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Post-transplant lymphoproliferative disorder

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bone marrow transplants and liver transplants. The highest rates of PTLD are seen with lung and heart transplants, which is primarily due to the need for higher levels of immunosuppression. The incidence of PTLD is highest in the first year after transplantation; roughly 80 percent of cases after transplant occur in the first year. Transplantation of unmatched or mismatched HLA bone marrow also increase the risk of PTLD.
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PTLD is the second most common malignancy that occurs as a complication following solid organ transplantation (skin cancer is the most common). Less commonly, PTLD occurs after hematopoietic stem cell transplantation. The incidence varies by the type of transplantation: the lowest rates are seen with
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Symptoms of PTLD are highly variable and nonspecific, and may include fever, weight loss, night sweats, and fatigue. Symptoms may be similar to those seen in infectious mononucleosis (caused by EBV). Pain or discomfort may result from lymphadenopathy or mass effect from growing tumors. Dysfunction
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The main risk factors for PTLD are the degree of immune suppression and the presence of Epstein-Barr virus. Specifically, higher levels of T cell immunosuppression increase the risk PTLD. Individuals who have never been infected by the Epstein-Barr virus (EBV negative) who receive an organ from a
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Definitive diagnosis is achieved by biopsying the involved tissue, which will reveal lymphoproliferative neoplasia. Most lesions will show malignant B cells, whereas a minority will show T cell neoplasia. CT imaging may show enlarged lymph nodes or a focal mass. PET scan may be helpful in the
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Laboratory findings may show abnormally low white blood cell, red cell counts, and platelet counts. In addition, serum uric acid and lactate dehydrogenase levels may be elevated, while serum calcium levels may be decreased. All of these findings together can suggest tumor lysis syndrome.
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proliferation due to therapeutic immunosuppression after organ transplantation. These patients may develop infectious mononucleosis-like lesions or polyclonal polymorphic B-cell hyperplasia. Some of these B cells may undergo mutations which will render them malignant, giving rise to a
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Neurologic symptoms, such as confusion or focal weakness, which may suggest involvement of the nervous system. This may be evaluated with an MRI of the brain with gadolinium based contrast and lumbar spinal tap with testing of the cerebral spinal fluid for EBV viral levels.
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The presence of respiratory symptoms, such as cough or shortness of breath, in the setting of immunosuppression may suggest infection. Opportunistic infections may present in a similar fashion to PTLD. Evaluation with sputum culture for bacteria,
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donor with prior EBV infection are 24 times more likely to develop PTLD. Similarly, CMV mismatching (with a CMV negative recipient from a CMV positive donor) increases the risk of PTLD.
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in adolescents, which is otherwise asymptomatic in children during their childhood. However, in immunosuppressed transplant patients, the lack of
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evaluation, which may show an increase in metabolic activity (PET avid) lesion, potentially guiding decisions on where to direct biopsies.
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In some patients, the malignant cell clone can become the dominant proliferating cell type, leading to frank lymphoma, a group of B cell
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and may be fatal. A phase 2 study of adoptively transferred EBV-specific T cells demonstrated high efficacy with minimal toxicity.
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further increases the risk of developing post-transplant lymphoproliferative disorder. Such antibodies include
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medication, and can also be treated with addition of anti-viral therapy. In some cases it will progress to
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Polyclonal PTLD may form tumor masses and present with symptoms due to a mass effect, e.g. symptoms of
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may occur in organs affected by PTLD. Lung or heart involvement may result in shortness of breath.
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Haque T, Wilkie GM, Jones MM, Higgins CD, Urquhart G, Wingate P, et al. (August 2007).
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Gottschalk S, Rooney CM, Heslop HE (2005). "Post-transplant lymphoproliferative disorders".
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immunosurveillance can lead to the proliferation of these EBV-infected B-lymphocytes.
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function, and can prevent the control of the B cell proliferation.
