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Eosinophilic granuloma

MedGen UID:
4979
•Concept ID:
C0014461
•
Neoplastic Process
Synonyms: Eosinophilic Granuloma; Eosinophilic Granulomas; Granuloma, Eosinophilic; Granulomas, Eosinophilic
SNOMED CT: Eosinophilic granuloma (128810002); Eosinophilic granuloma (129000002); Eosinophilic xanthomatous granuloma (129000002)
 
HPO: HP:0032253
Monarch Initiative: MONDO:0020517

Definition

A type of granuloma characterized morphologically by the predominance of Langerhans cells with characteristic grooved, folded, indented nuclei in the appropriate milieu that includes variable numbers of eosinophils and histiocytes including multinucleated forms, often appearing similar to osteoclasts or touton like giant cells, neutrophils and small lymphocytes. The concentration of the eosinophilic infiltrate varies from scattered mature cells to sheet-like masses of cells. Occasionally, areas of bone necrosis may interrupt the cellular infiltrate. The foamy cells may also be amassed in clumps, which are of no clinical significance because these clumps represent phagocytosis of lipid debris. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVEosinophilic granuloma
Follow this link to review classifications for Eosinophilic granuloma in Orphanet.

Conditions with this feature

DOCK2 deficiency
MedGen UID:
901370
•Concept ID:
C4225328
•
Disease or Syndrome
Immunodeficiency-40 is an autosomal recessive primary form of combined immunodeficiency mainly affecting T-cell number and function, with other more variable defects in B-cell and NK-cell function. Patients have onset of severe invasive bacterial and viral infections in early childhood and may die without bone marrow transplantation (summary by Dobbs et al., 2015).
See: Condition Record
DOCK2 deficiency

Professional guidelines

PubMed

Current concepts for the diagnosis and management of eosinophilic granuloma of bone.
Angelini A, Mavrogenis AF, Rimondi E, Rossi G, Ruggieri P
J Orthop Traumatol 2017 Jun;18(2):83-90. Epub 2016 Oct 21 doi: 10.1007/s10195-016-0434-7. PMID: 27770337Free PMC Article
Diagnosis and management of langerhans cell histiocytosis.
DiCaprio MR, Roberts TT
J Am Acad Orthop Surg 2014 Oct;22(10):643-52. doi: 10.5435/JAAOS-22-10-643. PMID: 25281259
Langerhans' cell histiocytosis: pathology, imaging and treatment of skeletal involvement.
Azouz EM, Saigal G, Rodriguez MM, Podda A
Pediatr Radiol 2005 Feb;35(2):103-15. Epub 2004 Jul 28 doi: 10.1007/s00247-004-1262-0. PMID: 15289942

Recent clinical studies

Etiology

Langerhans cell histiocytosis. Advances in pathogenesis and clinical practice.
Astigarraga I, García-Obregón S, Pérez-Martínez A, Gutiérrez-Carrasco I, Santa-María V, Iturrate CR, Reggiori ML, Carrillo TM, Torrent M; Grupo de Histiocitosis de la Sociedad Española de Hematología y Oncología Pediátricas (SEHOP)
An Pediatr (Engl Ed) 2022 Aug;97(2):130.e1-130.e7. Epub 2022 Jul 20 doi: 10.1016/j.anpede.2022.05.005. PMID: 35869015
2022 American College of Rheumatology/European Alliance of Associations for Rheumatology Classification Criteria for Eosinophilic Granulomatosis with Polyangiitis.
Grayson PC, Ponte C, Suppiah R, Robson JC, Craven A, Judge A, Khalid S, Hutchings A, Luqmani RA, Watts RA, Merkel PA; DCVAS Study Group
Ann Rheum Dis 2022 Mar;81(3):309-314. Epub 2022 Feb 2 doi: 10.1136/annrheumdis-2021-221794. PMID: 35110334
Evaluation of clinical benefit from treatment with mepolizumab for patients with eosinophilic granulomatosis with polyangiitis.
Steinfeld J, Bradford ES, Brown J, Mallett S, Yancey SW, Akuthota P, Cid MC, Gleich GJ, Jayne D, Khoury P, Langford CA, Merkel PA, Moosig F, Specks U, Weller PF, Wechsler ME
J Allergy Clin Immunol 2019 Jun;143(6):2170-2177. Epub 2018 Dec 19 doi: 10.1016/j.jaci.2018.11.041. PMID: 30578883Free PMC Article
Intracranial Rosai-Dorfman disease.
Huang BY, Zong M, Zong WJ, Sun YH, Zhang H, Zhang HB
J Clin Neurosci 2016 Oct;32:133-6. Epub 2016 Aug 23 doi: 10.1016/j.jocn.2015.12.046. PMID: 27561856
Eosinophilic granuloma.
Duncan WK, Post AC, McCoy BP
Oral Surg Oral Med Oral Pathol 1988 Jun;65(6):736-41. doi: 10.1016/0030-4220(88)90020-5. PMID: 3041337

