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Malignant Angioendotheliomatosis

Intravascular Lymphomatosis


Presentation

  • We review the characteristics and treatment of this uncommon disorder and present an illustrative case.[ncbi.nlm.nih.gov]
Fever
  • An early diagnosis of MAE in the setting of fever and SIADH may be possible via transbronchial biopsy.[ncbi.nlm.nih.gov]
  • Moreover, this disorder should be considered in the differential diagnosis of prolonged fever of unknown origin.[documents.mx]
  • Case Report A 65-year-old woman was admitted to Emory University Hospital for evaluation of fever of unknown origin, anasarca, and recurrence of hemolytic anemia.[myslide.es]
Weight Loss
  • The general signs and symptoms may include weight loss, fatigue, headaches, and frequent infections.[dovemed.com]
  • Central nervous system and cutaneous involvement is common, as is the presence of B symptoms including fever, weight loss, and night sweats. Morphologically, growth of neoplastic cells is restricted to the lumen of small vessels.[ncbi.nlm.nih.gov]
  • Presenting symptoms were fever (52%), neurological (51%), fatigue (46%), skin lesions (28%), night sweats (23%), weight loss (23%), edema (22%), gastrointestinal (14%), respiratory (14%), genitourinary (7%), musculoskeletal (5%) and cardiovascular (1%[bloodjournal.org]
Lymphadenopathy
  • There was no lymphadenopathy; however, the spleen was palpable 5 cm below the left costal margin.[myslide.es]
  • […] malignant, primary site (C84.1) M9702/3 Peripheral T-cell lymphoma NOS, primary site (C84.4) M9703/3 T-zone lymphoma (C84.2) M9704/3 Lymphoepithelioid lymphoma, malignant, primary site (C84.3) M9705/3 Peripheral T-cell lymphoma, AILD (angioimmunoblastic lymphadenopathy[www0.sun.ac.za]
  • There was no hepatosplenomegaly or lymphadenopathy. A skin biopsy was done, the results of which were consistent with APS.[docslide.com.br]
  • Research output : Contribution to journal › Article Evidence for a polyclonal nature of the cell infiltrate in sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) Paulli, M. , Bergamaschi, G. , Tonon, L., Viglio, A. , Rosso, R. ,[moh-it.pure.elsevier.com]
  • Immunoblastic lymphoma is the most common subtype of diffuse large B-cell lymphoma arising on the basis of natural immunodeficiency, immunosuppression, immunoproliferative states (such as angioimmunoblastic lymphadenopathy), and other immune-mediated[epathologies.com]
Fatigue
  • The general signs and symptoms may include weight loss, fatigue, headaches, and frequent infections.[dovemed.com]
  • Presenting symptoms were fever (52%), neurological (51%), fatigue (46%), skin lesions (28%), night sweats (23%), weight loss (23%), edema (22%), gastrointestinal (14%), respiratory (14%), genitourinary (7%), musculoskeletal (5%) and cardiovascular (1%[bloodjournal.org]
  • Case report A previously healthy 87-year-old caucasian male developed head- ache, fatigue and dizziness 3 months prior to death. He had a transient skin rash and subfebrile temperatures.[myslide.es]
  • Three weeks later, he reported fatigue and lethargy and was found to be severely anemic (hemoglobin, 7.8 g/dL).[ajnr.org]
  • Case Report A 68-year-old woman presented with pancytopenia, abdominal pain, generalized fatigue, and fever in September 2006.[hematologyandoncology.net]
Hypoxemia
  • We describe a case of intravascular lymphomatosis resulting in pulmonary hypertension, hypoxemia, and dyspnea.[ncbi.nlm.nih.gov]
  • Martusewicz-Boros M, Wiatr E, Radzikowska E, et al: Pulmonary intravascular large B-cell lymphoma as a cause of severe hypoxemia. J Clin Oncol 25:: 2137 , 2007 -2139, Link , Google Scholar 29.[doi.org]
  • Probably different stimuli (eg, bacteria, viruses, cryptoproteins) can lead to the vessels occlusion, hypoxemia, and subsequently endothelial cell proliferation.[emedicine.medscape.com]
Dyspnea
  • We describe a case of intravascular lymphomatosis resulting in pulmonary hypertension, hypoxemia, and dyspnea.[ncbi.nlm.nih.gov]
  • Clinical History: This 63 year-old male presented with progressive dyspnea over 4 months, dry cough, low-grade fever and chills, and 19-pound weight loss.[uscapknowledgehub.org]
Hypertension
  • Intravascular lymphomatosis should be considered in the differential diagnosis of pulmonary hypertension.[ncbi.nlm.nih.gov]
  • Case The patient was a 49 year-old Caucasian female legal assistant, with a history of mild hypertension, taking hydrochlorthiazide and metoprolol.[f1000research.com]
  • English Malignant hypertension Portuguese Hipertensão maligna Last Update: 2017-04-26 Usage Frequency: 1 Reference: Wikipedia English malignant neoplasms, Portuguese os tumores malignos, Last Update: 2017-03-15 Usage Frequency: 1 Reference: Translated.com[mymemory.translated.net]
  • Past medical history was significant for single vessel atherosclerotic cardiovascular disease, hyperlipidemia, hypertension, and peptic ulcer disease. Chest x-ray revealed a retrocardiac infiltrate.[uscapknowledgehub.org]
Short Arm
  • arm) as well as a novel translocation involving the short arms (p) of chro- mosomes no. 1 and no. 3.[documents.mx]
Aged Face
  • 451 The Archaeology of Joint Disease 463 Topical Retinoids in the Treatment of Aging of the Skin 477 Carbon Dioxide Laser Resurfacing of the Aged Face 483 Melasma 491 Skin Tumours in Prematurely Photoaged Skin after LongTerm 501 Surface Evaluation of[books.google.com]
Confusion
  • […] morphologic similarity of the malignant intravascular cells and peripheral blood cells, as well as the demonstration of surface membrane immunoglobulin on malignant blood cells suggested that, in some cases, unusual features of malignant lymphoma may be confused[ncbi.nlm.nih.gov]
  • Anemia was detected and the patient showed recurrent episodes of confusion and disorientation. The search for an underlying malignancy including bone marrow examination revealed no abnormalities. Upon admission he was alert and slightly confused.[myslide.es]
  • […] ligament . abdominopelvic sarcoma of perivascular epitheloid cells [2] primary extrapulmonary sugar tumour [2] Thus, it has been advocated that the above could be classified PEComas. [1] PEComas are rare and can have myriad features; therefore, they can be confused[en.wikipedia.org]
  • In the past, these cutaneous lesions have been confused with mycosis fungoides, sarcoidosis, vas- cular neoplasms, or cutaneous involvement by lymphoma or l e kemia . FIGS. 1 A- IC. Angiotropic lymphoma/malignant angioendothelio- matosis.[documents.mx]
  • Two weeks later, the patient was readmitted with increased confusion, headaches, lower extremity paresis, and gait instability.[ajnr.org]

