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2.1
Proliferating Angioendotheliomatosis
Prolif Angioendotheliomatosis

Presentation

In conclusion, DDA is a rare disease, however, it may present as an unbearable pain in the breast. [gs.amegroups.com]

Histological and immunohistological investigations of the malignant form of AEPS are presented: In a 69-year-old woman cutaneous lesions appeared 5 months before the diagnosis of B-immunoblastic lymphoma. [unboundmedicine.com]

Through comparison of the frequency with which key clinical and histologic features present in cases of each group, the authors provide improved clarity of the similarities and differences between these 3 entities. [pesquisa.bvsalud.org]

A literature review of the disease, including its controversial histiogenesis, treatment, and survival, is presented. In view of the rarity of the disease, only by studying more patients can we better understand the disease. [ncbi.nlm.nih.gov]

Clinical Presentation: Presents as erythematous macules, papules and plaques. Necrosis and ulceration may sometimes develop. The lesions measure from 1 to 3 cm or more in diameter. Site: The lesion may occur at any body site. [histopathology-india.net]

Entire Body System

  • Anemia

    貧血 anemia of chronic disease or disorders (ACD)慢性炎症性疾患に伴う貧血 anticoagulant抗凝血素 antigen presenting cells抗原提示細胞 antiphospholipid syndrome (APS)抗リン脂質抗体症候群 antithrombotic drug抗血栓薬 aplastic anemia (AA)再生不良性貧血 apoptosis アポトーシス autoimmune hemolytic anemia溶血性貧血 [tokyo-med.ac.jp]

    The main adverse effects of ruxolitinib are anemia and thrombocytopenia. [merckmanuals.com]

    Originele protocol Inclusion criteria Age 60 years or more Subjects with a cytopathologically confirmed diagnosis of (a) AML (M0-M2 and M4-M7, FAB classification, appendix A), or (b) refractory anemia with excess of blasts (RAEB) or refractory anemia [vademecumhematologie.nl]

    Diagnosis and treatment of cold agglutinin mediated autoimmune hemolytic anemia. Blood Rev 2012;26:107-15. 2. Porras-Luque JI, Fernandez-Herrera J, Dauden E, Fraga J, Fernandez-Villalta MJ, Garcia-Diez A. [ijdvl.com]

    Cooley’s anemia (thalassemia major), cryofibrinogenemia, cryoglobulinemia, macroglobulinemia, polycythemia vera, sickle cell anemia, thrombocytopenic purpura Henoch-Schönlein purpura antiphospholipid syndrome, Behçet’s disease, Berger d. [dermadvocate.net]

  • Lymphedema

    Angiosarcoma arising in chronic lymphedema (syndrome of Stewart and Treves) may be histologically indistinguishable from Kaposi's sarcoma, but may also present with large pleo-morphic cells occluding the vessel lumina and resembling the cases described [docslide.net]

    Epithelioid hemangioendothelioma Angiosarcoma Idiopathic (head and neck) Associated with chronic lymphedema (Stewart-Treves) Postirradiation Epithelioid 6. [slideshare.net]

    Keywords: Lymphedema; Synovitis; Aspiration 1. Introduction Various dermatoses are associated with rheumatoid arthritis (RA), including rheumatoid nodules, rheumatoid vasculitis, and neutrophilic dermatoses. [file.scirp.org]

    […] homozygous familial hypercholesterolemia, hyperapobetalipoproteinemia, myxedema, obstructive liver d. xanthoma, tuberous hyperlipoproteinemia type II & III xanthomas atherosclerosis, biliary disease, CTCL, diabetes mellitus, hypothyroidism, leukemia, lymphedema [dermadvocate.net]

    The diagnosis is massive localized lymphedema of the morbidly obese (and not liposarcoma). FIgure 4a. [orthopaedicsone.com]

  • Short Stature

    stature neurofibromatosis type II central neurofibromatosis; bilateral acoustic neuromas, meningioma, posterior subscapular cataracts, spinal glioma, unilateral schwanomma neuroma, acousticMEN type II, NF II neuroma, mucosal Sipple’s s. neutrophilic [dermadvocate.net]

    […] fingers (13), polydactyly (32), acral cyanosis (13), simian lines on the palms (14), anal atresia (16,36), rectovaginal and urethrovaginal fistulas (36), absent clitoris (36), hypospadias (37), short stature (13), diffuse demineralization of bones (14 [vdoc.pub]

  • Constitutional Symptom

    There were no constitutional symptoms, her past medical history was unremarkable, and she was not taking any oral medications. [medicaljournals.se]

    There was no lymphadenopathy, no gross pathology of other visceral organs, and no constitutional symptoms to suggest primary cancer or metastases. [applied-radiology.vmhost1.publishwithagility.com]

Liver, Gall & Pancreas

  • Hepatomegaly

    Hepatomegaly occurs in some patients. Lymphadenopathy is rare. Severe extramedullary hematopoiesis can disturb the function of organs in which it occurs, including the brain. [merckmanuals.com]

