67:
43:
410:
Erdheim–Chester disease was previously associated with high mortality rates. However, long-term survival is now more promising. Recent studies have reported that some patients receiving targeted therapies showed no disease progression. Targeted therapies using BRAF, MEK and/or other inhibitors have
415:
published guidelines for the diagnosis and treatment of the disease, stressing the importance of genetic testing: "Recent insights into their genomic architecture demonstrating mitogen-activated protein kinase/extracellular signal-regulated kinase pathway mutations have now enabled potential
243:
and histology are the main diagnostic features. Diagnosis can often be difficult because of the rareness of ECD as well as the need to differentiate it from LCH. A diagnosis from neurological imaging may not be definitive. The presence of symmetrical
1751:
Lachenal F, Cotton F, Desmurs-Clavel H, Haroche J, Taillia H, Magy N, et al. (October 2006). "Neurological manifestations and neuroradiological presentation of
Erdheim-Chester disease: report of 6 cases and systematic review of the literature".
142:
Long bone involvement is almost universal in ECD patients and is bilateral and symmetrical in nature. More than 50% of cases have some sort of extraskeletal involvement. This can include kidney, skin, brain and lung involvement, and less frequently
1789:
Mossetti G, Rendina D, Numis FG, Somma P, Postiglione L, Nunziata V (2003). "Biochemical markers of bone turnover, serum levels of interleukin-6/interleukin-6 soluble receptor and bisphosphonate treatment in
Erdheim-Chester disease".
1519:
de Abreu MR, Castro MO, Chung C, Trudell D, Biswal S, Wesselly M, Resnick D (2009). "Erdheim-Chester disease: case report with unique postmortem magnetic resonance imaging, high-resolution radiography, and pathologic correlation".
1651:
Haroche J, Amoura Z, Trad SG, Wechsler B, Cluzel P, Grenier PA, Piette JC (October 2006). "Variability in the efficacy of interferon-alpha in
Erdheim-Chester disease by patient and site of involvement: results in eight patients".
1578:
Haroche J, Amoura Z, Dion E, Wechsler B, Costedoat-Chalumeau N, Cacoub P, et al. (November 2004). "Cardiovascular involvement, an overlooked feature of
Erdheim-Chester disease: report of 6 new cases and a literature review".
1089:"The Mayo Clinic Histiocytosis Working Group Consensus Statement for the Diagnosis and Evaluation of Adult Patients With Histiocytic Neoplasms: Erdheim-Chester Disease, Langerhans Cell Histiocytosis, and Rosai-Dorfman Disease"
1890:
1875:
1549:
Drier A, Haroche J, Savatovsky J, Godenèche G, Dormont D, Chiras J, et al. (May 2010). "Cerebral, facial, and orbital involvement in
Erdheim-Chester disease: CT and MR imaging findings".
1811:
Perlat A, Decaux O, Sébillot M, Grosbois B, Desfourneaux V, Meadeb J (May 2009). "Erdheim-Chester disease with predominant mesenteric localization: lack of efficacy of interferon alpha".
1618:"Images in cardiovascular medicine. Cardiac involvement in Erdheim-Chester disease: magnetic resonance and computed tomographic scan imaging in a monocentric series of 37 patients"
433:
1482:
Brun AL, Touitou-Gottenberg D, Haroche J, Toledano D, Cluzel P, Beigelman-Aubry C, et al. (November 2010). "Erdheim-Chester disease: CT findings of thoracic involvement".
154:
Bone pain is the most frequent of all symptoms associated with ECD and mainly affects the lower limbs, knees and ankles. The pain is often described as mild but permanent, and
577:
Lutz SZ, Schmalzing M, Vogel-Claussen J, Adam P, May AE (September 2011). "" [Recurrent pericardial effusion as first manifestation of
Erdheim-Chester disease].
1418:
Boissel N, Wechsler B, Leblond V (November 2001). "Treatment of refractory
Erdheim-Chester disease with double autologous hematopoietic stem-cell transplantation".
828:"Dramatic efficacy of vemurafenib in both multisystemic and refractory Erdheim-Chester disease and Langerhans cell histiocytosis harboring the BRAF V600E mutation"
707:
Weidauer S, von
Stuckrad-Barre S, Dettmann E, Zanella FE, Lanfermann H (April 2003). "Cerebral Erdheim-Chester disease: case report and review of the literature".
