298:
at position 24.1, denoted 10q24.1. This gene is member 6 of the TNF-receptor superfamily (TNFRSF6). The Fas receptor contains a death domain and has been shown to play a central role in the physiological regulation of programmed cell death. Normally, stimulation of recently activated T cells by
383:
therapies. In most reported cases, these cause B cell lymphoproliferative disorders; however, some T cell variations have been described. The T cell variations are usually caused by the prolonged use of T cell suppressant drugs, such as
242:. Individuals who have some sort of dysfunction with their immune system are susceptible to develop a lymphoproliferative disorder because when any of the numerous control points of the immune system become dysfunctional,
601:
586:
201:
1570:
303:
of the cell and is important for eliminating T cells that are repeatedly stimulated by antigens. As a result of the mutation in the Fas receptor gene, there is no recognition of Fas by
1314:
186:
400:, which infects >90% of the world population, is also a common cause of these disorders, being responsible for a wide range of non-malignant, pre-malignant, and malignant
401:
868:
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1340:
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17:
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1007:
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1108:
906:
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1206:
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285:
175:
1379:
367:
infection because it is highly associated with acquired immunodeficiency, which often leads to lymphoproliferative disorders.
299:
antigen leads to coexpression of Fas and Fas receptor on the T cell surface. The engagement of Fas by Fas receptor results in
1198:
749:
143:
1644:
1527:
451:
290:
Some children with autoimmune lymphoproliferative disorders are heterozygous for a mutation in the gene that codes for the
263:
196:
159:
524:
Rezk SA, Zhao X, Weiss LM (September 2018). "Epstein-Barr virus (EBV)-associated lymphoid proliferations, a 2018 update".
1483:
1103:
901:
785:
1431:
1142:
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122:
672:
514:
Abbas, A.K and
Lichtman, A.H. Cellular and Molecular Immunology. Fifth Edition. Elsevier Saunders. Philadelphia. 2005
1335:
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is a very common cause of lymphoproliferative disorders. In children, the most common is believed to be congenital
330:
323:
181:
334:
1565:
1557:
1416:
1294:
1233:
1219:
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https://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=gene&dopt=full_report&list_uids=355
1473:
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882:
153:
74:
1505:
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69:
are produced in excessive quantities. These disorders primarily present in patients who have a compromised
1446:
1319:
1025:
1361:
438:
250:
that have been identified to cause lymphoproliferative disorders; however, there are also acquired and
1249:
1090:
1012:
342:
212:
Lymphoproliferative disorders are a set of disorders characterized by the abnormal proliferation of
1451:
1279:
616:
917:
235:
1468:
1345:
1324:
1238:
1019:
316:
315:
Boys with X-linked immunodeficiency syndrome are at a higher risk of mortality associated with
164:
86:
1477:
1067:
1040:
961:
863:
376:
65:) refer to a specific class of diagnoses, comprising a group of several conditions, in which
657:
307:, leading to a primitive population of T cells that proliferates in an uncontrolled manner.
1274:
886:
797:
705:
459:"Idiopathic Interstitial Pneumonias: Interstitial Lung Diseases: Merck Manual Professional"
8:
1074:
858:
828:
605:
273:
117:
102:
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397:
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infections, and are predisposed to develop a lymphoproliferative disorder or lymphoma.
82:
78:
1618:
1269:
1258:
1048:
938:
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541:
380:
348:
Even though ataxia telangiectasia is an autosomal recessive disorder, people who are
191:
41:
1288:
553:
458:
352:
for this still have an increased risk of developing a lymphoproliferative disorder.
246:
or deregulation of lymphocytes is more likely to occur. There are several inherited
1388:
1167:
1035:
934:
645:
533:
243:
138:
90:
73:. Due to this factor, there are instances of these conditions being equated with "
537:
1161:
1156:
965:
849:
715:
710:
621:
360:
133:
128:
1608:
413:
326:(CVID) are also at a higher risk of developing a lymphoproliferative disorder.
595:
1633:
1515:
1214:
1123:
1079:
896:
770:
489:
428:
329:
Some disorders that predispose a person to lymphoproliferative disorders are
295:
220:
70:
1118:
780:
545:
349:
304:
291:
269:
488:
Winter, S.S. Lymphoproliferative disorders. Emedicine. December 20, 2006.