    There were no other dermatological findings.No lymphadenopathy, hepatomegaly or splenomegalywere present. His past medical history included lungtuberculosis, heavy smoking, chronic bronchitis andpartial respiratory insufficiency. [docslide.com.br]

    However, patients can present with numerous other signs and symptoms, including fever, hepatomegaly, urinary obstruction, renal failure, adrenomegaly and dyspnea. [nature.com]

  • Jaundice

    Less commonly patients presented with dyspnea, anemia, thrombocytopenia, fever, jaundice, hepatomegaly, multiorgan failure and urinary obstruction. Eleven patients were diagnosed in life and seven were diagnosed at autopsy. [nature.com]

    […] porokeratosis of Mibelli Bazex’s s. cancer of upper respiratory system; tongue, pharynx, esophagus, lung Becker’s nevus developmental defects (spina bifida, breast hypoplasia, limb asymmetry), smooth muscle hamartoma biliary cirrhosis hyperpigmentation, jaundice [dermadvocate.net]

Cardiovascular

  • Vascular Disease

    In the case reported, there are three distinctive features which imply that this rare syndrome should be recognized as a pattern of vascular disease. (1) There was focal proliferation of cells, similar to neoplastic reticular cells, associated with the [academic.naver.com]

    Who is at Risk for Developing this Disease? The disease is rare but can occur in any age group and either gender. It is almost always associated with underlying vascular occlusion. The source of the occlusion may be localized or systemic. [dermatologyadvisor.com]

    Illustrated here from a biopsy of a patient with an unclassified collagen vascular disease presenting with nodules and livedo reticularis is a muscular vessel (artery) whose endothelium is disrupted by lymphocytes (endothelialitis) and media and adventia [vgrd.org]

    Its basis is usually atherosclerotic vascular disease, cholesterol emboli, mural thrombi and rarely, fragmentation of intracardiac tumors. [docslide.us]

  • Heart Disease

    or any other condition that does not qualify within the generally accepted medical definition of Ischemic heart disease. [veterans.perkinslawtalk.com]

    (Predisposing Factors) The following conditions are known to be associated with Reactive Angioendotheliomatosis: Heart diseases involving the valves Sarcoidosis Antiphospholipid syndrome Polymyalgia rheumatic Liver cirrhosis or liver disease Cryoglobulinemia [dovemed.com]

Skin

  • Angiomatosis

    “Diffuse dermal angiomatosis”. J Cutan Pathol. vol. 33. 2006 Sep. pp. 646-8. (This is another report of diffuse dermal angiomatosis secondary to peripheral atherosclerosis. [dermatologyadvisor.com]

    Figure 1 Left breast of a patient with diffuse dermal angiomatosis. [gs.amegroups.com]

    Diffuse dermal angiomatosis: a variant of reactive cutaneous angioendotheliomatosis. J Cutan Pathol. 1994 Aug. 21(4):363-70. [Medline]. Yang H, Ahmed I, Mathew V, Schroeter AL. Diffuse dermal angiomatosis of the breast. [emedicine.medscape.com]

    1 Diffuse dermal angiomatosis mimicking inflammatory breast carcinoma. 61 28744985 2018 2 Resolution of reactive angioendotheliomatosis in an arteriovenous fistula with innominate vein angioplasty. 61 Vacharathit V...Kirksey L 29192720 2018 3 IMAGING [malacards.org]

  • Cutaneous Manifestation

    [Systemic proliferative angioendotheliomatosis: a cutaneous manifestation of malignant B-cell lymphomas. Histologic and immunohistologic studies of two cases]. [unboundmedicine.com]

    Seminars in Diagnostic Pathology 4(1): 18-27, 1987 Angioendotheliomatosis proliferans systemisata a cutaneous manifestation of malignant b cell lymphoma. Hautarzt 42(6): 384-390, 1991 Malignant systemic angioendotheliomatosis. [eurekamag.com]

    manifestation of Hughes syndrome. 61 Nikam B...Jamale V 26231975 2015 7 Recurrent reactive angioendotheliomatosis in pregnancy: a case report. 61 Bin Saif GA...Sangueza OP 26228728 2015 8 Intravascular/Intralymphatic Histiocytosis: A Report of 3 Cases [malacards.org]

    Reactive angioendotheliomatosis as a presenting cutaneous manifestation of Hughes syndrome. Lupus. 2015 Dec. 24 (14):1557-61. [Medline]. Lomater C, Gerloni V, Gattinara M, Mazzotti J, Cimaz R, Fantini F. [emedicine.medscape.com]

  • Dermatitis

    2017 DERMATOLOGICAL PHARMACEUTICAL COMPOSITION FOR THE TREATMENT OF SKIN INFLAMMATION DISEASES, SUCH AS DERMATITIS, ATOPIC DERMATITIS, VITILIGO, ALOPECIA AREATA, ACNE, PSORIASIS … I Umbert Millet EP Patent EP2,311,454, 2015 2015 [scholar.google.com]

    lichen planus, seborrheic dermatitis, stasis dermatitis, tinea corporis, xerosis lichen spinulosis arsphenamine, gold, HIV, Hodgkin’s d., lichen scrofulosorum, mycotic id reactions, seborrheic dermatitis, syphilis, thallium linear IgA disease cancer [dermadvocate.net]