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PTLD may spontaneously regress on reduction or cessation of
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Non-mycosis fungoides CD30− cutaneous large T-cell lymphoma
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In immunocompetent patients, Epstein-Barr virus can cause
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Post-transplant lymphoproliferative disorder, pleomorphic
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The disease is an uncontrolled proliferation of B cell
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Unsourced material may be challenged and 196:Depletion of T cells by use of anti-T cell 2243:Jessner lymphocytic infiltrate of the skin 1914: 1845: 1807: 1789:Primary cutaneous follicle center lymphoma 1534:Primary cutaneous follicle center lymphoma 1405: 1394: 1361: 1340: 1326: 1318: 1052: 609: 595: 587: 493: 391:Nourse JP, Jones K, Gandhi MK (May 2011). 88: 46: 29: 467: 408: 285:Learn how and when to remove this message 1769:Primary cutaneous marginal zone lymphoma 1567:Primary cutaneous marginal zone lymphoma 2231:with bandlike and perivascular patterns 2193:Autoimmune lymphoproliferative syndrome 348: 2310:Epstein–Barr virus–associated diseases 2040:Enteropathy-associated T-cell lymphoma 7: 2188:X-linked lymphoproliferative disease 370:10.1146/annurev.med.56.082103.104727 263:adding citations to reliable sources 2144:Large granular lymphocytic leukemia 1764:Intravascular large B-cell lymphoma 995:Canadian Society of Transplantation 990:American Society of Transplantation 397:American Journal of Transplantation 200:in the prevention or treatment of 25: 181:However, calcineurin inhibitors ( 921:United Network for Organ Sharing 906:National Transplant Organization 410:10.1111/j.1600-6143.2011.03499.x 235: 163:, an endogenous, pro-regulatory 1996:CD30+ cutaneous T-cell lymphoma 1035:Organ transplantation in Israel 2226:Cutaneous lymphoid hyperplasia 2218:Cutaneous lymphoid hyperplasia 2077:Adult T-cell leukemia/lymphoma 1955:Adult T-cell leukemia/lymphoma 1894:Anaplastic large-cell lymphoma 1774:Primary cutaneous immunocytoma 1715:Splenic marginal zone lymphoma 1040:Organ transplantation in Japan 1011:Organ transplantation in China 985:American Society of Nephrology 1: 2274:Lymphoproliferative disorders 2184:Lymphoproliferative disorders 2134:Extranodal NK-T-cell lymphoma 2006:Lymphomatoid papulosis type A 1985:Lymphomatoid papulosis type B 1899:Lymphomatoid papulosis type A 1759:Diffuse large B-cell lymphoma 916:Trillium Gift of Life Network 896:National Marrow Donor Program 2166:Acute biphenotypic leukaemia 2055:Subcutaneous T-cell lymphoma 954:Halachic Organ Donor Society 881:Gift of Life Marrow Registry 469:10.1182/blood-2006-12-063008 167:, has also been implicated. 