Diagnosis

Current concepts for the diagnosis and management of eosinophilic granuloma of bone.
Angelini A, Mavrogenis AF, Rimondi E, Rossi G, Ruggieri P
J Orthop Traumatol 2017 Jun;18(2):83-90. Epub 2016 Oct 21 doi: 10.1007/s10195-016-0434-7. PMID: 27770337Free PMC Article
Benign tumors of the spine.
Thakur NA, Daniels AH, Schiller J, Valdes MA, Czerwein JK, Schiller A, Esmende S, Terek RM
J Am Acad Orthop Surg 2012 Nov;20(11):715-24. doi: 10.5435/JAAOS-20-11-715. PMID: 23118137
Eosinophilic granuloma.
Duncan WK, Post AC, McCoy BP
Oral Surg Oral Med Oral Pathol 1988 Jun;65(6):736-41. doi: 10.1016/0030-4220(88)90020-5. PMID: 3041337
Cranial eosinophilic granuloma.
Arseni C, Dănăilă L, Constantinescu A
Neurochirurgia (Stuttg) 1977 Nov;20(6):189-99. doi: 10.1055/s-0028-1090377. PMID: 593478
Eosinophilic granuloma.
Oldham RK
South Med J 1971 Aug;64(8):978-82. doi: 10.1097/00007611-197108000-00016. PMID: 5565339

Therapy

Evaluation of clinical benefit from treatment with mepolizumab for patients with eosinophilic granulomatosis with polyangiitis.
Steinfeld J, Bradford ES, Brown J, Mallett S, Yancey SW, Akuthota P, Cid MC, Gleich GJ, Jayne D, Khoury P, Langford CA, Merkel PA, Moosig F, Specks U, Weller PF, Wechsler ME
J Allergy Clin Immunol 2019 Jun;143(6):2170-2177. Epub 2018 Dec 19 doi: 10.1016/j.jaci.2018.11.041. PMID: 30578883Free PMC Article
All That Wheezes..
Divakaran S, Vaidya A, Kobzik L, Dellaripa P
N Engl J Med 2017 Jul 27;377(4):e6. doi: 10.1056/NEJMimc1613182. PMID: 28745993
Eosinophilic granuloma.
Jordan DR, McDonald H, Noel L, Nizalik E
Arch Ophthalmol 1993 Jan;111(1):134-5. doi: 10.1001/archopht.1993.01090010138040. PMID: 8424711
Eosinophilic granuloma.
Oldham RK
South Med J 1971 Aug;64(8):978-82. doi: 10.1097/00007611-197108000-00016. PMID: 5565339
Histiocytosis.
el-Serafy S, Reda M
J Laryngol Otol 1971 Aug;85(8):857-67. doi: 10.1017/s0022215100074144. PMID: 5565086