Workup

  • Because brain biopsies are not routinely included in the diagnostic workup of non-neoplastic CNS diseases, a number of unconfirmed nonvasculitic conditions may mimic PACNS.[ncbi.nlm.nih.gov]
  • Negative workup. Surgical biopsy. p63 CK5/6 LCA LCA CD20 CD20 CD20 Diagnostic proposé: Lymphome malin diffus à grandes cellules B, variante anaplasique Proposed diagnosis: Diffuse Large B-Cell Lymphoma, Anaplastic variant.[epathologies.com]
  • Results for a workup for anemia, including blood cultures, HIV testing, and a Coomb’s antiglobulin test were negative.[ajnr.org]
Pulmonary Infiltrate
  • The absence of pulmonary infiltrates precluded any lung biopsy. Autopsy revealed malignant angioendotheliomatosis involving multiple organs including the alveolar septa and pulmonary vasculature.[ncbi.nlm.nih.gov]
Anisocytosis
  • The blood smear showed eryth- rocyte anisocytosis with rare schistocytes. The persistence of anasarca despite normalization of the serum albumin, along with evidence of DIC, suggested a small vessel process. Skin and muscle biopsies were obtained.[myslide.es]
Karyotype Abnormal
  • In addition, cytogenetic analysis of peripheral blood mononuclear cells revealed the presence of circulating malignant cells with karyotype abnormalities similar to those seen in some lymphomas and leukemias.[documents.mx]
Erythrocyte Sedimentation Rate Abnormal
  • Patients fre- quently develop numerous abnormalities, including hy- poalbuminemia, hyponatremia, hypochloremia, elevated erythrocyte sedimentation rate, abnormal liver and renal function test results, and positive serology for rheumatoid factor and antinuclear[documents.mx]

Treatment

  • Although malignant angioendotheliomatosis is usually fatal, early treatment with chemotherapeutic agents is helpful. We review the characteristics and treatment of this uncommon disorder and present an illustrative case.[ncbi.nlm.nih.gov]
  • The treatment is directed towards addressing specific signs and symptoms based on the organs involved The prognosis depends on many factors including the type of lymphoma, progression of the condition, response to treatment, and overall health of the[dovemed.com]