792:
393:
239:
232:
66:
578:
202:
Primary cutaneous acral CD8 positive T cell lymphoproliferative disorder
688:
389:
251:
217:
213:
46:
375:
There are many lymphoproliferative disorders that are associated with
640:
418:
385:
300:
1603:
692:
423:
247:
50:
433:
1151:
590:
228:
224:
974:
839:
823:
687:
1437:
1331:
1315:
Non-mycosis fungoides CD30â cutaneous large T-cell lymphoma
1310:
1228:
970:
945:
925:
921:
808:
726:
721:
501:
Entrez Gene. FAS Fas (TNF receptor superfamily, member 6).
96:
402:
Epstein-Barr virus-associated lymphoproliferative diseases
279:
187:
EpsteinâBarr virus-associated lymphoproliferative diseases
1177:
1172:
1062:
364:
257:
27:
Excessive production of lymphocytes by the immune system
568:
1385:Peripheral T-cell lymphoma not otherwise specified
1207:Precursor T acute lymphoblastic leukemia/lymphoma
761:Precursor B acute lymphoblastic leukemia/lymphoma
1631:
951:Nodular lymphocyte predominant Hodgkin lymphoma
1341:Secondary cutaneous CD30+ large-cell lymphoma
673:
523:
1031:Post-transplant lymphoproliferative disorder
986:immunoproliferative immunoglobulin disorders
171:Post-transplant lymphoproliferative disorder
85:of immunoproliferative disordersâalong with
1544:Diffuse infiltrative lymphocytosis syndrome
517:
1583:Jessner lymphocytic infiltrate of the skin
1129:Primary cutaneous follicle center lymphoma
874:Primary cutaneous follicle center lymphoma
680:
666:
490:http://www.emedicine.com/ped/topic1345.htm
97:Lymphoproliferative disorders (examples)
310:
223:. The two major types of lymphocytes are
1109:Primary cutaneous marginal zone lymphoma
907:Primary cutaneous marginal zone lymphoma
484:
482:
480:
478:
476:
474:
1571:with bandlike and perivascular patterns
1533:Autoimmune lymphoproliferative syndrome
286:Autoimmune lymphoproliferative syndrome
280:Autoimmune lymphoproliferative disorder
176:Autoimmune lymphoproliferative syndrome
14:
1632:
1380:Enteropathy-associated T-cell lymphoma
341:(an X-linked recessive disorder), and
294:, which is located on the long arm of
81:, lymphoproliferative disorders are a
661:
471:
258:X-linked Lymphoproliferative disorder
1528:X-linked lymphoproliferative disease
508:
495:
370:
264:X-linked lymphoproliferative disease
197:X-linked lymphoproliferative disease
160:Lymphocyte-variant hypereosinophilia
1640:Lymphocytic immune system disorders
1484:Large granular lymphocytic leukemia
1104:Intravascular large B-cell lymphoma
24:
355:
123:Hemophagocytic lymphohistiocytosis
25:
1656:
564:
331:severe combined immunodeficiency
324:common variable immunodeficiency
272:is associated with a T cell and
1336:CD30+ cutaneous T-cell lymphoma
182:Lymphoid interstitial pneumonia
144:Waldenström's macroglobulinemia
1566:Cutaneous lymphoid hyperplasia
1558:Cutaneous lymphoid hyperplasia
1417:Adult T-cell leukemia/lymphoma
1295:Adult T-cell leukemia/lymphoma
1234:Anaplastic large-cell lymphoma
1114:Primary cutaneous immunocytoma
1055:Splenic marginal zone lymphoma
276:lymphoproliferative disorder.
