade 3 lesion. [ijpmonline.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
- Multiple Pulmonary Nodules
This demonstrates the potential for diagnostic difficulty in a case presentation of multiple pulmonary nodules. Extensive work-up for neoplastic, infectious, inflammatory, and autoimmune etiologies needs to be done in such cases. [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]
A chest radiograph revealed bilateral nodules of variable sizes predominantly in the lower lung fields, and a computed tomography (CT) scan of the thorax showed multiple small excavated pulmonary nodules with poorly defined margins (fig. 1 ⇓ ). [erj.ersjournals.com]
- 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]
- Atelectasis
Cyclophosphamide therapy was started and 1 year later, there was complete regression of endobronchial tumor and atelectasis in LUL as well as multiple lung nodules ( Figure 5 ). [synapse.koreamed.org]
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]