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Angioimmunoblastic Lymphadenopathy

Angio Immunoblastic Lymphadenopathy


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]
Generalized Lymphadenopathy
  • 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]
  • Sonography of the abdomen indicated homogenous splenomegaly.[medicaljournals.se]
  • 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]
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
  • Although complete remission of AML was achieved, fever persisted and atypical skin rash followed by generalized lymphadenopathy along with polyclonal hypergammaglobulinemia appeared.[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
  • 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]
  • […] 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]
  • loss Itching Many of these symptoms can also be warning signs of other illnesses.[webmd.com]
  • 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]
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]
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]
  • Arbor 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]
  • […] n 18 Page 1228 and 1229: 1217 perpendicular plate of palatin Page 1230 and 1231: 1219 petrochemicalinsetticida md Pe Page 1232 and 1233: 1221 phagocyteWeber-Peutz nt -µ; Page 1234 and 1235: 1223 phantom limb* Phalangida npl Page 1236 and 1237: 1225 pharyngeal[yumpu.com]
Diarrhea
  • The patient presented with fever, abdominal mass, ascites, diarrhea, generalized lymphadenopathy, anemia, and marked peripheral eosinophilia. She had multiple polyps in the colon, as well as in the stomach and duodenum.[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]
Hepatosplenomegaly
  • 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]
Arthralgia
  • 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]
  • 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]
  • 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]
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]
  • sweats Fatigue Weight loss Itching Many of these symptoms can also be warning signs of other illnesses.[webmd.com]
  • 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]
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]
Excoriation
  • A 60 year old male presented with pruritus, excoriations and enlargement of several groups of lymph nodes associated with constitutional symptoms following the intake of an aspirin preparation.[ncbi.nlm.nih.gov]

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]
Pericardial Effusion
  • During a leukopenic period, the patient suffered from pericarditis with massive pericardial effusion in which human herpesvirus 6 (HHV-6) DNA was detected.[ncbi.nlm.nih.gov]
Hyperviscosity
  • Fundoscopy revealed papilledema compatible with hyperviscosity syndrome. Plasmapheresis resulted in a slow recovery of vision when suddenly a bilateral loss of vision occurred.[ncbi.nlm.nih.gov]
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]

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]

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]
  • The prognosis of this disorder is poor; 75% of patients die within 2 years or develop a lymphoid malignancy. The rest usually go into a sustained remission.[annals.org]

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 GHL[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]
  • 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 [ 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]
  • 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]
Sex distribution
Age distribution

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]

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