Presentation
Two patients presented with a symmetrical inflammatory polyarthropathy. Both patients fulfilled the diagnostic criteria for angioimmunoblastic lymphadenopathy. We present the two case histories and review the current literature. [ncbi.nlm.nih.gov]
Immune System
- Generalized Lymphadenopathy
lymphadenopathy [ lim-fad″ĕ-nop´ah-the ] angioimmunoblastic lymphadenopathy ( angioimmunoblastic lymphadenopathy with dysproteinemia (AILD)) a systemic disorder resembling lymphoma characterized by fever, night sweats, weight loss, generalized lymphadenopathy [medical-dictionary.thefreedictionary.com]
A 55-year-old Japanese man presented with red papular lesions on the trunk and limbs, oedema, and generalized lymphadenopathy. [ncbi.nlm.nih.gov]
- Splenomegaly
A woman 68 years of age had fever, malaise, diffuse lymphadenopathy, splenomegaly followed by abdominal pain, and diarrhea. A lymph node biopsy specimen showed nonspecific follicular hyperplasia. Symptoms were responsive initially to prednisone. [ncbi.nlm.nih.gov]
Her symptoms had developed progressively, and a physical examination revealed bilateral cervical lymphadenopathy and splenomegaly. [jpatholtm.org]
The presence of particular symptoms, of possible splenomegaly, of elevated indices of inflammation should all be evaluated very carefully in order to avoid unnecessarily spending time investigating a restricted area for a LAM that actually only appears [kipdf.com]
Sonography of the abdomen indicated homogenous splenomegaly. [medicaljournals.se]
Entire Body System
- Lymphadenopathy
Angioimmunoblastic lymphadenopathy with dysproteinemia is a disorder characterized by an acute onset of generalized lymphadenopathy associated with fever, malaise, pruritus, night sweats, and hepatosplenomegaly. [ncbi.nlm.nih.gov]
- Fever
[…] lymphadenopathy [ lim-fad″ĕ-nop´ah-the ] angioimmunoblastic lymphadenopathy ( angioimmunoblastic lymphadenopathy with dysproteinemia (AILD)) a systemic disorder resembling lymphoma characterized by fever, night sweats, weight loss, generalized lymphadenopathy [medical-dictionary.thefreedictionary.com]
Angioimmunoblastic lymphadenopathy (AILD) is a rare disorder characterised by generalised lymphadenopathy, fever, hepatosplenomegaly, immune hemolytic anemia and polyclonal hypergammaglobulinemia. [ncbi.nlm.nih.gov]
- Anemia
AILD is characterized by generalized lymphadenopathy, hepatosplenomegaly, immunological abnormalities, polyclonal hypergammaglobulinemia and anemia. [ncbi.nlm.nih.gov]
[…] with dysproteinemia (AILD)) a systemic disorder resembling lymphoma characterized by fever, night sweats, weight loss, generalized lymphadenopathy, hepatosplenomegaly, macropapular rash, polyclonal hypergammaglobulinemia, and Coombs'-positive hemolytic anemia [medical-dictionary.thefreedictionary.com]
- Weight Loss
[…] lymphadenopathy [ lim-fad″ĕ-nop´ah-the ] angioimmunoblastic lymphadenopathy ( angioimmunoblastic lymphadenopathy with dysproteinemia (AILD)) a systemic disorder resembling lymphoma characterized by fever, night sweats, weight loss, generalized lymphadenopathy [medical-dictionary.thefreedictionary.com]
Patients typically have generalized lymphadenopathy, fever, weight loss, skin rash, polyclonal hypergammaglobulinemia and autoimmune phenomena, and are susceptible to infections. The clinical course is usually aggressive. [ncbi.nlm.nih.gov]
Other symptoms may include a skin rash, fever, weight loss, or night sweats. ICD-10-CM C86.5 is grouped within Diagnostic Related Group(s) (MS-DRG v 36.0): 820 Lymphoma and leukemia with major o.r. [icd10data.com]
Most patients present with generalized lymphadenopathy, hepatosplenomegaly, skin rash and general symptoms (fever, weight loss). Polyclonal hypergammaglobulinemia is a common finding. [atlasgeneticsoncology.org]
- Malaise
Angioimmunoblastic lymphadenopathy with dysproteinemia is a disorder characterized by an acute onset of generalized lymphadenopathy associated with fever, malaise, pruritus, night sweats, and hepatosplenomegaly. [ncbi.nlm.nih.gov]
Symmetric RA-like polyarthritis in the pre-icteric phase of acute hepatitis Associated with rash, fever, malaise and myalgias Positive RF in approx. 25 % of cases Low levels of C3 and C4 Spontaneous resolution after 2 to 3 weeks II. [usdocument.net]
