96:
58:
1455:. Dosage regimens are selected on the basis of patient tolerance. Hematological response rates to the dexamethasone/melphalan regimens have been reported to be in the 80% range with neurological response rates approaching 100%. Patients successfully treated with the high-dose dexamethasone/melphalan regimen have been further treated with
1434:
monitored every 2–3 months for symptoms and disease progression. Otherwise, treatment is divided based on the local versus systemic spread of its clonal plasma cells. Patients with one or two plasmacytoma bone lesions and no clonal plasma cells in their bone marrow biopsy specimens are treated by surgical removal or
1510:). A double blind study of 25 POEMS syndrome patients found significantly better results (VEGF reduction, neuromuscular function improvement, quality of life improvement) in patients treated with thalidomide plus dexamethasone compared to patients treated with a thalidomide placebo plus dexamethasone.
510:
The signs and symptoms of POEMS syndrome are highly variable. This often leads to long delays (e.g. 13–18 months) between the onset of initial symptoms and diagnosis. In addition to the signs and symptoms indicated by the POEMS acronym, the PEST acronym is used to describe some of the other signs and
1438:
of their tumors. These treatments can relieve many of the syndromes clinical manifestations including neuropathies, have a 10-year overall survival of 70% and a 6-year progression-free survival of 62%. Patients with >2 plasmacytoma bone lesions and/or increases in bone marrow clonal plasma cells
1405:
cytokine and have an inferior overall survival compared to POEMS syndrome patients. Treatment of patients with this POEMS syndrome variant who have evidence of bone lesions and/or myeloma proteins are the same as those for POEMS syndrome patients. In the absence of these features, treatment with
1433:
treatment regimens are tailored to treat the clinical manifestations and prognosis for the rate of progression of the POEMS syndrome in each patient. In rare cases, patients may have minimal or no symptoms at presentation or after successful treatment of their disorder. These patients may be
2223:
2208:
2193:
931:
with sclerotic rims, or a mixture of the two with a soup-bubble look. About half of the patients have a single bone lesion, whereas the other half have several lesions. The most prevalent locations for bone lesions are the
2345:
1478:
to treat POEMS syndrome patients. While the mechanism of action of these immunomodulators are not clear, they do inhibit the production of cytokines suspected of contributing to POEMS syndrome such as
1588:
Kaushik M, Pulido JS, Abreu R, Amselem L, Dispenzieri A (2011). "Ocular findings in patients with polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes syndrome".
2485:
2338:
1459:. In 59 patients treated with the chemotherapy/transplantation regimen, the Mayo Clinic reported progression-free survival rates of 98%, 94%, and 75% at 1, 2, and 5 years, respectively.
1401:
should not be diagnosed as having POEMS syndrome. They are better classified as having
Castleman disease variant of POEMS syndrome. These patients may exhibit high blood levels of the
1537:, first described the combination of osteosclerotic myeloma, polyneuropathy and various unusual features (such as pigmentation and clubbing) in two patients aged 54 and 67, in 1956.
721:
The diagnosis of POEMS syndrome is based on meeting its two mandatory criteria, meeting at least one of its 3 other major criteria, and meeting at least one of its 6 minor criteria.
2331:
1525:
suspected to be the result of overly rapid lowering of VEGF levels. It therefore remains doubtful as to whether this will become part of standard treatment for POEMS syndrome.
901:
can occur in CD patients with or without POEMS syndrome. CD and POEMS tend to overlap, with roughly 15–24% of POEMS syndrome patients also having CD, with the majority having
701:. Nonetheless, it seems likely that some of these paraneoplastic factors, operating individually, make a major contribution to certain features of the disease. For example,
2393:
629:, and venous sinus thrombosis. Some features have been observed in patients with POEMS syndrome but are not yet certain to form part of the syndrome itself. These include
241:
225:
2955:
2478:
2734:
2471:
2541:
626:
276:
2536:
1393:
Patients diagnosed as having
Castleman disease but also exhibiting many of the symptoms and signs of POEMS syndrome but lacking evidence of a
1373:
to myeloma proteins, other tests can give abnormal results supporting the diagnosis of POEMS syndrome. These included raised blood levels of
51:
Crow–Fukase syndrome, Osteosclerotic myeloma, PEP syndrome, Polyneuropathy-endocrinopathy-plasma cell dyscrasia syndrome, Takatsuki syndrome.
1456:
2636:
158:
673:
may underlie some, but are insufficient to explain all, of the multi-organ features of the disease. It is suggested that various other
665:
have been described, the exact mechanism behind its development, progression, and manifestations remain elusive. Overproduction of the
1197:
1014:
1006:
1002:
998:
962:
957:
702:
678:
670:
588:
2749:
2674:
2774:
2408:
245:
2739:
2945:
1448:
502:
POEMS syndrome typically begins in middle age – the average age at onset is 50 – and affects up to twice as many men as women.
2836:
709:, would seem likely to be the major contributor to the pathologic hyper-vascularization changes seem in many tissues, such as
261:
2684:
1370:
887:
2641:
2531:
2744:
2354:
2626:
2940:
2864:
2656:
1507:
1463:
1342:
744:
617:) is the most common ocular sign of POEMS syndrome, occurring in ≥29% of cases. Less frequent ocular findings include
2800:
2596:
2551:
2699:
2606:
2427:
2415:
2795:
2679:
1896:
2754:
2591:
2503:
2238:
573:
162:
2960:
2950:
2664:
1522:
1173:
1134:. Extravascular volume overload can cause significant morbidity and corresponds with a lower survival rate.
