Эозинофильная гранулема
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Эозинофильная гранулема человека характеризуется аномальной пролиферацией клеток Лангерганса (LCS). LCS представляют собой антиген-презентативные клетки, полученные из дендритных клеток . У людей эозинофильные гранулемы рассматриваются как доброкачественные опухоли, которые встречаются в основном у детей и подростков. Например, довольно редкое состояние, и его частота выше в белом, чем в черном популяции, также немного больше влияет на мужчин, чем женщины. Например, развивается у 4-5 детей (в возрасте до 15 лет) на миллион/год и у 1 или 2 взрослых на миллион/год. [ 1 ]
Этиология, например, еще до конца не изучена. Тем не менее, начало аномальной пролиферации LC может быть вызвано вирусными стимулами ( EBV , вирусом герпеса человека ), бактериальными токсинами или дефектной регуляцией продукции IL-1 и IL-10 . [ 2 ] Другим возможным объяснением может быть дефект в сигнальном пути RAS/MAPK из -за мутации сигнальных белков. В частности, было опубликовано, что около 50% случаев EG имели мутированный BRAF V600 E ген [ 3 ] и около 21% показали мутация в MAP2K 1. [ 4 ]
Signs and symptoms
[edit]Clinically, single or multiple lesions are present mostly on the axial skeleton - on the skull, spine, ribs, pelvis and long bones. Specifically, thoracic spine and frontal bone within the skull are the most affected in children. On the other hand, cervical spine and jaw bone are the major spots of EG lesions in adults. Symptoms of EG include stiffness, local pain, edema of surrounding tissues, posture change, and many others, depending on the affected bone.[1]
Lung EG is even less common. Its prevalence has not been published, however, lung EG was already diagnosed in approximately 5% of all lung biotic samples. Such condition may be asymptomatic, but also a non-productive cough, dyspnea, chest pain, fatigue, and spontaneous pneumothorax can occur.[1] Higher risk of lung EG is being associated with smoking.[5]
Diagnosis
[edit]Diagnostic options of EG comprise X-ray, CT and MR. Histopathological examination of the bioptic sample is focused on the presence of mononuclear LCs with prominent nuclear grooves (coffee bean shaped nuclei) with addition of eosinophils. Such examination also utilizes CD1 and CD207 (Langerin) staining.[6] Electron microscopy examination of the sample is based on detection of Birbeck granules, specific "tennis racquet" shaped inclusion within cytoplasm of LCs.[1]
Treatment
[edit]Single lesions spontaneously regress often, especially in children, thus are followed up in regular intervals. Patients with spinal lesions are immobilized to minimize the pain. More complicated cases with multiple lesions should be treated with corticosteroids, particularly with an intralesional injection of methylprednisolon. Corticosteroids also represent an option for pulmonary EG treatment.[1] Operative treatment includes bone grafting and surgical fixation. In advanced stages of the disease, lung transplantation may be required. High-risk patients with multiple lesions (CNS-risk bones, lungs) may undergo chemotherapy.[7]
Cats and Dogs
[edit]
Feline EG
[edit]Feline eosinophilic granuloma complex (EGC) is relatively common condition, characterized by number of patterns affecting oral cavity, skin and mucocutaneous junctions of cats. EGC can be triggered by various stimuli, however, the feline allergic disease is the most relevant one. Among the others, insect bites (fleas), fungi infections and viruses (Feline leukemia virus, feline immunodeficiency virus) may be responsible.[8]
Three primary clinical lesions of EGC include indolent (also referred to as eosinophilic or rodent) ulcer, eosinophilic plaque and eosinophilic granuloma.[9]
Signs and symptoms
[edit]The lesions mostly appear on the upper lip as ulcers, but they also develop on the gums and palate. Other locations like foot pads are not exceptional and are typical for developing plaques instead of ulcers.[9] Such plaques may be itchy and swelling. In the case of oral ulceration, bleeding, pain and loss of appetite may be present.[10]
Treatment
[edit]The treatment of the EGC generally consists of hypoallergenic diet combined with insect bite prevention. Corticosteroids are administered either orally or by long-acting intralesional injections. Alternatively, cyclosporine provides similar effect.[10] Commonly used drugs include: trimethoprim/sulfadiazine, cephalexin, amoxicillin, trihydrate-clavulanate, clindamycin, methylprednisolone, dexamethasone, triamcinolone, and fluocinolone. Megestrol acetate may be used but due to side effects it's only recommended for severe and reoccurring cases.[11]
Canine EG
[edit]Canine eosinophilic granuloma (CEG) is extremely rare autoinflammatory state affecting primarily oral cavity and surrounding areas of transition between mucosa and hairy skin. However, there has also been described forms affecting lungs or digits.[12] It may appear independently on the breed or age, although, particular breeds are more prone to the disease, such German shepherds, Labrador retrievers, Huskies and Cavalier King Charles spaniels. The disease also tends to appear mainly in dogs aged under 3 years.[13]
Eosinophilic granuloma lesions in dogs primarily consists of eosinophils with addition of various cell subtypes such macrophages, neutrophils, plasmocytes, lymphocytes, mast cells and many others.[13]
Signs and symptoms
[edit]CEG usually manifests as a plaque on the palate, gums, lips or tongue. These are proliferative white or reddish lesions that may be painful and can be the cause of halitosis and hemorrhage that are often present. Loss of appetite is the most obvious symptom indicating the illness. The lesions may spontaneously appear and wane.[14] Biopsy is usually required for confirmation of the diagnosis.