867:
1350:"18F-fluorodeoxyglucose-positron emission tomography scanning is more useful in followup than in the initial assessment of patients with Erdheim-Chester disease"
162:
occurs in some patients and is usually bilateral, symmetric and painless, and in most cases it occurs several years before the final diagnosis. Recurrent
1063:
753:
2075:
2001:
346:, an oral agent approved in 2019, targets the BRAF protein. It was approved after showing dramatic efficacy in ECD patients harboring the
503:
424:
Approximately 500 cases had been reported in the literature as of 2014. ECD affects predominantly adults, with a mean age of 53 years.
1991:
315:
is said to offer the greatest likelihood of reaching a diagnosis. It would appear that approximately half these patients harbor point
2185:
260:. ECD is not a common cause of exophthalmos but can be diagnosed by biopsy. However, like all biopsies, this may be inconclusive.
110:
in 2016. Onset typically is in middle age, although younger patients have been documented. The disease involves an infiltration of
169:
A review of 59 case studies by
Veyssier-Belot et al. in 1996 reported the following symptoms in order of frequency of occurrence:
1955:
1385:"Pulmonary involvement in Erdheim-Chester disease: a single-center study of thirty-four patients and a review of the literature"
2123:
1852:
261:
1236:
776:"Erdheim-Chester disease: The role of video-assisted thoracoscopic surgery in diagnosing and treating cardiac involvement"
464:
of and promoting research into ECD. ECD families and patients are also supported by the
Histiocytosis Association, Inc.
2180:
2084:
2030:
2128:
2065:
2055:
2012:
2094:
886:
1313:
Aouba A, Georgin-Lavialle S, Pagnoux C, Martin Silva N, Renand A, Galateau-Salle F, et al. (November 2010).
264:
may be used for diagnostic confirmation and also for therapeutic relief of recurrent pericardial fluid drainage.
385:, a drug that targets a mutation in the CSF1R pathway and has shown sustained, complete response in limited use.
2050:
1973:
1449:"Successful treatment of Erdheim-Chester disease, a non-Langerhans-cell histiocytosis, with interferon-alpha"
273:
1683:"Imatinib mesylate for platelet-derived growth factor receptor-beta-positive Erdheim-Chester histiocytosis"
2175:
1948:
614:"Bilateral adrenal infiltration in Erdheim-Chester disease. Report of seven cases and literature review"
915:"Sustained, complete response to pexidartinib in a patient with CSF1R-mutated Erdheim-Chester disease"
2144:
1986:
658:
Veyssier-Belot C, Cacoub P, Caparros-Lefebvre D, Wechsler J, Brun B, Remy M, et al. (May 1996).
107:
66:
2040:
2025:
1905:
826:
Haroche J, Cohen-Aubart F, Emile JF, Arnaud L, Maksud P, Charlotte F, et al. (February 2013).
304:
178:
1383:
Arnaud L, Pierre I, Beigelman-Aubry C, Capron F, Brun AL, Rigolet A, et al. (November 2010).
1260:
2118:
2105:
1616:
Haroche J, Cluzel P, Toledano D, Montalescot G, Touitou D, Grenier PA, et al. (June 2009).
2170:
2165:
2045:
1996:
1941:
1681:
Haroche J, Amoura Z, Charlotte F, Salvatierra J, Wechsler B, Graux C, et al. (June 2008).
316:
257:
202:
1136:
Haroche J, Arnaud L, Cohen-Aubart F, Hervier B, Charlotte F, Emile JF, Amoura Z (April 2014).
2080:
2060:
913:
Abeykoon JP, Lasho TL, Dasari S, Rech KL, Ranatunga WK, Manske MK, et al. (March 2022).
461:
1431:
2088:
1978:
163:
127:
962:
Myra C, Sloper L, Tighe PJ, McIntosh RS, Stevens SE, Gregson RH, et al. (June 2004).
612:
Haroche J, Amoura Z, Touraine P, Seilhean D, Graef C, Birmelé B, et al. (June 2007).
8:
2113:
2017:
1894:
1348:
Arnaud L, Malek Z, Archambaud F, Kas A, Toledano D, Drier A, et al. (October 2009).
288:
1087:
Goyal G, Young JR, Koster MJ, Tobin WO, Vassallo R, Ryu JH, et al. (October 2019).
1777:
1604:
1507:
1284:
1194:
1118:
1039:
1012:
1011:
Aziz SN, Proano L, Cruz C, Tenemaza MG, Monteros G, Hassen G, et al. (June 2022).
988:
963:
939:
914:
800:
775:
732:
689:
253:
183:
166:
can be a manifestation, as can morphological changes in adrenal size and infiltration.
