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T-cell prolymphocytic leukemia

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Clonal TCR gene rearrangements for the γ and δ chains are typically found. The most frequent chromosomal abnormality is the inversion of chromosome 14, specifically inv 14(q11;q32). This is found in 80% of cases, while 10% of cases show a reciprocal translocation of
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that attacks white blood cells, has been used in treatment with greater success than previous options. In one study of previously treated people with T-PLL, people who had a complete response to alemtuzumab survived a median of 16 months after treatment.
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T-cell prolymphocytic leukemia is difficult to treat, and it does not respond to most available chemotherapeutic drugs. Many different treatments have been attempted, with limited success in certain patients:
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Brito-Babapulle V, Catovsky D (1991). "Inversions and tandem translocations involving chromosome 14q11 and 14q32 in T-prolymphocytic leukemia and T-cell leukemias in patients with ataxia telangiectasia".
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Sorour A, Brito-Babapulle V, Smedley D, Yuille M, Catovsky D (2000). "Unusual breakpoint distribution of 8p abnormalities in T-prolymphocytic leukemia: a study with YACS mapping to 8p11-p12".
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Maljaei SH, Brito-Babapulle V, Hiorns LR, Catovsky D (1998). "Abnormalities of chromosomes 8, 11, 14, and X in T-prolymphocytic leukemia studied by fluorescence in situ hybridization".
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involvement. T-PLL is a very rare leukemia, primarily affecting adults over the age of 30. It represents 2% of all small lymphocytic leukemias in adults. Other names include
1603: 1817: 451: 380:, the median survival time was 7.5 months after diagnosis. More recently, some patients have survived five years and more, although the median survival is still low. 376:
T-PLL is an extremely rare aggressive disease, and patients are not expected to live normal lifespans. Before the recent introduction of better treatments, such as
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Elaine Sarkin Jaffe, Nancy Lee Harris, World Health Organization, International Agency for Research on Cancer, Harald Stein, J.W. Vardiman (2001).
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About four men are diagnosed with this disease for every three women. Despite its overall rarity, it is also the most common type of mature
298:- is present in 60% of cases, the CD4+/CD8+ immunophenotype is present in 25%, and the CD4-/CD8+ immunophenotype is present in 15% of cases. 1746: 1858: 1702: 1322: 1120: 1004: 219:
are usually round to oval in shape, with occasional patients having cells with a more irregular nuclear outline that is similar to the
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Matutes E, Garcia Talavera J, O'Brien M, Catovsky D (September 1986). "The morphological spectrum of T-prolymphocytic leukaemia".
227:. A small cell variant comprises 20% of all T-PLL cases, and the Sézary cell-like (cerebriform) variant is seen in 5% of cases. 1784: 1776: 1635: 1513: 1452: 1332: 1273: 1110: 1036: 1832: 1742: 1692: 1588: 1317: 1101: 1724: 1630: 1613: 1421: 975: 884: 1665: 1538: 1244: 1580: 1468: 1309: 1231: 365: 230:
Marrow involvement is typically diffuse with morphology similar to what is observed in peripheral blood. In the
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People affected by T-cell prolymphocytic leukemia typically have systemic disease at presentation, including
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Due to the systemic nature of this disease, leukemic cells can be found in peripheral blood,
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are seen approximately 75% of patients, including idic (8p11), t(8;8)(p11-12;q12), and
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It is postulated that the originating cell line for this disease is a mature (post-
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Valbuena JR, Herling M, Admirand JH, Padula A, Jones D, Medeiros LJ (March 2005).
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Most patients with T-cell prolymphocytic leukemia require immediate treatment.
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Pathology and genetics of tumours of haematopoietic and lymphoid tissues
676:"High remission rate in T-cell prolymphocytic leukemia with CAMPATH-1H" 155:. A high lymphocyte count (> 100 x 10/L) along with low amounts of 1370: 389: 251: 231: 184: 164: 156: 144: 98: 75: 1193: 1058: 1042: 148: 94: 82: 364:
Some patients who successfully respond to treatment also undergo
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Matutes E, Brito-Babapulle V, Swansbury J, et al. (1991).
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Dearden CE, Matutes E, Cazin B, et al. (September 2001).