Prognosis

  • In general, the prognosis of Intravascular Large B-Cell Lymphoma of Lung is often poor General information on lymphoma and lymphocytes: Lymphoma is a type of cancer stemming from uncontrollably dividing lymphocytes (type of white blood cells).[dovemed.com]
  • Dementia is the most common neurological symptom that occurs in about half of the patients with central nervous system pathology, and is associated with poorer prognosis.[karger.com]
  • The primary involvement of the skin has been suggested to have a significantly better prognosis overall (for review see (5)).[medicaljournals.se]
  • Reactive angioendotheliomatosis has a good prognosis and usually regresses when the underlying disease, if discovered, is successfully treated. The disease sometimes regresses spontaneously. The prognosis depends on the underlying cause.[emedicine.medscape.com]
  • The authors provide an in-depth description of the clinical and morphologic aspects of each entity and detail their clinicopathologic correlation, differential diagnosis, prognosis, and therapy.[link.springer.com]

Etiology

  • David Da Ines et al: Hepatic capsular retraction: spectrum of diagnosis at MRI: Acta Radiologica Short Reports 3(11): 1-11, 2014 A Blachar et al: Hepatic capsular retraction: spectrum of benign and malignant etiologies: Abdom Imaging 27: 690-699, 2002[trc-rad.jp]
  • The patient reported being told that the etiologies of her anemia and pericarditis were unclear despite extensive testing.[hematologyandoncology.net]
  • Etiology Different triggers (eg, subacute bacterial endocarditis; circulating immune complexes; fibrin; cholesterol emboli; viruses, such as hepatitis C; atriovenous fistula [17] ; atherosclerotic emboli; trauma; metal implants [18] ; drugs such as trabectedin[emedicine.medscape.com]
  • Etiology The etiology of most pcmzl remains unclear.[healthdocbox.com]
  • […] cases found an accumulation of structural aberrations in chromosomes 1, 6, and 18, especially 1p (67% of cases) and trisomy 18 (67% of cases). [ 62 ] A translocation, t(1;3)(p22;p21), has also been reported. [ 63 ] There is little support for a viral etiology[uscapknowledgehub.org]

Epidemiology

  • Generally, AL occurs in adults, but to date, no distinctive epidemiological features have been identified, owing to the small number of cases that have been reported in the literature (3).[path.upmc.edu]
  • Epidemiology Frequency About 100 cases of malignant angioendotheliomatosis and about 40 cases of the reactive form have been described worldwide.[emedicine.medscape.com]
  • Epidemiology Cutaneous MZL affects both men and women, with a median age of 50 years and a range of years. pcmzl shows a predilection for the trunk and upper extremities.[healthdocbox.com]
  • EPIDEMIOLOGY An American epidemiological study published in 2009, based on analysis of 3,884 cases of PCL diagnosed in the United States between 2001 and 2005, revealed that 71% were PCTCL (7.7 cases/ million inhabitants/ year) and 29% were PCBCL (3.1[zdoc.site]
Sex distribution
Age distribution

Pathophysiology

  • In the former, thromboplastins re- leased from intracytoplasmic granules of leukemic cells are thought to trigger the coagulation mechanism.19 In the latter, the pathophysiology is less clear, although the initiation of DIC may be related to the function[myslide.es]
  • Pathophysiology The pathogenesis of reactive angioendotheliomatosis is still not fully elucidated.[emedicine.medscape.com]
  • Although there appears to be a possible association between intravascular lymphoma and nodal NHL, the pathophysiologic relationships are poorly understood.[hematologyandoncology.net]

Prevention

  • […] cancer cells, a process called immune surveillance Please find comprehensive information on Intravascular Large B-Cell Lymphoma of Lung regarding definition, distribution, risk factors, causes, signs & symptoms, diagnosis, complications, treatment, prevention[dovemed.com]
  • The mechanism is different from that of nuclear staining by basic dyes such as thionine or toluidine blue, staining by basic dyes occurs only from solutions that are less acidic than hemalum, and it is prevented by prior chemical or enzymatic extraction[wikivisually.com]
  • Author Robert A Schwartz, MD, MPH Professor and Head of Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, Rutgers New Jersey Medical School; Visiting Professor, Rutgers University School of Public[emedicine.medscape.com]
  • Research output : Contribution to journal › Article Sirolimus prevents short-term renal changes induced by ischemia-reperfusion injury in rats Esposito, C. , Grosjean, F. , Torreggiani, M., Esposito, V., Mangione, F. , Villa, L., Sileno, G., Rosso, R.[moh-it.pure.elsevier.com]
  • Some challenge the notion of IVLBCL concomitantly involving the CNS extravascularly due to the fact that they are thought to have absent surface adhesion molecules CD29, CD54 (ICAM-1), and CD11a, preventing them from being able to cross small CNS vessels[jpatholtm.org]

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