1899:
1634:
1617:
1592:
1916:
1828:
1799:
1769:
1739:
1704:
1669:
1639:
1596:
1566:
1537:
1499:
1470:
1435:
1406:
1371:
1336:
1159:
1122:
1110:
1044:
993:
944:
849:
805:
724:
681:
676:
659:
635:
594:
474:
388:
55:
1781:
1198:
1179:
Virchows Archiv für Pathologische Anatomie und Physiologie und für Klinische Medizin
693:
2070:
1820:
1761:
1729:
1694:
1661:
1629:
1608:
1588:
1558:
1533:
1529:
1511:
1491:
1460:
1427:
1396:
1361:
1326:
1186:
1149:
1100:
1034:
1024:
983:
975:
934:
926:
839:
795:
787:
736:
716:
671:
625:
586:
296:
88:
1716:
Janku F, Amin HM, Yang D, Garrido-Laguna I, Trent JC, Kurzrock R (November 2010).
1910:
1824:
1699:
1682:
1331:
1314:
1105:
1088:
844:
827:
527:
482:", the final diagnosis of a 6-year-old boy who presents with bloody diarrhea and
223:
148:
144:
100:
791:
307:
infiltration by lipid-laden or foamy histiocytes, and are usually surrounded by
1921:
1465:
1448:
479:
371:
240:
212:
1884:
1765:
1495:
1315:"Rationale and efficacy of interleukin-1 targeting in Erdheim-Chester disease"
1154:
1137:
1013:"Vemurafenib in the Treatment of Erdheim Chester Disease: A Systematic Review"
720:
660:"Erdheim-Chester disease. Clinical and radiologic characteristics of 59 cases"
327:. In some, there is histiocyte proliferation, and on staining, the section is
2159:
1964:
1734:
1717:
657:
441:
277:
228:
218:
103:
42:
1562:
979:
1847:
1832:
1803:
1773:
1743:
1708:
1673:
1643:
1600:
1570:
1541:
1503:
1474:
1439:
1410:
1375:
1340:
1163:
1114:
1048:
997:
964:"Treatment of Erdheim-Chester disease with cladribine: a rational approach"
948:
853:
809:
728:
639:
598:
590:
553:
382:
377:
284:
188:
159:
80:
685:
1029:
706:
630:
613:
412:
399:
353:
343:
281:
252:
signal changes on T2-weighted images seem to be typical of ECD, however,
123:
1867:
1190:
1064:"FDA Approves Oral MEK Inhibitor Cobimetinib for Histiocytic Neoplasms"
364:
276:(LCH) in a number of ways. Unlike LCH, ECD does not stain positive for
245:
119:
115:
96:
92:
1665:
1401:
1384:
1366:
1349:
930:
394:
292:
198:
173:
131:
356:, an oral inhibitor of MEK1 and MEK2, was approved in November 2022.
308:
300:
193:
60:
457:
1212:
437:
208:
155:
1933:
1135:
576:
1879:
1718:"Response of histiocytoses to imatinib mesylate: fire to ashes"
483:
445:
432:
The first case of ECD was reported by the American pathologist
312:
84:
825:
773:
611:
324:
111:
1447:
Braiteh F, Boxrud C, Esmaeli B, Kurzrock R (November 2005).
328:
249:
868:"FDA Approves First Treatment for Erdheim-Chester Disease"
325:
codon 600 substituting the amino acid glutamine for valine
534:. United States National Library of Medicine. 8 July 2008
504:"Erdheim-Chester Disease Declared a Histiocytic Neoplasm"
460:
is a support and advocacy group with the goal of raising
339:
There are two FDA-approved targeted drugs to treat ECD.
961:
912:
1010:
256:
and metabolic diseases must also be considered in the
1086:
774:
Egan A, Sorajja D, Jaroszewski D, Mookadam F (2012).
416:
treatment with targeted therapies in most patients."
1857:
887:"FDA Approves Cobimetinib for Histiocytic Neoplasms"
618:
The Journal of Clinical Endocrinology and Metabolism
1061:
106:). It was declared a histiocytic neoplasm by the
2157:
1241:NORD (National Organization for Rare Disorders)
1177:Chester W (1930). "Über Lipoidgranulomatose".
2076:Hereditary progressive mucinous histiocytosis
2002:Congenital self-healing reticulohistiocytosis
1949:
1261:"What Do I Do Now? - Erdheim-Chester Disease"
780:International Journal of Surgery Case Reports
653:
651:
649:
748:
746:
411:been dramatically efficacious. In 2019, the
1129:
570:
99:(technically, this disease is termed a non-
1956:
1942:
646:
65:
41:
1733:
1698:
1633:
1464:
1400:
1365:
1330:
1153:
1104:
1038:
1028:
987:
938:
843:
799:
743:
675:
629:
1432:10.7326/0003-4819-135-9-200111060-00027
1176:
1062:The ASCO Post Staff (2 November 2022).