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Non-mycosis fungoides CD30− cutaneous large T-cell lymphoma
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In the peripheral blood, T-PLL consists of medium-sized
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cyclophosphamide, doxorubicin, vincristine, prednisone
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T-prolymphocytic leukemia/T-cell lymphocytic leukemia.
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The 38: 29: 781: 740: 691: 487: 1328:Primary cutaneous marginal zone lymphoma 1126:Primary cutaneous marginal zone lymphoma 350:(CHOP), etoposide, bleomycin (VAPEC-B). 338:(pentostatin, fludarabine, cladribine), 234:, the leukemic cell infiltrate both the 1790:with bandlike and perivascular patterns 1752:Autoimmune lymphoproliferative syndrome 401: 1599:Enteropathy-associated T-cell lymphoma 509: 507: 465: 463: 461: 443: 419: 417: 415: 413: 411: 409: 407: 405: 72:T-cell-prolymphocytic leukemia (T-PLL) 7: 1747:X-linked lymphoproliferative disease 1703:Large granular lymphocytic leukemia 1323:Intravascular large B-cell lymphoma 342:, and various forms of combination 167:serologies are negative, and serum 126:enlargement of the liver and spleen 107:T-cell chronic lymphocytic leukemia 528:10.1111/j.1365-2141.1986.tb07579.x 163:in the blood are common findings. 25: 171:are within normal limits with no 725:"T-cell prolymphocytic leukemia" 254:and around the skin appendages. 111:"knobby" type of T-cell leukemia 1555:CD30+ cutaneous T-cell lymphoma 1785:Cutaneous lymphoid hyperplasia 1777:Cutaneous lymphoid hyperplasia 1636:Adult T-cell leukemia/lymphoma 1514:Adult T-cell leukemia/lymphoma 1453:Anaplastic large-cell lymphoma 1333:Primary cutaneous immunocytoma 1274:Splenic marginal zone lymphoma 489:10.1182/blood.V78.12.3269.3269 130:enlargement of the lymph nodes 33:T-cell-prolymphocytic leukemia 1: 1833:Lymphoproliferative disorders 1743:Lymphoproliferative disorders 1693:Extranodal NK-T-cell lymphoma 1565:Lymphomatoid papulosis type A 1544:Lymphomatoid papulosis type B 1458:Lymphomatoid papulosis type A 1318:Diffuse large B-cell lymphoma 642:10.1016/S0165-4608(00)00239-9 607:10.1016/S0165-4608(97)00410-X 368:to consolidate the response. 1725:Acute biphenotypic leukaemia 1614:Subcutaneous T-cell lymphoma 572:10.1016/0165-4608(91)90228-M 1666:Aggressive NK-cell leukemia 1539:Pleomorphic T-cell lymphoma 1245:Lymphomatoid granulomatosis 783:10.1309/93P4-2RNG-5XBG-3KBE 1875: 1859:Acute lymphocytic leukemia 742:10.1177/107327489800500102 919: 366:stem cell transplantation 221:cerebriform nuclear shape 46: 37: 18:Leukemia, T-Cell, chronic 1818:Hematological malignancy 1671:Blastic NK cell lymphoma 1499:Granulomatous slack skin 1255:Classic Hodgkin lymphoma 1154:Classic Hodgkin lymphoma 723:Matutes Estella (1998). 693:10.1182/blood.V98.6.1721 356:(Campath), an anti-CD52 132:, and skin infiltrates. 