2107:Aggressive NK-cell leukemia 1980:Pleomorphic T-cell lymphoma 1686:Lymphomatoid granulomatosis 959:Kidney Foundation of Canada 2326: 1016:harvesting from Falun Gong 964:National Kidney Foundation 820:Non-heart-beating donation 1360: 1247: 846:Graft-versus-host disease 358:Annual Review of Medicine 159:(EBV). Production of an 106:) is the name given to a 54: 45: 2300:Transplantation medicine 2259:Hematological malignancy 2112:Blastic NK cell lymphoma 1940:Granulomatous slack skin 1696:Classic Hodgkin lymphoma 1595:Classic Hodgkin lymphoma 911:NHS Blood and Transplant 172:infectious mononucleosis 1287:Immunosuppressive drugs 1023:Organ donation in India 155:latently infected with 1307:Frankenstein's monster 1302:Total body irradiation 1028:Gurgaon kidney scandal 886:Human Tissue Authority 324:non-Hodgkin's lymphoma 2138:Angiocentric lymphoma 1728:AIDS-related lymphoma 1552:Splenic marginal zone 1292:Lung allocation score 1229:Jean-Michel Dubernard 618:Organ transplantation 2236:with nodular pattern 1935:Pagetoid reticulosis 1547:marginal zone B-cell 1099:J. Hartwell Harrison 856:Transplant rejection 437:webpath.med.utah.edu 308:Pneumocystis carinii 259:improve this section 202:transplant rejection 1735:Helicobacter pylori 1562:Nodal marginal zone 1489:Hairy cell leukemia 1355:and related disease 1208:AndrĂ© van der Merwe 1192:Richard C. Lillehei 865:Transplant networks 649:Xenotransplantation 644:Autotransplantation 632:Allotransplantation 126:patients following 2279:Lymphoid leukemias 2199:Leukemoid reaction 2035:Angioimmunoblastic 1701:Burkitt's lymphoma 1063:Christiaan Barnard 659:Organs and tissues 561:External resources 157:Epstein–Barr virus 134:Signs and symptoms 67:Epstein–Barr virus 2287: 2286: 2212: 2211: 2152: 2151: 2086: 2085: 2014: 2013: 1930:Mycosis fungoides 1797: 1796: 1655: 1654: 1599:Nodular sclerosis 1514:follicular B cell 1315: 1314: 1297:Machine perfusion 1277:Edmonton protocol 1272:Biomedical tissue 1260: 1259: 1171:Vladimir Demikhov 1073:Adrian Kantrowitz 825:Organ procurement 584: 583: 433:"Hematopathology" 320:immunosuppressant 295: 294: 287: 221:bowel obstruction 216:(muromonab-CD3). 97: 96: 27:Medical condition 16:(Redirected from 2317: 2049:Lennert lymphoma 1915: 1846: 1808: 1673:Primary effusion 1406: 1395: 1362: 1342: 1335: 1328: 1319: 1119:Michael Woodruff 1053: 784:Medical grafting 611: 604: 597: 588: 494: 482: 481: 471: 462:(4): 1123–1131. 447: 441: 440: 429: 423: 422: 412: 388: 382: 381: 353: 290: 283: 279: 276: 270: 239: 231: 128:organ transplant 124:immunosuppressed 93: 92: 50: 30: 21: 2325: 2324: 2320: 2319: 2318: 2316: 2315: 2314: 2290: 2289: 2288: 2283: 2247: 2208: 2170: 2158: 2148: 2116: 2095: 2082: 2059: 2020: 2010: 1959: 1904: 1852: 1850: 1825: 1820: 1814: 1793: 1745: 1651: 1628: 1616: 1572: 1510:germinal center 1494: 1463: 1425: 1401: 1399: 1379: 1374: 1368: 1356: 1346: 1316: 1311: 1256: 1243: 1212: 1196: 1180: 1149: 1123: 1104:John P. 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303: 299: 289: 286: 278: 268: 264: 260: 254: 253: 249: 244:This section 242: 238: 233: 232: 226: 224: 222: 217: 215: 211: 207: 203: 199: 194: 192: 188: 184: 179: 177: 173: 168: 166: 162: 158: 154: 146: 144: 140: 133: 131: 129: 125: 122:occurring in 121: 116: 114: 109: 105: 101: 91: 85: 82: 80: 76: 72: 68: 64: 61: 57: 53: 49: 44: 40: 36: 31: 19: 2127: 2070: 2064:By infection 1945: 1944: 1926: 1887: 1873: 1851:development/ 1779:Plasmacytoma 1733: 1721: 1707: 1690: 1678: 1666: 1660:By infection 1643: 1604: 1541: 1508: 1482: 1451: 1441:naive B cell 1439: 1400:development/ 1161:Fikri Alican 1135:Fikri Alican 850: 568: 544: 536: 521: 509: 459: 455: 445: 436: 427: 400: 396: 386: 364:(1): 29–44. 