458:Erdheim–Chester Disease Global Alliance
2158:
2124:Interdigitating dendritic cell sarcoma
1792:Clinical and Experimental Rheumatology
451:
1937:
1231:
1229:
908:
906:
821:
819:
137:
968:The British Journal of Ophthalmology
955:
884:
262:Video-assisted thoracoscopic surgery
35:Polyostotic sclerosing histiocytosis
767:
579:Deutsche Medizinische Wochenschrift
151:and heart involvement is observed.
114:-laden macrophages, multinucleated
13:
2085:Multicentric reticulohistiocytosis
2031:Hemophagocytic lymphohistiocytosis
1306:
1226:
903:
816:
211:caused by interlobular septal and
14:
2197:
2129:Follicular dendritic cell sarcoma
2066:Progressive nodular histiocytosis
2056:Generalized eruptive histiocytoma
2013:non-Langerhans cell histiocytosis
1963:
1840:
1635:10.1161/CIRCULATIONAHA.108.825075
1593:10.1097/01.md.0000145368.17934.91
436:in 1930, during his visit to the
360:Other treatment options include:
272:Histologically, ECD differs from
2186:Diseases named after discoverers
2095:Indeterminate cell histiocytosis
677:10.1097/00005792-199605000-00005
516:– via erdheim-chester.org.
118:, an inflammatory infiltrate of
1992:Hand–Schüller–Christian disease
1277:
1253:
1205:
1170:
1080:
1055:
1004:
878:
860:
419:
1534:10.1016/j.clinimag.2008.09.009
919:American Journal of Hematology
756:. M. D. Anderson Cancer Center
700:
605:
546:
520:
496:
1:
2051:Benign cephalic histiocytosis
1974:Langerhans cell histiocytosis
489:
274:Langerhans cell histiocytosis
77:Erdheim–Chester disease (ECD)
1825:10.1016/j.jbspin.2008.09.013
1722:Journal of Clinical Oncology
1700:10.1182/blood-2008-03-148304
1332:10.1182/blood-2010-04-279240
1142:Current Rheumatology Reports
1106:10.1016/j.mayocp.2019.02.023
845:10.1182/blood-2012-07-446286
486:is Erdheim–Chester disease.
405:
334:
267:
234:
7:
1420:Annals of Internal Medicine
792:10.1016/j.ijscr.2011.12.001
10:
2202:
1466:10.1182/blood-2005-06-2238
885:Rosa K (2 November 2022).
427:
2181:Syndromes affecting blood
2137:
2103:
2010:
1971:
1861:
1766:10.1007/s00415-006-0160-9
1496:10.1007/s00330-010-1830-7
1285:"Internet Movie Database"
1265:Histiocytosis Association
1237:"Erdheim Chester disease"
1213:"Erdheim–Chester Disease"
1155:10.1007/s11926-014-0412-0
1138:"Erdheim-Chester disease"
754:"Erdheim Chester Disease"
721:10.1007/s00234-003-0950-z
558:Histiocytosis Association
554:"Erdheim-Chester Disease"
528:"Erdheim–Chester disease"
108:World Health Organization
54:
49:
40:
26:
21:
2026:Juvenile xanthogranuloma
1735:10.1200/JCO.2010.29.9073
1654:Arthritis and Rheumatism
1389:Arthritis and Rheumatism
1354:Arthritis and Rheumatism
532:Medical Subject Headings
478:, season 2 episode 17, "
467:
179:Retroperitoneal fibrosis
31:Erdheim–Chester syndrome
2119:Langerhans cell sarcoma
2106:malignant histiocytosis
2036:Erdheim-Chester disease
1563:10.1148/radiol.10090320
1093:Mayo Clinic Proceedings
980:10.1136/bjo.2003.035584
299:. Tissue samples show
122:and histiocytes in the
85:abnormal multiplication
50:Chester-Erdheim disease
22:Erdheim–Chester disease
2046:Sea-blue histiocytosis
1997:Eosinophilic granuloma
872:Pharmacy Practice News
591:10.1055/s-0031-1286368
258:differential diagnosis
203:central nervous system
87:of a specific type of
2145:Rosai–Dorfman disease
2081:Reticulohistiocytosis
2061:Xanthoma disseminatum
1987:Letterer–Siwe disease
1215:. ECD Global Alliance
83:characterized by the
2089:Reticulohistiocytoma
2041:Niemann–Pick disease
1979:X-type histiocytosis
1754:Journal of Neurology
1030:10.7759/cureus.25935
631:10.1210/jc.2006-2018
164:pericardial effusion
126:, and a generalized
2114:Histiocytic sarcoma
2018:Non-X histiocytosis
452:Society and culture
305:xanthogranulomatous
289:electron microscopy
2176:Skeletal disorders
1484:European Radiology
1191:10.1007/BF01942684
874:. 6 November 2017.