Prognosis

Langerhans cell histiocytosis. Advances in pathogenesis and clinical practice.
Astigarraga I, García-Obregón S, Pérez-Martínez A, Gutiérrez-Carrasco I, Santa-María V, Iturrate CR, Reggiori ML, Carrillo TM, Torrent M; Grupo de Histiocitosis de la Sociedad Española de Hematología y Oncología Pediátricas (SEHOP)
An Pediatr (Engl Ed) 2022 Aug;97(2):130.e1-130.e7. Epub 2022 Jul 20 doi: 10.1016/j.anpede.2022.05.005. PMID: 35869015
Benign tumors of the spine.
Thakur NA, Daniels AH, Schiller J, Valdes MA, Czerwein JK, Schiller A, Esmende S, Terek RM
J Am Acad Orthop Surg 2012 Nov;20(11):715-24. doi: 10.5435/JAAOS-20-11-715. PMID: 23118137
Craniopharyngioma.
Garnett MR, Puget S, Grill J, Sainte-Rose C
Orphanet J Rare Dis 2007 Apr 10;2:18. doi: 10.1186/1750-1172-2-18. PMID: 17425791Free PMC Article
Eosinophilic granuloma.
Duncan WK, Post AC, McCoy BP
Oral Surg Oral Med Oral Pathol 1988 Jun;65(6):736-41. doi: 10.1016/0030-4220(88)90020-5. PMID: 3041337
Histiocytosis X.
Favara BE, McCarthy RC, Mierau GW
Hum Pathol 1983 Aug;14(8):663-76. doi: 10.1016/s0046-8177(83)80138-5. PMID: 6347865

Clinical prediction guides

2022 American College of Rheumatology/European Alliance of Associations for Rheumatology Classification Criteria for Eosinophilic Granulomatosis with Polyangiitis.
Grayson PC, Ponte C, Suppiah R, Robson JC, Craven A, Judge A, Khalid S, Hutchings A, Luqmani RA, Watts RA, Merkel PA; DCVAS Study Group
Ann Rheum Dis 2022 Mar;81(3):309-314. Epub 2022 Feb 2 doi: 10.1136/annrheumdis-2021-221794. PMID: 35110334
Acetabular roof lesions in children: a descriptive study and literature review.
Wang J, Wang Z, Qin J
BMC Musculoskelet Disord 2020 Aug 24;21(1):575. doi: 10.1186/s12891-020-03601-x. PMID: 32831075Free PMC Article
Evaluation of clinical benefit from treatment with mepolizumab for patients with eosinophilic granulomatosis with polyangiitis.
Steinfeld J, Bradford ES, Brown J, Mallett S, Yancey SW, Akuthota P, Cid MC, Gleich GJ, Jayne D, Khoury P, Langford CA, Merkel PA, Moosig F, Specks U, Weller PF, Wechsler ME
J Allergy Clin Immunol 2019 Jun;143(6):2170-2177. Epub 2018 Dec 19 doi: 10.1016/j.jaci.2018.11.041. PMID: 30578883Free PMC Article
Pseudotumors and tumors of the temporomandibular joint. A review.
Poveda-Roda R, Bagán JV, Sanchis JM, Margaix M
Med Oral Patol Oral Cir Bucal 2013 May 1;18(3):e392-402. doi: 10.4317/medoral.18799. PMID: 23385515Free PMC Article
Benign tumors of the spine.
Thakur NA, Daniels AH, Schiller J, Valdes MA, Czerwein JK, Schiller A, Esmende S, Terek RM
J Am Acad Orthop Surg 2012 Nov;20(11):715-24. doi: 10.5435/JAAOS-20-11-715. PMID: 23118137

Recent systematic reviews

Otologic Manifestations of Langerhans Cell Histiocytosis: A Systematic Review.
Chen T, Ashman PE, Bojrab DI 2nd, Johnson AP, Benson B, Hong RS, Svider PF
Otolaryngol Head Neck Surg 2022 Jan;166(1):48-59. Epub 2021 May 4 doi: 10.1177/01945998211004590. PMID: 33945752
Role of Stereotactic Radiosurgery in Intracranial Histiocytosis: a Systematic Review of Literature of an Emerging Modality for Localized Disease.
Tripathi M, Maskara P, Deora H, Bansal D, Mohindra S, Tripathi S, Kaur R, Sheehan JP, Rana R, Kumar N
World Neurosurg 2021 Jun;150:64-70. Epub 2021 Mar 17 doi: 10.1016/j.wneu.2021.03.047. PMID: 33744421
Unisystem Langerhans cell histiocytosis in maxillofacial region in pediatrics: comprehensive and systematic review.
Chugh A, Kaur A, Kumar Patnana A, Kumar P, Chugh VK
Oral Maxillofac Surg 2021 Dec;25(4):429-444. Epub 2021 Feb 16 doi: 10.1007/s10006-021-00949-9. PMID: 33591444

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