Respiratoric
- Pleural Effusion
Occasionally, pulmonary involvement, including pleural effusion, has also been observed. Two cases of AILD accompanied by pleural effusion are reported here. [ncbi.nlm.nih.gov]
2017;8:17763 ) Clinical features Generalized lymphadenopathy (stage III or IV disease), hepatosplenomegaly, and skin rash B symptoms: fever, night sweats, weight loss ( Hsi: Hematopathology, Second Edition, 2012 ) Less common: arthralgias or arthritis, pleural [pathologyoutlines.com]
- Aspiration
A case of AILD with pulmonary involvement that was initially mistaken for tuberculosis on fine needle aspiration cytology and put on antituberculous treatment for three months, is presented here. [ncbi.nlm.nih.gov]
Lymph node staging in patients with clinically negative neck examinations by ultrasound and ultrasound-guided aspiration cytology. [books.google.de]
When the cause of lymphadenopathy is not clear, doctors may advise having tests such as fine needle aspiration cytology, or FNAC. [verywell.com]
You might also have one of these tests to help diagnose, stage, or manage lymphoma: Bone marrow aspiration or biopsy. [webmd.com]
Univariate and multivariate analysis of the diagnostic results of transbronchial needle aspiration were performed ... [citeulike.org]
- Cough
[…] that kill bugs and weeds Were treated for Hodgkin or non-Hodgkin lymphoma in the past Were treated for cancer with radiation Symptoms Warning signs of lymphoma include: Swollen glands (lymph nodes), often in the neck, armpit, or groin that are painless Cough [webmd.com]
[…] staging system: unexplained fever > 38°C; weight loss >10% body weight over 6 months, night sweats = pruritus other sistemic: pruritus Tumor-related symptoms Superficial adenopathy > 1cm for more than 4 weeks (wax and wane in low-grade lymphomas) Thorax (cough [slideplayer.it]
Case presentation Clinical summary A 40 year-old-male patient presented to his general practitioner with a 3-weeks history of myalgia, arthralgia, fever (37.5°C) and cough which persisted despite a broad-spectrum antibiotic therapy. [diagnosticpathology.biomedcentral.com]
[…] mediastinal structures: a) compression of the vena cava (headache, congestion of the head and neck, turgor of the jugular veins, congestion of the upper part of the thorax and the arms, small mantle edema); b) compression of the bronchial branches (harsh dry cough [kipdf.com]
A 74-year-old man presented with cough, sputum and mental change. Chest imaging showed massive mediastinal lymphadenopathy with obstructive pneumonia, and multiple metastases in lung and adrenal gland. Bronchoscopy ... [citeulike.org]
- Pharyngitis
A case of AILD with an ulcer of the lateral pharyngeal wall was studied for viral infection, immunohistologic findings and T-cell receptor (TCR) V beta rearrangement. [ncbi.nlm.nih.gov]
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Gastrointestinal
- Diarrhea
A woman 68 years of age had fever, malaise, diffuse lymphadenopathy, splenomegaly followed by abdominal pain, and diarrhea. A lymph node biopsy specimen showed nonspecific follicular hyperplasia. Symptoms were responsive initially to prednisone. [ncbi.nlm.nih.gov]
Arthritis in Brucellosis Mainly monoarticular Affecting weight-bearing joints Some patients with pauciarticular involvement Few with a symmetric, rheumatoid-like pattern of arthritis The challenge of Whipple disease Middle-aged men with weight loss and diarrhea [usdocument.net]
The patient presented with fever, abdominal mass, ascites, diarrhea, generalized lymphadenopathy, anemia, and marked peripheral eosinophilia. [28] A presenting sign of angioimmunoblastic T-cell lymphoma can be saggy skin. [29] Differential Diagnosis Angioimmunoblastic [emedicine.medscape.com]
Liver, Gall & Pancreas
- Hepatosplenomegaly
There are cellular infiltration of lymphocytes, immunoblasts, and plasma cells; change or effacement of lymph node architecture; hepatosplenomegaly; maculopapular rash; polyclonal hypergammaglobulinemia; and Coombs' positive hemolytic anemia. [medical-dictionary.thefreedictionary.com]
AILD is characterized by generalized lymphadenopathy, hepatosplenomegaly, immunological abnormalities, polyclonal hypergammaglobulinemia and anemia. [ncbi.nlm.nih.gov]
- Hepatomegaly