13:
1:
1614:Lymphoproliferative disorders
1524:Lymphoproliferative disorders
1474:Extranodal NK-T-cell lymphoma
1346:Lymphomatoid papulosis type A
1325:Lymphomatoid papulosis type B
1239:Lymphomatoid papulosis type A
1099:Diffuse large B-cell lymphoma
538:10.1016/j.humpath.2018.05.020
444:
154:Langerhans cell histiocytosis
75:immunoproliferative disorders
59:Lymphoproliferative disorders
36:Lymphoproliferative disorders
1506:Acute biphenotypic leukaemia
1395:Subcutaneous T-cell lymphoma
113:Acute lymphoblastic leukemia
108:Chronic lymphocytic leukemia
7:
1645:Infectious causes of cancer
1447:Aggressive NK-cell leukemia
1320:Pleomorphic T-cell lymphoma
1026:Lymphomatoid granulomatosis
407:
18:Lymphoproliferative disease
10:
1661:
439:Myeloproliferative disease
283:
261:
1591:
1556:
1514:
1496:
1460:
1430:
1403:
1358:
1303:
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748:
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231:, which are derived from
77:"; although, in terms of
40:
35:
1599:Hematological malignancy
1452:Blastic NK cell lymphoma
1280:Granulomatous slack skin
1036:Classic Hodgkin lymphoma
935:Classic Hodgkin lymphoma
339:WiskottâAldrich syndrome
335:ChĂ©diakâHigashi syndrome
236:hematopoietic stem cells
207:
149:WiskottâAldrich syndrome
505:. Accessed March 2007.
492:. Accessed March 2007.
311:Other inherited causes
165:Pityriasis lichenoides
87:hypergammaglobulinemia
1478:Angiocentric lymphoma
1068:AIDS-related lymphoma
892:Splenic marginal zone
377:organ transplantation
343:ataxiaâtelangiectasia
1576:with nodular pattern
1275:Pagetoid reticulosis
887:marginal zone B-cell
1075:Helicobacter pylori
902:Nodal marginal zone
829:Hairy cell leukemia
695:and related disease
274:natural killer cell
118:Hairy cell leukemia
103:Follicular lymphoma
1619:Lymphoid leukemias
1539:Leukemoid reaction
1375:Angioimmunoblastic
1041:Burkitt's lymphoma
632:External resources
398:Epstein-Barr virus
317:EpsteinâBarr virus
268:A mutation on the
1627:
1626:
1552:
1551:
1492:
1491:
1426:
1425:
1354:
1353:
1270:Mycosis fungoides
1137:
1136:
995:
994:
939:Nodular sclerosis
854:follicular B cell
655:
654:
381:immunosuppressant
371:Iatrogenic causes
192:Castleman disease
56:
55:
30:Medical condition
16:(Redirected from
1652:
1389:Lennert lymphoma
1255:
1254:
1186:
1185:
1148:
1147:
1013:Primary effusion
746:
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469:
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244:immunodeficiency
139:Multiple myeloma
134:T-cell lymphomas
129:B-cell lymphomas
91:paraproteinemias
33:
32:
21:
1660:
1659:
1655:
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1653:
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850:germinal center
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526:Human Pathology
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361:Viral infection
358:
356:Acquired causes
313:
288:
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266:
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210:
167:(PL, PLC, PLVA)
99:
31:
28:
23:
22:
15:
12:
11:
5:
1658:
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1609:Leukemia cutis
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1338:
1328:
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1289:SĂ©zary disease
1283:
1282:
1277:
1272:
1263:
1261:
1252:
1246:
1245:
1243:
1242:
1236:
1224:
1223:
1220:Prolymphocytic
1210:
1196:
1194:
1183:
1182:
1181:
1175:
1145:
1139:
1138:
1135:
1134:
1132:
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1116:
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1101:
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1059:
1058:
1057:
1045:
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1043:
1038:
1033:
1028:
1016:
1003:
1001:
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996:
993:
992:
990:
989:
980:
978:
958:
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942:
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929:
914:
913:
911:
910:
904:
899:
894:
878:
877:
871:
866:
861:
845:
843:
836:
835:
833:
832:
820:
818:Prolymphocytic
814:
812:
805:
804:
802:
801:
789:
776:
774:
767:
766:
764:
754:
752:
743:
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573:Classification
566:
565:External links
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507:
494:
470:
449:
448:
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443:
442:
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414:Evans syndrome
409:
406:
372:
369:
357:
354:
322:Children with
312:
309:
284:Main article:
281:
278:
262:Main article:
259:
256:
248:gene mutations
209:
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1525:
1522:
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1516:Lymphocytosis
1513:
1507:
1504:
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1501:
1495:
1485:
1482:
1479:
1475:
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1402:
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1371:
1370:Hepatosplenic
1368:
1367:
1365:
1363:
1357:
1347:
1344:
1342:
1339:
1337:
1333:
1330:
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1251:
1247:
1240:
1237:
1235:
1231:
1230:
1226:
1225:
1221:
1217:
1216:
1215:prolymphocyte
1212:
1211:
1208:
1204:
1200:
1197:
1195:
1187:
1184:
1179:
1176:
1174:
1171:
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1169:
1163:
1158:
1153:
1149:
1146:
1144:
1140:
1130:
1127:
1125:
1124:Plasmacytosis
1122:
1120:
1117:
1115:
1112:
1110:
1107:
1105:
1102:
1100:
1097:
1096:
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1080:MALT lymphoma
1077:
1076:
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1064:
1060:
1056:
1053:
1052:
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1050:
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883:marginal zone
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429:Lymphocytosis
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411:
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350:heterozygotes
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297:
296:chromosome 10
293:
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221:lymphocytosis
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71:immune system
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39:
34:
19:
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1523:
1467:
1410:
1404:By infection
1285:
1284:
1266:
1227:
1213:
1191:development/
1119:Plasmacytoma
1073:
1061:
1047:
1018:
1006:
1000:By infection
983:
944:
881:
848:
822:
791:
781:naive B cell
779:
740:development/
639:
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462:. Retrieved
453:
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314:
305:Fas receptor
292:Fas receptor
289:
270:X chromosome
267:
211:
79:nomenclature
62:
58:
57:
1286:aggressive:
1259:MF+variants
798:Mantle cell
793:mantle zone
394:ciclosporin
240:bone marrow
233:pluripotent
214:lymphocytes
67:lymphocytes
1634:Categories
1362:peripheral
859:Follicular
689:Leukaemias
464:2008-12-09
445:References
390:tacrolimus
252:iatrogenic
218:monoclonal
47:Hematology
1497:Lymphoid+
1267:indolent:
1250:Cutaneous
1091:Cutaneous
869:GCB DLBCL
864:Burkitt's
693:lymphomas
641:eMedicine
532:: 18â41.
419:Leukaemia
386:sirolimus
301:apoptosis
42:Specialty
1604:leukemia
1162:leukemia
1157:lymphoma
716:leukemia
711:lymphoma
646:ped/1345
554:47010934
546:29885408
424:Lymphoma
408:See also
333:(SCID),
254:causes.
83:subclass
51:oncology
1592:General
1499:myeloid
1461:T or NK
1432:NK cell
786:CLL/SLL
622:D008232
434:Myeloma
238:in the
229:T cells
225:B cells
216:into a
1436:(most
1412:HTLV-1
1304:Non-MF
1193:marker
1166:(most
1152:T cell
824:CD11c+
742:marker
720:(most
706:B cell
611:238.79
552:
544:
396:. The
178:(ALPS)
1359:Other
1229:CD30+
975:CD138
946:CD20+
840:CD79a
596:D47.9
550:S2CID
392:, or
208:Types
156:(LCH)
125:(HLH)
1438:CD56
1332:CD30
1311:CD30
1199:TdT+
1143:T/NK
1008:KSHV
984:see
971:CD38
962:PCDs
926:CD30
922:CD15
897:MALT
809:CD22
750:TdT+
727:CD20
722:CD19
617:MeSH
606:9-CM
542:PMID
379:and
227:and
89:and
63:LPDs
49:and
1469:EBV
1334:+:
1313:-:
1203:ALL
1178:CD8
1173:CD4
1168:CD3
1063:HIV
1049:HCV
1020:EBV
924:+,
771:CD5
757:ALL
602:ICD
587:ICD
534:doi
365:HIV
1636::
1201::
1189:By
977:+)
973:+/
966:PP
928:+)
918:RS
738:By
691:,
644::
620::
609::
594::
591:10
548:.
540:.
530:79
528:.
473:^
404:.
388:,
345:.
337:,
93:.
1535:)
1526:(
1480:)
1476:/
1472:(
1440:)
1434:/
1419:)
1415:(
1391:)
1387:(
1241:)
1232:(
1222:)
1218:(
1209:)
1205:(
1180:)
1164:)
1159:,
1155:(
1082:)
1078:(
1070:)
1066:(
1015:)
1011:(
969:(
964:/
953:)
949:(
941:)
937:(
920:(
909:)
890:(
885:/
876:)
857:(
852:/
842:+
831:)
827:(
811:+
800:)
796:(
788:)
784:(
773:+
763:)
759:(
729:)
718:)
713:,
709:(
681:e
674:t
667:v
604:-
589:-
579:D
556:.
536::
467:.
61:(
20:)
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