295:does not disclose
254:multiple sclerosis
184:Diabetes insipidus
138:Signs and symptoms
2153:
2152:
1931:
1930:
1760:(10): 1267–1277.
1728:(31): e633–e636.
1693:(11): 5413–5415.
1666:10.1002/art.22165
1660:(10): 3330–3336.
1628:(25): e597–e598.
1490:(11): 2579–2587.
1402:10.1002/art.27672
1395:(11): 3504–3512.
1367:10.1002/art.24848
1360:(10): 3128–3138.
1325:(20): 4070–4076.
1099:(10): 2054–2071.
931:10.1002/ajh.26441
585:(39): 1952–1956.
389:Radiation therapy
317:mutations of the
89:white blood cells
74:
73:
16:Medical condition
2193:
2071:Papular xanthoma
1958:
1951:
1944:
1935:
1934:
1859:
1858:
1836:
1813:Joint Bone Spine
1807:
1785:
1747:
1737:
1712:
1702:
1677:
1647:
1637:
1612:
1574:
1545:
1522:Clinical Imaging
1515:
1478:
1468:
1459:(9): 2992–2994.
1443:
1414:
1404:
1379:
1369:
1344:
1334:
1300:
1299:
1297:
1295:
1281:
1275:
1274:
1272:
1271:
1257:
1251:
1250:
1248:
1247:
1233:
1224:
1223:
1221:
1220:
1209:
1203:
1202:
1174:
1168:
1167:
1157:
1133:
1127:
1126:
1108:
1084:
1078:
1077:
1075:
1074:
1059:
1053:
1052:
1042:
1032:
1008:
1002:
1001:
991:
959:
953:
952:
942:
910:
901:
900:
898:
897:
882:
876:
875:
864:
858:
857:
847:
838:(9): 1495–1500.
823:
814:
813:
803:
771:
765:
764:
762:
761:
750:
741:
740:
704:
698:
697:
679:
655:
644:
643:
633:
624:(6): 2007–2012.
609:
603:
602:
574:
568:
567:
565:
564:
550:
544:
543:
541:
539:
524:
518:
517:
515:
514:
508:
500:
297:Birbeck granules
157:
79:is an extremely
70:
69:
45:
19:
18:
2201:
2200:
2196:
2195:
2194:
2192:
2191:
2190:
2156:
2155:
2154:
2149:
2133:
2099:
2016:
2006:
1977:
1967:
1962:
1932:
1927:
1926:
1870:
1843:
1810:
1788:
1750:
1715:
1680:
1650:
1615:
1577:
1548:
1518:
1481:
1446:
1417:
1382:
1347:
1312:
1309:
1307:Further reading
1304:
1303:
1293:
1291:
1283:
1282:
1278:
1269:
1267:
1259:
1258:
1254:
1245:
1243:
1235:
1234:
1227:
1218:
1216:
1211:
1210:
1206:
1175:
1171:
1134:
1130:
1085:
1081:
1072:
1070:
1060:
1056:
1009:
1005:
960:
956:
911:
904:
895:
893:
883:
879:
866:
865:
861:
824:
817:
772:
768:
759:
757:
752:
751:
744:
705:
701:
656:
647:
610:
606:
575:
571:
562:
560:
552:
551:
547:
537:
535:
526:
525:
521:
512:
510:
506:
502:
501:
497:
492:
472:In the TV show
470:
454:
434:William Chester
430:
422:
408:
350:V600E mutation.
337:
270:
237:
224:Hypopituitarism
149:pituitary gland
140:
101:Langerhans-cell
64:
17:
12:
11:
5:
2199:
2189:
2188:
2183:
2178:
2173:
2168:
2151:
2150:
2148:
2147:
2141:
2139:
2135:
2134:
2132:
2131:
2126:
2121:
2116:
2110:
2108:
2101:
2100:
2098:
2097:
2092:
2078:
2073:
2068:
2063:
2058:
2053:
2048:
2043:
2038:
2033:
2028:
2022:
2020:
2008:
2007:
2005:
2004:
1999:
1994:
1989:
1983:
1981:
1969:
1968:
1961:
1960:
1953:
1946:
1938:
1929:
1928:
1925:
1924:
1913:
1902:
1887:
1871:
1866:
1865:
1863:
1862:Classification
1856:
1855:
1842:
1841:External links
1839:
1838:
1837:
1819:(3): 315–317.
1808:
1798:(2): 232–236.