    Annales de Dermatologie et de Syphiligraphie 99(2): 121-133, 1972 Infantile gluteal granuloma tappeiner and pfleger should it be considered contact dermatitis vegetans or infant halogenosis. [eurekamag.com]

    In the rare cases of implant-associated dermatitis without signs of infection (e.g., fever and elevated CRP levels or ESR), a hypersensitivity reaction should be considered. [quiz.jbjs.org]

Workup

A thorough diagnostic workup and subsequent 3-year clinical follow-up revealed no sign of an underlying infectious or neoplastic disorder. [karger.com]

The usual workup utilized brain CT which is inadequate tool to evaluate nonhemorrhagic brain lesions. 3. Conclusion Intravascular lymphoma is a rare and fatal disease that presents with a myriad of nonspecific symptoms. [hindawi.com]

Treatment

CO_2激光治疗组115 例; 短句来源 更多 Treatment of the proliferative hemangiomas 增殖性血管瘤治疗方法的选择 短句来源 Treatment Methods of Infantile Body Surface Proliferative Hemangiomas 婴幼儿体表增殖性血管瘤治疗方法的探讨 短句来源 Proliferative lupus vulgaris 增殖性寻常狼疮 短句来源 Clinical analysis of treatment [dict.cnki.net]

[…] a malignant type – Intravascular large B-cell lymphoma Treatment[edit] This section is empty. [en.wikipedia.org]

In a few cases treatment with palliative chemotherapy has been effective. [wiki30.com]

A literature review of the disease, including its controversial histiogenesis, treatment, and survival, is presented. In view of the rarity of the disease, only by studying more patients can we better understand the disease. [ncbi.nlm.nih.gov]

Prognosis

The authors describe in-depth the clinical and morphologic aspects of each entity and detail their clinicopathologic correlation, differential diagnosis, prognosis, and therapy. [books.google.com]

(Outcomes/Resolutions) The prognosis of Reactive Angioendotheliomatosis is linked to the prognosis of the underlying condition it is associated with. [dovemed.com]

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]

Prognosis Extremely poor; short survival is the norm despite aggressive chemotherapy. [medical-dictionary.thefreedictionary.com]

Etiology

A brain magnetic resonance imaging (MRI) was completed to exclude a cerebrovascular etiology of her acute symptoms. The MRI was reported as abnormal due to the presence of “white matter disease”. [hindawi.com]

Because the patient had no laboratory signs of infection, another etiology was considered. [quiz.jbjs.org]

Classification of these conditions often presents many nosological challenges, since underlying etiologies and pathogenetics are often not known. [doctors-hospitals-medical-cape-town-south-africa.blaauwberg.net]

Patients with SVCS will benefit from our specialty having an attentive and current awareness of the disease pathogenesis, since the radiologist is in the unique position of being able to identify etiology early in the course of disease, allowing for prompt [applied-radiology.vmhost1.publishwithagility.com]

(Etiology) The exact cause of Reactive Angioendotheliomatosis is unknown They are thought to occur as a reactive process to an existing underlying health condition. [dovemed.com]

Epidemiology

Definition / general Intravascular proliferation of endothelial cells Epidemiology Typically seen in skin; extremely rare in colon, with only one case reported Clinical features Only reported patient experienced massive gastrointestinal bleeding Gross [pathologyoutlines.com]

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]

Society ERS European Respiratory Society ESTS European Society of Thoracic Surgeons IASLC International Association for the Study of Lung Cancer IMIG International Mesothelioma Interest Group NCCN National Comprehensive Cancer Network SEER Surveillance, Epidemiology [haigan.gr.jp]

Epidemiology Frequency About 100 cases of malignant angioendotheliomatosis and about 40 cases of the reactive form have been described worldwide. [emedicine.medscape.com]

Pathophysiology

Pathophysiology The pathophysiology is not fully understood, but occlusion of blood vessel lumens appears to be common to all variants of the disease. [dermatologyadvisor.com]

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

Current medical research have not established a method of preventing Reactive Angioendotheliomatosis Regular medical screening at periodic intervals with blood tests, scans, and physical examinations, may be advised for those who have this condition What [dovemed.com]

PRISMS (Prevention of Relapses and Disability by Interferon b-1a Subcutaneously in Multiple Sclerosis). Randomised double-blind placebo-controlled study of interferon beta-1a in relapsing/remitting multiple sclerosis. [ge-bu.nl]

All female patients who can become pregnant must be enrolled in the iPLEDGE program, which is designed to prevent pregnancy while using this medication. [gs.amegroups.com]

The service-connected disabilities must be sufficient to prevent the veteran from performing the mental or physical tasks required to obtain or keep employment. [veterans.perkinslawtalk.com]

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