Treatment
[edit]Although the etiology of the disease is not understood completely, it is believed that the most relevant triggers of the autoimmune reaction are various environmental stimuli, especially food allergens and insect bites. Thus, the treatment usually includes transition to hypoallergenic diet and protection from ectoparasites. Medication includes immunosuppressives (prednisone, azathioprine) either alone, or in combination with antibiotics or other drugs (cytostatics - chlorambucil). Remission of the lesions may occur in some cases, but in the others low doses of life-long immunosuppression may be necessary.[13] Recent publications mention electrochemotherapy as a novel therapeutical method of CEG providing selective disappearance of the granuloma mass.[14]
References
[edit]- ^ Jump up to: a b c d e Jha, Suman K.; De Jesus, Orlando (2022), "Eosinophilic Granuloma", StatPearls, Treasure Island (FL): StatPearls Publishing, PMID 32644464, retrieved 2022-01-31
- ^ Chadha, Manish; Agarwal, Anil; Agarwal, Nidhi; Singh, Manoj Kumar (June 2007). "Solitary eosinophilic granuloma of the radius. An unusual differential diagnosis". Acta Orthopaedica Belgica. 73 (3): 413–417. ISSN 0001-6462. PMID 17715738.
- ^ Badalian-Very, Gayane; Vergilio, Jo-Anne; Degar, Barbara A.; MacConaill, Laura E.; Brandner, Barbara; Calicchio, Monica L.; Kuo, Frank C.; Ligon, Azra H.; Stevenson, Kristen E.; Kehoe, Sarah M.; Garraway, Levi A. (2010-09-16). "Recurrent BRAF mutations in Langerhans cell histiocytosis". Blood. 116 (11): 1919–1923. doi:10.1182/blood-2010-04-279083. ISSN 1528-0020. PMC 3173987. PMID 20519626.
- ^ Tran, Gary; Huynh, Thy N.; Paller, Amy S. (March 2018). "Langerhans cell histiocytosis: A neoplastic disorder driven by Ras-ERK pathway mutations". Journal of the American Academy of Dermatology. 78 (3): 579–590.e4. doi:10.1016/j.jaad.2017.09.022. ISSN 1097-6787. PMID 29107340. S2CID 46767736.
- ^ Ma, Jennifer; Laird, James H.; Chau, Karen W.; Chelius, Monica R.; Lok, Benjamin H.; Yahalom, Joachim (January 2019). "Langerhans cell histiocytosis in adults is associated with a high prevalence of hematologic and solid malignancies". Cancer Medicine. 8 (1): 58–66. doi:10.1002/cam4.1844. ISSN 2045-7634. PMC 6346231. PMID 30597769.
- ^ Kumar, Neeta; Sayed, Shahin; Vinayak, Sudhir (2011-01-20). "Diagnosis of Langerhans cell histiocytosis on fine needle aspiration cytology: a case report and review of the cytology literature". Pathology Research International. 2011: 439518. doi:10.4061/2011/439518. ISSN 2042-003X. PMC 3034992. PMID 21331166.
- ^ Kobayashi, Masayuki; Tojo, Arinobu (December 2018). "Langerhans cell histiocytosis in adults: Advances in pathophysiology and treatment". Cancer Science. 109 (12): 3707–3713. doi:10.1111/cas.13817. ISSN 1349-7006. PMC 6272080. PMID 30281871.
- ^ Muse, Russell; Williamson, Nicola (2008-01-01), Morgan, Rhea V. (ed.), "CHAPTER 91 - Erosive and Ulcerative Diseases of the Skin", Handbook of Small Animal Practice (Fifth Edition), Saint Louis: W.B. Saunders, pp. 885–900, ISBN 978-1-4160-3949-5, retrieved 2022-01-31
- ^ Jump up to: a b Hopke, Kaitlin P; Sargent, Sandra J (2019-12-16). "Novel presentation of eosinophilic granuloma complex in a cat". JFMS Open Reports. 5 (2): 2055116919891548. doi:10.1177/2055116919891548. ISSN 2055-1169. PMC 6918502. PMID 31897305.
- ^ Jump up to: a b "Feline Eosinophilic Granuloma Complex In Cats | VCA Animal Hospitals". vcahospitals.com. Retrieved 2022-01-31.
- ^ Rhodes, Karen Helton; Werner, Alexander H. (2011-01-25). Blackwell's Five-Minute Veterinary Consult Clinical Companion. Ames, Iowa: Wiley-Blackwell. p. 123. ISBN 978-0-8138-1596-1.
- ^ Abbott, Dawn E. E.; Allen, Andrew L. (March 2020). "Canine eosinophilic pulmonary granulomatosis: case report and literature review". Journal of Veterinary Diagnostic Investigation. 32 (2): 329–335. doi:10.1177/1040638720907659. ISSN 1040-6387. PMC 7081515. PMID 32180541.
- ^ Jump up to: а беременный в Мендельсон, Даниэль; Льюис, Джон Р.; Скотт, Кристин Иглесиас; Браун, Дороти С.; Рейтер, Александр М. (март 2019 г.). «Клиникопатологические особенности, факторы риска и предрасположенность, а также реакция на лечение эозинофильного заболевания полости рта у 24 собак (2000-2016)» . Журнал ветеринарной стоматологии . 36 (1): 25–31. doi : 10.1177/0898756419834785 . ISSN 0898-7564 . PMID 31138045 . S2CID 169032822 .
- ^ Jump up to: а беременный Tellado, Matías Nicolás; Мичински, Себастьян Диего; Олайз, Нахуэль; Маглиетти, Фелипе; Маршалл, Гильермо (2014-10-16). «Клык пероральная эозинофильная гранулема, обработанная электрохимиотерапией» . Отчеты о случаях в ветеринарной медицине . 2014 : E519197. Doi : 10.1155/2014/519197 . HDL : 11336/19287 . ISSN 2090-7001 .