1786:
1748:
1713:
1678:
1648:
1613:
1587:(6): 371–392.
1575:
1557:(2): 586–594.
1546:
1528:(2): 150–153.
1516:
1479:
1444:
1426:(9): 844–845.
1415:
1380:
1345:
1308:
1305:
1302:
1301:
1276:
1252:
1225:
1204:
1185:(2): 561–602.
1169:
1128:
1079:
1054:
1003:
974:(6): 844–847.
954:
925:(3): 293–302.
902:
877:
859:
815:
786:(3): 107–110.
766:
742:
715:(4): 241–245.
709:Neuroradiology
699:
670:(3): 157–169.
645:
604:
569:
545:
519:
494:
493:
491:
488:
469:
466:
453:
450:
429:
426:
421:
418:
407:
404:
403:
402:
397:
391:
386:
380:
375:
372:corticosteroid
368:
358:
357:
351:
336:
333:
278:S-100 proteins
269:
266:
241:osteosclerosis
236:
233:
232:
231:
226:
221:
219:Kidney failure
216:
206:
196:
191:
186:
181:
176:
156:juxtaarticular
139:
136:
72:
71:
58:
52:
51:
47:
46:
38:
37:
28:
24:
23:
15:
9:
6:
4:
3:
2:
2198:
2187:
2184:
2182:
2179:
2177:
2174:
2172:
2171:Histiocytosis
2169:
2167:
2166:Rare diseases
2164:
2163:
2161:
2146:
2143:
2142:
2140:
2136:
2130:
2127:
2125:
2122:
2120:
2117:
2115:
2112:
2111:
2109:
2107:
2102:
2096:
2093:
2090:
2086:
2082:
2079:
2077:
2074:
2072:
2069:
2067:
2064:
2062:
2059:
2057:
2054:
2052:
2049:
2047:
2044:
2042:
2039:
2037:
2034:
2032:
2029:
2027:
2024:
2023:
2021:
2019:
2014:
2009:
2003:
2000:
1998:
1995:
1993:
1990:
1988:
1985:
1984:
1982:
1980:
1975:
1970:
1966:
1965:Histiocytosis
1959:
1954:
1952:
1947:
1945:
1940:
1939:
1936:
1923:
1919:
1918:
1914:
1912:
1908:
1907:
1903:
1901:
1897:
1896:
1892:
1888:
1886:
1882:
1881:
1877:
1873:
1872:
1869:
1864:
1860:
1854:
1850:
1849:
1845:
1844:
1834:
1830:
1826:
1822:
1818:
1814:
1809:
1805:
1801:
1797:
1793:
1787:
1783:
1779:
1775:
1771:
1767:
1763:
1759:
1755:
1749:
1745:
1741:
1736:
1731:
1727:
1723:
1719:
1714:
1710:
1706:
1701:
1696:
1692:
1688:
1684:
1679:
1675:
1671:
1667:
1663:
1659:
1655:
1649:
1645:
1641:
1636:
1631:
1627:
1623:
1619:
1614:
1610:
1606:
1602:
1598:
1594:
1590:
1586:
1582:
1576:
1572:
1568:
1564:
1560:
1556:
1552:
1547:
1543:
1539:
1535:
1531:
1527:
1523:
1517:
1513:
1509:
1505:
1501:
1497:
1493:
1489:
1485:
1480:
1476:
1472:
1467:
1462:
1458:
1454:
1450:
1445:
1441:
1437:
1433:
1429:
1425:
1421:
1416:
1412:
1408:
1403:
1398:
1394:
1390:
1386:
1381:
1377:
1373:
1368:
1363:
1359:
1355:
1351:
1346:
1342:
1338:
1333:
1328:
1324:
1320:
1316:
1311:
1310:
1290:
1286:
1280:
1266:
1262:
1256:
1242:
1238:
1232:
1230:
1214:
1208:
1200:
1196:
1192:
1188:
1184:
1180:
1173:
1165:
1161:
1156:
1151:
1147:
1143:
1139:
1132:
1124:
1120:
1116:
1112:
1107:
1102:
1098:
1094:
1090:
1083:
1069:
1068:The ASCO Post
1065:
1058:
1050:
1046:
1041:
1036:
1031:
1026:
1023:(6): e25935.
1022:
1018:
1014:
1007:
999:
995:
990:
985:
981:
977:
973:
969:
965:
958:
950:
946:
941:
936:
932:
928:
924:
920:
916:
909:
907:
892:
888:
881:
873:
869:
863:
855:
851:
846:
841:
837:
833:
829:
822:
820:
811:
807:
802:
797:
793:
789:
785:
781:
777:
770:
755:
749:
747:
738:
734:
730:
726:
722:
718:
714:
710:
703:
695:
691:
687:
683:
678:
673:
669:
665:
661:
654:
652:
650:
641:
637:
632:
627:
623:
619:
615:
608:
600:
596:
592:
588:
584:
581:(in German).