361: 357: 351: 337: 333: 330:Epidemiology 317: 307: 304: 300: 296: 281: 275:January 2020 272: 257:Please help 245: 218: 195: 180: 169: 150: 141: 137: 117: 103: 99: 98: 1946:aggressive: 1919:MF+variants 1458:Mantle cell 1453:mantle zone 1239:Bruce Reitz 891:LifeSharers 869:departments 830:Organ trade 672:Bone marrow 187:ciclosporin 153:lymphocytes 38:Other names 2294:Categories 2022:peripheral 1519:Follicular 1349:Leukaemias 742:islet cell 712:Heart–lung 546:DiseasesDB 343:References 198:antibodies 183:tacrolimus 84:Immunology 56:Micrograph 2157:Lymphoid+ 1927:indolent: 1910:Cutaneous 1751:Cutaneous 1529:GCB DLBCL 1524:Burkitt's 1353:lymphomas 1004:Countries 717:Intestine 570:eMedicine 541:: M9970/1 314:Treatment 246:does not 227:Diagnosis 120:lymphomas 79:Specialty 71:HES stain 60:bronchial 2305:Lymphoma 2264:leukemia 1822:leukemia 1817:lymphoma 1376:leukemia 1371:lymphoma 1282:Eye bank 1185:Pancreas 969:ORGANIZE 930:Advocacy 737:Pancreas 575:ped/2851 518:: D47.Z1 478:17468341 419:21521464 378:15660500 165:cytokine 113:lymphoma 2252:General 2159:myeloid 2121:T or NK 2092:NK cell 1446:CLL/SLL 682:Corneal 267:removed 252:sources 2096:(most 2072:HTLV-1 1964:Non-MF 1853:marker 1826:(most 1812:T cell 1484:CD11c+ 1402:marker 1380:(most 1366:B cell 1092:Kidney 1049:People 901:NOD-Lb 774:Vagina 769:Uterus 764:Thymus 759:Spleen 722:Kidney 532:238.77 476:  417:  376:  191:T cell 176:T-cell 147:Causes 108:B cell 86:  63:biopsy 2019:Other 1889:CD30+ 1635:CD138 1606:CD20+ 1500:CD79a 1217:Other 1201:Penis 1128:Liver 1056:Heart 749:Penis 727:Liver 707:Heart 677:Brain 625:Types 551:34154 538:ICD-O 456:Blood 58:of a 2098:CD56 1992:CD30 1971:CD30 1859:TdT+ 1803:T/NK 1668:KSHV 1644:see 1631:CD38 1622:PCDs 1586:CD30 1582:CD15 1557:MALT 1469:CD22 1410:TdT+ 1387:CD20 1382:CD19 1154:Lung 949:DKMS 754:Skin 732:Lung 702:Head 697:Hand 692:Face 667:Bone 637:ABOi 527:9-CM 474:PMID 415:PMID 374:PMID 250:any 248:cite 214:OKT3 212:and 185:and 104:PTLD 41:PTLD 2129:EBV 1994:+: 1973:-: 1863:ALL 1838:CD8 1833:CD4 1828:CD3 1723:HIV 1709:HCV 1680:EBV 1584:+, 1431:CD5 1417:ALL 687:Eye 523:ICD 511:ICD 464:doi 460:110 405:doi 366:doi 261:by 210:ALG 206:ATG 2296:: 1861:: 1849:By 1637:+) 1633:+/ 1626:PP 1588:+) 1578:RS 1398:By 1351:, 573:: 549:: 530:: 515:10 472:. 458:. 454:. 435:. 413:. 401:11 399:. 395:. 372:. 362:56 360:. 208:, 130:. 115:. 69:. 2195:) 2186:( 2140:) 2136:/ 2132:( 2100:) 2094:/ 2079:) 2075:( 2051:) 2047:( 1901:) 1892:( 1882:) 1878:( 1869:) 1865:( 1840:) 1824:) 1819:, 1815:( 1742:) 1738:( 1730:) 1726:( 1675:) 1671:( 1629:( 1624:/ 1613:) 1609:( 1601:) 1597:( 1580:( 1569:) 1550:( 1545:/ 1536:) 1517:( 1512:/ 1502:+ 1491:) 1487:( 1471:+ 1460:) 1456:( 1448:) 1444:( 1433:+ 1423:) 1419:( 1389:) 1378:) 1373:, 1369:( 1341:e 1334:t 1327:v 610:e 603:t 596:v 525:- 513:- 503:D 480:. 466:: 439:. 421:. 407:: 380:. 368:: 288:) 282:( 277:) 273:( 269:. 255:. 102:( 73:. 20:)

Index

Post-transplant lymphoproliferative disorder, pleomorphic

Micrograph
bronchial
biopsy
Epstein–Barr virus
HES stain
Specialty
Immunology
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B cell
lymphoma
lymphomas
immunosuppressed
organ transplant
lymphocytes
Epstein–Barr virus
interleukin-10
cytokine
infectious mononucleosis
T-cell
tacrolimus
ciclosporin
T cell
antibodies
transplant rejection
ATG
ALG
OKT3
bowel obstruction

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