In one series, other symptoms included weight loss (58%), hepatomegaly (60%), polyclonal hyperglobulinemia (65%), and generalized adenopathy (87%). Patients are usually aged 40-90 years. [emedicine.medscape.com]
Musculoskeletal
- Arthritis
The arthritis was difficult to control, requiring large doses of corticosteroids, which produced significant side effects. [opus.bath.ac.uk]
In this article, we report on a patient with seronegative Rheumatoid Arthritis of 18 years duration who recently developed AILD. [ncbi.nlm.nih.gov]
- Arthralgia
2017;31:223, Oncotarget 2017;8:17763 ) Clinical features Generalized lymphadenopathy (stage III or IV disease), hepatosplenomegaly, and skin rash B symptoms: fever, night sweats, weight loss ( Hsi: Hematopathology, Second Edition, 2012 ) Less common: arthralgias [pathologyoutlines.com]
Autoimmunity Rev 2008 Rheumatic syndromes frequently observed with HIV infection Articular syndromes Arthralgia Reiter s syndrome Psoriatic arthritis Undifferentiated spondyloarthropathy Idiopathic or HIV-associated arthritis Septic arthritis Osteonecrosis [usdocument.net]
She reported no night sweats, loss of weight, or arthralgia. A physical examination conducted at presentation revealed bilateral, multiple enlarged lymph nodes on both sides of the neck with splenomegaly. [jpatholtm.org]
Case presentation Clinical summary A 40 year-old-male patient presented to his general practitioner with a 3-weeks history of myalgia, arthralgia, fever (37.5°C) and cough which persisted despite a broad-spectrum antibiotic therapy. [diagnosticpathology.biomedcentral.com]
Skin
- Night Sweats
Angioimmunoblastic lymphadenopathy with dysproteinemia is a disorder characterized by an acute onset of generalized lymphadenopathy associated with fever, malaise, pruritus, night sweats, and hepatosplenomegaly. [ncbi.nlm.nih.gov]
[…] lymphadenopathy [ lim-fad″ĕ-nop´ah-the ] angioimmunoblastic lymphadenopathy ( angioimmunoblastic lymphadenopathy with dysproteinemia (AILD)) a systemic disorder resembling lymphoma characterized by fever, night sweats, weight loss, generalized lymphadenopathy [medical-dictionary.thefreedictionary.com]
B indicates that an affected individual has experienced drenching night sweats, unexplained fever (above 38 degrees Celsius), and/or unexplained weight loss (i.e., loss of at least 10 percent of total body weight in the six months prior to diagnosis). [rarediseases.org]
sweats, weight loss ( Hsi: Hematopathology, Second Edition, 2012 ) Less common: arthralgias or arthritis, pleural effusion, ascites, pulmonary involvement, neurological involvement and gastrointestinal involvement Polyclonal hypergammaglobulinemia and [pathologyoutlines.com]
- Eczema
1759 uvula cerebellii uterovaginale Page 1772 and 1773: 1761 vaginal fornixV* V 26766; 2678 Page 1774 and 1775: 1763 valve of coronary sinus26759 v Page 1776 and 1777: 1765 vanillylmandelic acid* valvula Page 1778 and 1779: 1767 vascular dementiai eczema [yumpu.com]
Workup
Biopsies of the bone marrow, lymph node, and skin are key in diagnosing AILD (see Workup). Many agents have been used to treat AILD, but none has proved universally or consistently effective (see Treatment and Management). [emedicine.medscape.com]
Infectious workup ... Note (first note only) ID: 613521981 Abstract Drug rash with eosinophilia and systemic symptoms (DRESS) syndrome is a rare, complex, idiosyncratic drug reaction that can be fatal. [citeulike.org]
Serum
- Lymphopenia
It is characterized by acute onset, severe constitutional symptoms, cervical or generalized lymphadenopathy, lymphopenia, and polyclonal hypergammaglobulinemia, all of which are highly suggestive of a viral origin. [ncbi.nlm.nih.gov]
Lymphopenia was seen in 2 patients. 4. Lymphocytes with azure granules which encompass T-suppressors were found to exceed normal values by a factor of 15. 5. [scipers.com]
[…] clinical presentation Testing for clonal T cell receptor rearrangement can be helpful ( eMedicine ) Laboratory Polyclonal hypergammaglobulinemia and autoimmune hemolytic anemia ( Hsi: Hematopathology, Second Edition, 2012 ) Eosinophilia, thrombocytopenia, lymphopenia [pathologyoutlines.com]
- Hyperglobulinemia