580:
573:
559:
555:
549:
533:
529:
523:
509:. 18 May 2016
505:
499:
495:
487:
485:
481:
477:
476:
465:
463:
459:
449:
447:
443:
442:Jakob Erdheim
439:
435:
425:
417:
414:
401:
398:
396:
392:
390:
387:
384:
381:
379:
376:
373:
369:
366:
363:
362:
361:
355:
352:
349:
345:
342:
341:
340:
332:
331:+ and CD1a-.
330:
326:
322:
320:
314:
310:
306:
302:
298:
294:
290:
286:
285:glycoproteins
283:
279:
275:
265:
263:
259:
255:
251:
247:
242:
230:
229:Liver failure
227:
225:
222:
220:
217:
214:
210:
207:
204:
200:
197:
195:
192:
190:
187:
185:
182:
180:
177:
175:
172:
171:
170:
167:
165:
161:
152:
150:
146:
135:
133:
129:
125:
121:
117:
113:
109:
105:
104:histiocytosis
102:
98:
94:
90:
86:
82:
78:
68:
62:
59:
57:
53:
48:
44:
39:
36:
32:
29:
25:
20:
2035:
1915:
1904:
1889:
1874:
1846:
1816:
1812:
1795:
1791:
1757:
1753:
1725:
1721:
1690:
1686:
1657:
1653:
1625:
1621:
1584:
1580:
1554:
1550:
1525:
1521:
1487:
1483:
1456:
1452:
1423:
1419:
1392:
1388:
1357:
1353:
1322:
1318:
1292:. Retrieved
1288:
1279:
1268:. Retrieved
1264:
1255:
1244:. Retrieved
1240:
1217:. Retrieved
1207:
1182:
1178:
1172:
1145:
1141:
1131:
1096:
1092:
1082:
1071:. Retrieved
1067:
1057:
1020:
1016:
1006:
971:
967:
957:
922:
918:
894:. Retrieved
890:
880:
871:
862:
835:
831:
783:
779:
769:
758:. Retrieved
712:
708:
702:
667:
663:
621:
617:
607:
582:
578:
572:
561:. Retrieved
557:
548:
536:. Retrieved
531:
522:
511:. Retrieved
498:
473:
471:
455:
440:pathologist
431:
423:
420:Epidemiology
409:
383:Pexidartinib
378:Chemotherapy
359:
347:
338:
318:
301:xanthomatous
282:Group 1 CD1a
271:
238:
199:Neurological
189:Exophthalmos
168:
160:Exophthalmos
153:
145:retroorbital
141:
95:, or tissue
81:rare disease
76:
75:
34:
30:
1622:Circulation
413:Mayo Clinic
400:Ciclosporin
354:Cobimetinib
344:Vemurafenib
239:Radiologic
205:involvement
158:in nature.
124:bone marrow
120:lymphocytes
116:giant cells
97:macrophages
93:histiocytes
27:Other names
2160:Categories
1917:DiseasesDB
1294:27 October
1270:2016-03-01
1246:2016-03-01
1219:2009-05-08
1148:(4): 412.