630:Cancer Genet. Cytogenet 595:Cancer Genet. Cytogenet 560:Cancer Genet. Cytogenet 314:Also, abnormalities of 290:. The immunophenotype 1697:Angiocentric lymphoma 1287:AIDS-related lymphoma 1111:Splenic marginal zone 344:chemotherapy regimens 311:(t(14;14)(q11;q32)). 1795:with nodular pattern 1494:Pagetoid reticulosis 1106:marginal zone B-cell 1294:Helicobacter pylori 1121:Nodal marginal zone 1048:Hairy cell leukemia 914:and related disease 770:Am. J. Clin. Pathol 358:monoclonal antibody 1838:Lymphoid leukemias 1758:Leukemoid reaction 1594:Angioimmunoblastic 1260:Burkitt's lymphoma 851:External resources 120:Signs and symptoms 1846: 1845: 1771: 1770: 1711: 1710: 1645: 1644: 1573: 1572: 1489:Mycosis fungoides 1356: 1355: 1214: 1213: 1158:Nodular sclerosis 1073:follicular B cell 874: 873: 282:and negative for 69: 68: 27:Medical condition 16:(Redirected from 1866: 1608:Lennert lymphoma 1474: 1405: 1367: 1232:Primary effusion 965: 954: 921: 901: 894: 887: 878: 808: 796: 795: 785: 761: 755: 754: 744: 720: 714: 713: 695: 671: 654: 653: 625: 619: 618: 590: 584: 583: 554: 548: 547: 511: 502: 501: 491: 467: 456: 455: 449: 441: 421: 336:purine analogues 302:Genetic findings 42: 30: 21: 1874: 1873: 1869: 1868: 1867: 1865: 1864: 1863: 1849: 1848: 1847: 1842: 1806: 1767: 1729: 1717: 1707: 1675: 1654: 1641: 1618: 1579: 1569: 1518: 1463: 1411: 1409: 1384: 1379: 1373: 1352: 1304: 1210: 1187: 1175: 1131: 1069:germinal center 1053: 1022: 984: 960: 958: 938: 933: 927: 915: 905: 875: 870: 869: 846: 845: 819: 805: 800: 799: 763: 762: 758: 722: 721: 717: 673: 672: 657: 627: 626: 622: 592: 591: 587: 556: 555: 551: 516:Br. J. Haematol 513: 512: 505: 482:(12): 3269–74. 469: 468: 459: 442: 438: 423: 422: 403: 398: 386: 374: 328: 304: 264:immunophenotype 260: 258:Immunophenotype 225:Sézary syndrome 198: 193: 181: 157:red blood cells 122: 28: 23: 22: 15: 12: 11: 5: 1872: 1870: 1862: 1861: 1851: 1850: 1844: 1843: 1841: 1840: 1835: 1830: 1828:Leukemia cutis 1825: 1820: 1814: 1812: 1808: 1807: 1805: 1804: 1799: 1798: 1797: 1792: 1781: 1779: 1773: 1772: 1769: 1768: 1766: 1765: 1760: 1755: 1749: 1739: 1737: 1731: 1730: 1728: 1727: 1721: 1719: 1713: 1712: 1709: 1708: 1706: 1705: 1700: 1683: 1681: 1677: 1676: 1674: 1673: 1668: 1662: 1660: 1647: 1646: 1643: 1642: 1640: 1639: 1626: 1624: 1620: 1619: 1617: 1616: 1611: 1601: 1596: 1591: 1585: 1583: 1575: 1574: 1571: 1570: 1568: 1567: 1562: 1557: 1547: 1546: 1541: 1536: 1526: 1524: 1520: 1519: 1517: 1516: 1508:Sézary disease 1502: 1501: 1496: 1491: 1482: 1480: 1471: 1465: 1464: 1462: 1461: 1455: 1443: 1442: 1439:Prolymphocytic 1429: 1415: 1413: 1402: 1401: 1400: 1394: 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1343:Plasmacytosis 1341: 1339: 1336: 1334: 1331: 1329: 1326: 1324: 1321: 1319: 1316: 1315: 1313: 1311: 1307: 1300: 1299:MALT lymphoma 1296: 1295: 1291: 1288: 1284: 1283: 1279: 1275: 1272: 1271: 1270: 