In one series, other symptoms included weight loss (58%), hepatomegaly (60%), polyclonal hyperglobulinemia (65%), and generalized adenopathy (87%). Patients are usually aged 40-90 years. [emedicine.medscape.com]
Other Pathologies
- Lymph Node Hyperplasia
Atypical lymph node hyperplasia with fatal outcome. A report on the histopathological, im- munological and clinical investigations of the cases. Recent Adv RES Res 14: 13-34, 1974. Frizzera G, Moran EM, and Rappaport H. [docslide.com.br]
Other problems to be considered in patients with possible angioimmunoblastic lymphoma include the following: Multicentric giant lymph node hyperplasia Acute viral lymphadenitis Hypersensitivity syndrome (eg, dapsone) [31] Lymphoma Leukemia Viral exanthema [emedicine.medscape.com]
Pleura
- Pleural Effusion
Occasionally, pulmonary involvement, including pleural effusion, has also been observed. Two cases of AILD accompanied by pleural effusion are reported here. [ncbi.nlm.nih.gov]
2017;8:17763 ) Clinical features Generalized lymphadenopathy (stage III or IV disease), hepatosplenomegaly, and skin rash B symptoms: fever, night sweats, weight loss ( Hsi: Hematopathology, Second Edition, 2012 ) Less common: arthralgias or arthritis, pleural [pathologyoutlines.com]
Treatment
Six received interferon as primary and six as secondary treatment after the failure of previous treatment with prednisone or chemotherapy. [ncbi.nlm.nih.gov]
Continued Treatment The treatment you get depends on what type of lymphoma you have and its stage. [webmd.com]
Prognosis
The prognosis of AILD is generally poor, with a median survival of less than 20 months. Our patient died two and a half months after the diagnosis was made due to sepsis caused by Staphylococcus aureus isolated in hemoculture. [ncbi.nlm.nih.gov]
They often had poor response to therapies, resulted in unfavorable prognosis. Therefore, it was suspected that the cystological findings of lymph nodes were correlative to the clinical findings and prognoses of patients with this disease. [ci.nii.ac.jp]
Etiology
The data support the hypothesis that EBV was not etiologically related to AILD in this case, and that EBV proliferation may occur after the onset of the disease. [ncbi.nlm.nih.gov]
Article in Italian | MEDLINE | ID: mdl-3991968 Subject(s) Anemia, Hemolytic, Autoimmune/etiology Blood Protein Disorders/complications Immunoblastic Lymphadenopathy/complications Aged Humans Male Related documents PubMed Links powered by iAHx-2.10-126 [pesquisa.bvsalud.org]
Etiology The disease is rare Clinics The disease preferentially affects elderly males (male-to-female ratio 3:1, median age around 60 years). [atlasgeneticsoncology.org]
[…] dysproteinemia, immunoblastic lymphadenopathy, lymphogranulomatous X and immunodysplastic disease ( Hsi: Hematopathology, Second Edition, 2012 ) Acknowledged entity since the 1994 Revised European American Lymphoma (REAL) Classification ( Blood 1994;84:1361 ) Etiology [pathologyoutlines.com]
Epidemiology
Epidemiology The typical patient with angioimmunoblastic T-cell lymphoma (AILT/AILD) is either middle-aged or elderly, and no gender preference for this disease has been observed. [wiki.clinicalflow.com]
Epidemiology [ edit ] The typical patient with angioimmunoblastic T-cell lymphoma (AITL) is either middle-aged or elderly, and no gender preference for this disease has been observed. [1] AITL comprises 15–20% of peripheral T-cell lymphomas and 1–2% of [en.wikipedia.org]
It was intended that such material would have constituted the basis of a hypothetic decision-making tree, but this was impossible because of the lack of epidemiological investigation and registry data. [kipdf.com]
Epidemiology The exact incidence of angioimmunoblastic lymphadenopathy with dysproteinemia (AILD) is not known. In the United States, approximately 1-2% of non-Hodgkin lymphomas are associated with AILD. [emedicine.medscape.com]
Pathophysiology
Pathophysiology Evidence exists that angioimmunoblastic lymphadenopathy with dysproteinemia (AILD) develops in a serial fashion. The initial reaction may be an unbalanced immune response to an unknown antigen. [emedicine.medscape.com]
Prevention
Prevention of this potentially fatal disease in immunocompromised patients by early diagnosis and proper management of intestinal strongyloidiasis is emphasized. [ncbi.nlm.nih.gov]