1073:2022-11-19
896:2022-11-19
760:2007-08-26
563:2017-12-21
513:2018-07-18
490:References
370:High-dose
365:Interferon
246:cerebellar
215:thickening
132:long bones
2138:Ungrouped
1551:Radiology
1123:201713697
462:awareness
406:Prognosis
395:debulking
393:Surgical
335:Treatment
293:cytoplasm
268:Histology
235:Diagnosis
194:Xanthomas
174:Bone pain
128:sclerosis
56:Specialty
2104:WHO-III/
1833:19119043
1804:12747282
1782:27976718
1774:17063320
1744:20733125
1709:18502845
1674:17009306
1644:19564564
1601:15525849
1581:Medicine
1571:20413768
1542:19237062
1504:20563815
1475:16020507
1440:11694122
1411:20662053
1376:19790052
1341:20724540
1199:27359311
1164:24532298
1115:31472931
1049:35844342
998:15148234
949:34978715
854:23258922
810:22288060
729:12687308
694:32150913
664:Medicine
640:17405844
599:21935854
438:Austrian
309:fibrosis
291:of cell
147:tissue,
61:Oncology
2011:WHO-II/
1911:D031249
1609:1426013
1512:5775587
1040:9282605
989:1772168
940:9536810
891:OncLive
801:3267285
737:9513277
686:8965684
538:19 June
428:History
374:therapy
311:. Bone
250:pontine
213:pleural
209:Dyspnea
130:of the
91:called
1972:WHO-I/
1853:CHORUS
1831:
1802:
1780:
1772:
1742:
1707:
1672:
1642:
1607:
1599:
1569:
1540:
1510:
1502:
1473:
1438:
1409:
1374:
1339:
1197:
1162:
1121:
1113:
1047:
1037:
1017:Cureus
996:
986:
947:
937:
852:
808:
798:
735:
727:
692:
684:
638:
597:
484:ataxia
480:All In
446:Vienna
313:biopsy
287:, and
63:
1922:29792
1900:202.3
1885:C96.1
1848:01067
1778:S2CID
1687:Blood
1605:S2CID
1508:S2CID
1453:Blood
1319:Blood
1195:S2CID
1119:S2CID
832:Blood
733:S2CID
690:S2CID
507:(PDF)
475:House
468:Media
112:lipid
1906:MeSH
1895:9-CM
1829:PMID
1800:PMID
1770:PMID
1740:PMID
1705:PMID
1670:PMID
1640:PMID
1597:PMID
1567:PMID
1538:PMID
1500:PMID
1471:PMID
1436:PMID
1407:PMID
1372:PMID
1337:PMID
1296:2021
1289:IMDB
1160:PMID
1111:PMID
1045:PMID
994:PMID
945:PMID
850:PMID
806:PMID
725:PMID
682:PMID
636:PMID
595:PMID
540:2008
456:The
348:BRAF
329:CD68
321:gene
319:BRAF
248:and
201:and
1891:ICD
1876:ICD
1851:at
1821:doi
1762:doi
1758:253
1730:doi
1695:doi
1691:111
1662:doi
1630:doi
1626:119
1589:doi
1559:doi
1555:255
1530:doi
1492:doi
1461:doi
1457:106
1428:doi
1424:135
1397:doi
1362:doi
1327:doi
1323:116
1187:doi
1183:279
1150:doi
1101:doi
1035:PMC
1025:doi
984:PMC
976:doi
935:PMC
927:doi
840:doi
836:121
796:PMC
788:doi
717:doi
672:doi
626:doi
587:doi
583:136
444:in
323:at
303:or
280:or
33:or
2162::
2087:,
1920::
1909::
1898::
1883::
1880:10
1827:.
1817:76
1815:.
1796:21
1794:.
1776:.
1768:.
1756:.
1738:.
1726:28
1724:.
1720:.
1703:.
1689:.
1685:.
1668:.
1658:54
1656:.
1638:.
1624:.
1620:.
1603:.
1595:.
1585:83
1583:.
1565:.
1553:.
1536:.
1526:33
1524:.
1506:.
1498:.
1488:20
1486:.
1469:.
1455:.
1451:.
1434:.
1422:.
1405:.
1393:62
1391:.
1387:.
1370:.
1358:60
1356:.
1352:.
1335:.
1321:.
1317:.
1287:.
1263:.
1239:.
1228:^
1193:.
1181:.
1158:.
1146:16
1144:.
1140:.
1117:.
1109:.
1097:94
1095:.
1091:.
1066:.
1043:.
1033:.
1021:14
1019:.
1015:.
992:.
982:.
972:88
970:.
966:.
943:.
933:.
923:97
921:.
917:.
905:^
889:.
870:.
848:.
834:.
830:.
818:^
804:.
794:.
782:.
778:.
745:^
731:.
723:.
713:45
711:.
688:.
680:.
668:75
666:.
662:.
648:^
634:.
622:92
620:.
616:.
593:.
556:.
530:.
448:.
367:-α
134:.
2091:)
2083:(
2015:/
1976:/
1957:e
1950:t
1943:v
1893:-
1878:-
1868:D
1835:.
1823::
1806:.
1784:.
1764::
1746:.
1732::
1711:.
1697::
1676:.
1664::
1646:.
1632::
1611:.
1591::
1573:.
1561::
1544:.
1532::
1514:.
1494::
1477:.
1463::
1442:.
1430::
1413:.
1399::
1378:.
1364::
1343:.
1329::
1298:.
1273:.
1249:.
1222:.
1201:.
1189::
1166:.
1152::
1125:.
1103::
1076:.
1051:.
1027::
1000:.
978::
951:.
929::
899:.
856:.
842::
812:.
790::
784:3
763:.
739:.
719::
696:.
674::
642:.
628::
601:.
589::
566:.
542:.
Text is available under the Creative Commons Attribution-ShareAlike License. Additional terms may apply.