1269: 1265: 1261: 1258: 1256: 1253: 1251: 1248: 1246: 1243: 1242: 1241: 1240: 1236: 1233: 1229: 1228: 1224: 1223: 1221: 1217: 1207: 1206: 1201: 1200: 1198: 1195: 1191: 1186: 1182: 1178: 1171: 1167: 1166: 1162: 1159: 1155: 1152: 1151: 1149: 1146: 1142: 1138: 1134: 1127: 1124: 1122: 1119: 1117: 1114: 1112: 1108: 1107: 1103: 1102:marginal zone 1099: 1098: 1094: 1091: 1089: 1086: 1084: 1081: 1079: 1075: 1074: 1070: 1066: 1065: 1063: 1060: 1056: 1049: 1045: 1044: 1040: 1038: 1035: 1034: 1032: 1029: 1025: 1018: 1014: 1013: 1009: 1006: 1002: 1001: 997: 996: 994: 991: 987: 981: 977: 974: 972: 970: 966: 963: 955: 952: 947: 944: 943: 942: 936: 931: 926: 922: 918: 913: 909: 902: 897: 895: 890: 888: 883: 882: 879: 866: 862: 861: 857: 856: 853: 849: 842: 838: 837: 833: 831: 827: 826: 822: 821: 818: 813: 809: 802: 793: 789: 784: 779: 776:(3): 456–64. 775: 771: 767: 760: 757: 752: 748: 743: 738: 734: 730: 726: 719: 716: 711: 707: 703: 699: 694: 689: 686:(6): 1721–6. 685: 681: 677: 670: 668: 666: 664: 662: 660: 656: 651: 647: 643: 639: 636:(2): 128–32. 635: 631: 624: 621: 616: 612: 608: 604: 600: 596: 589: 586: 581: 577: 573: 569: 565: 561: 553: 550: 545: 541: 537: 533: 529: 525: 522:(1): 111–24. 521: 517: 510: 508: 504: 499: 495: 490: 485: 481: 477: 473: 466: 464: 462: 458: 453: 447: 439: 437:92-832-2411-6 433: 429: 428: 420: 418: 416: 414: 412: 410: 408: 406: 402: 395: 393: 391: 383: 381: 379: 371: 369: 367: 362: 359: 355: 351: 349: 345: 341: 337: 331: 325: 323: 321: 317: 312: 310: 309:chromosome 14 301: 299: 297: 293: 289: 285: 281: 277: 273: 269: 265: 257: 255: 253: 249: 245: 241: 237: 233: 228: 226: 222: 218: 214: 211: 207: 203: 195: 190: 188: 186: 178: 176: 174: 170: 169:immunoglobins 166: 162: 158: 154: 150: 146: 142: 138: 133: 131: 128:, widespread 127: 119: 117: 116: 112: 108: 104: 100: 96: 92: 88: 84: 80: 77: 73: 65: 61: 58: 56: 52: 49: 48:Prolymphocyte 45: 41: 36: 31: 19: 1686: 1629: 1623:By infection 1504: 1503: 1485: 1446: 1438: 1432: 1410:development/ 1338:Plasmacytoma 1292: 1280: 1266: 1237: 1225: 1219:By infection 1202: 1163: 1100: 1067: 1041: 1010: 1000:naive B cell 998: 959:development/ 858: 834: 823: 773: 769: 759: 735:(1): 19–24. 732: 728: 718: 683: 679: 633: 629: 623: 601:(2): 110–6. 598: 594: 588: 563: 559: 552: 519: 515: 479: 475: 426: 387: 384:Epidemiology 375: 363: 352: 346:, including 340:chlorambucil 332: 329: 316:chromosome 8 313: 305: 261: 229: 204:with single 199: 182: 173:paraproteins 134: 123: 114: 110: 106: 74:is a mature 71: 70: 1505:aggressive: 1478:MF+variants 1017:Mantle cell 1012:mantle zone 378:alemtuzumab 354:Alemtuzumab 202:lymphocytes 141:bone marrow 137:lymph nodes 91:lymph nodes 87:bone marrow 1581:peripheral 1078:Follicular 908:Leukaemias 566:(1): 1–9. 396:References 392:leukemia. 268:neoplastic 248:paracortex 244:lymph node 240:white pulp 210:basophilic 196:Morphology 187:) T-cell. 60:Hematology 1716:Lymphoid+ 1486:indolent: 1469:Cutaneous 1310:Cutaneous 1088:GCB DLBCL 1083:Burkitt's 912:lymphomas 446:cite book 372:Prognosis 326:Treatment 320:trisomy 8 213:cytoplasm 191:Diagnosis 175:present. 161:platelets 55:Specialty 1853:Category 1823:leukemia 1381:leukemia 1376:lymphoma 935:leukemia 930:lymphoma 860:Orphanet 792:15716243 751:10761013 710:26664911 702:11535503 650:11063795 544:22030549 236:red pulp 223:seen in 206:nucleoli 79:leukemia 64:oncology 1811:General 1718:myeloid 1680:T or NK 1651:NK cell 1005:CLL/SLL 841:D015461 830:M9834/3 615:9614908 580:1913594 536:3489482 498:1742486 1655:(most 1631:HTLV-1 1523:Non-MF 1412:marker 1385:(most 1371:T cell 1043:CD11c+ 961:marker 939:(most 925:B cell 790:  749:  708:  700:  648:  613:  578:  542:  534:  496:  434:  390:T cell 278:, and 252:dermis 242:, and 232:spleen 217:nuclei 185:thymic 179:Causes 165:HTLV-1 151:, and 145:spleen 113:, and 101:, and 99:spleen 76:T-cell 1578:Other 1448:CD30+ 1194:CD138 1165:CD20+ 1059:CD79a 865:86871 825:ICD-O 706:S2CID 680:Blood 540:S2CID 476:Blood 149:liver 95:liver 83:blood 1657:CD56 1551:CD30 1530:CD30 1418:TdT+ 1362:T/NK 1227:KSHV 1203:see 1190:CD38 1181:PCDs 1145:CD30 1141:CD15 1116:MALT 1028:CD22 969:TdT+ 946:CD20 941:CD19 836:MeSH 788:PMID 747:PMID 698:PMID 646:PMID 611:PMID 576:PMID 532:PMID 494:PMID 452:link 432:ISBN 288:CD1a 286:and 238:and 208:and 159:and 153:skin 103:skin 1688:EBV 1553:+: 1532:-: 1422:ALL 1397:CD8 1392:CD4 1387:CD3 1282:HIV 1268:HCV 1239:EBV 1143:+, 990:CD5 976:ALL 778:doi 774:123 737:doi 688:doi 638:doi 634:121 603:doi 599:103 568:doi 524:doi 484:doi 296:CD8 292:CD4 284:TdT 280:CD7 276:CD3 272:CD2 1855:: 1420:: 1408:By 1196:+) 1192:+/ 1185:PP 1147:+) 1137:RS 957:By 910:, 863:: 839:: 828:: 786:. 772:. 768:. 745:. 731:. 727:. 704:. 696:. 684:98 682:. 678:. 658:^ 644:. 632:. 609:. 597:. 574:. 564:55 562:. 538:. 530:. 520:64 518:. 506:^ 492:. 480:78 478:. 474:. 460:^ 448:}} 444:{{ 404:^ 322:. 294:+/ 274:, 147:, 143:, 139:, 109:, 97:, 93:, 89:, 85:, 62:, 1754:) 1745:( 1699:) 1695:/ 1691:( 1659:) 1653:/ 1638:) 1634:( 1610:) 1606:( 1460:) 1451:( 1441:) 1437:( 1428:) 1424:( 1399:) 1383:) 1378:, 1374:( 1301:) 1297:( 1289:) 1285:( 1234:) 1230:( 1188:( 1183:/ 1172:) 1168:( 1160:) 1156:( 1139:( 1128:) 1109:( 1104:/ 1095:) 1076:( 1071:/ 1061:+ 1050:) 1046:( 1030:+ 1019:) 1015:( 1007:) 1003:( 992:+ 982:) 978:( 948:) 937:) 932:, 928:( 900:e 893:t 886:v 817:D 794:. 780:: 753:. 739:: 733:5 712:. 690:: 652:. 640:: 617:. 605:: 582:. 570:: 546:. 526:: 500:. 486:: 454:) 440:. 20:)

Index

Leukemia, T-Cell, chronic

Prolymphocyte
Specialty
Hematology
oncology
T-cell
leukemia
blood
bone marrow
lymph nodes
liver
spleen
skin
enlargement of the liver and spleen
enlargement of the lymph nodes
lymph nodes
bone marrow
spleen
liver
skin
red blood cells
platelets
HTLV-1
immunoglobins
paraproteins
thymic
lymphocytes
nucleoli
basophilic

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