662:
449:
182:
153:
28:
2669:
2494:
2444:
861:
785:
780:
759:
is often the first trait, and it may be the only initial symptom. Clinical examination may show
577:
480:. Many of the signs and symptoms in POEMS syndrome are due at least in part to the release of an aberrant
441:
220:
166:
2311:
2903:
2891:
2875:
2847:
2571:
2511:
2366:
1394:
1247:
1177:
1030:
898:
789:
756:
618:
614:
427:
2202:
2859:
2843:
2831:
2819:
2387:
1181:
1131:
1115:
1097:
857:
531:
358:
141:
95:
2726:
2714:
2420:
2227:
1518:
1300:
824:
406:
1287:
is often one of the earliest signs of POEMS disease and is usually bilateral. Patients tend to be
1013:
cells and causes an increase in vascular permeability that is both fast and reversible. Increased
2561:
2521:
2300:
1747:
1669:
1561:
1362:
1235:
966:
622:
581:
253:
170:
145:
104:
677:
produced by the clonal plasma cells, perhaps working in concert with each other as well as with
2323:
57:
2824:
2611:
2601:
2526:
2404:
2249:
2161:
2112:
2071:
2015:
1971:
1926:
1918:
1825:
1807:
1739:
1731:
1661:
1605:
1219:
1193:
1165:
1157:
1143:
1038:
883:
878:
768:
592:
210:
121:
84:
1562:"POEMS syndrome — About the Disease — Genetic and Rare Diseases Information Center"
2908:
2884:
2764:
2759:
2621:
2566:
2452:
2399:
2383:
2151:
2143:
2102:
2061:
2053:
2005:
1963:
1908:
1815:
1799:
1721:
1653:
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1499:
1354:
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1326:
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539:
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399:
1868:
1102:
80% of POEMS patients are reported to have extravascular volume overload including,
2917:
2769:
2516:
2432:
2156:
2131:
2066:
2041:
1820:
1440:
1227:
1215:
1209:
1026:
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913:
850:
735:
638:
553:
546:
420:
413:
392:
378:
351:
272:
257:
186:
113:
2232:
1418:, a monoclonal antibody preparation directed against interleukin-6, may be justified.
1307:
has been noted in 29–64% of patients and is associated with an unfavorable prognosis.
2934:
2583:
1751:
1503:
1487:
1475:
1444:
1402:
1267:
1147:
994:
970:
894:
842:
835:
694:
630:
473:
1913:
1673:
17:
2689:
2463:
2378:
2289:
1513:
Since VEGF plays a central role in the symptoms of POEMS syndrome, some have tried
1471:
1435:
1378:
1338:
1330:
1320:
1288:
1259:
1243:
1185:
1161:
1073:
1065:
1061:
1055:
1034:
1018:
928:
706:
565:
561:
385:
370:
348:
297:
280:
137:
129:
125:
72:
2243:
1601:
2709:
1514:
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1430:
1398:
1382:
1366:
1304:
1284:
1279:
1263:
1231:
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990:
973:
are significantly higher in POEMS patients and correlate with illness activity.
846:
838:
828:
817:
813:
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797:
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752:
646:
605:
512:
496:
362:
305:
293:
249:
233:
109:
76:
2254:
2010:
1993:
1710:"POEMS Syndrome: 2019 Update on diagnosis, risk-stratification, and management"
1242:. POEMS syndrome can also cause Vascular-type skin changes including
2704:
2217:
2057:
1967:
1657:
1521:
directed against VEGF. While some reports were positive, others have reported
1415:
1251:
1223:
1189:
982:
974:
890:
710:
634:
610:
569:
493:
301:
288:
174:
133:
1922:
1811:
1735:
1156:
Approximately 84% of POEMS syndrome patients have characteristics of several
603:
The more common features of the disease are summarized in the acronym POEMS:
2284:
2260:
1452:
1407:
748:
557:
437:
313:
178:
69:
2165:
2147:
2116:
2107:
2075:
2019:
1975:
1930:
1829:
1803:
1743:
1665:
1609:
2693:
2556:
2357:
2306:
1495:
1410:, a monoclonal antibody preparation directed against B cells bearing the
1292:
1239:
986:
945:
941:
764:
740:
686:
674:
650:
585:
489:
481:
477:
469:
461:
445:
354:
89:
2185:
1644:
Warsame R, Yanamandra U, Kapoor P (2017). "POEMS Syndrome: an Enigma".
1534:
1491:
1483:
1358:
1296:
1119:
1107:
1089:
902:
760:
698:
523:
457:
453:
374:
366:
1726:
1709:
1439:
are treated with a low-dose or high-dose chemotherapy regimen, i.e. a
689:
detected in, and suspected of contributing to, POEMS syndrome include
2212:
2197:
933:
920:
916:
853:
831:
820:
1353:
In addition to tests corresponding to the above findings, such as
1022:
642:
535:
117:
2265:
2295:
1479:
1411:
1374:
978:
804:
become altered. POEMS syndrome is often associated with an
690:
2467:
2327:
1869:"POEMS Syndrome — Symptoms, Causes, Treatment — NORD"
591:, and an overlap with the signs and symptoms of multicentric
1017:
could explain several clinical features of POEMS, including
685:, mediate many of the features of POEMS syndrome. The other
1792:
Mediterranean
Journal of Hematology and Infectious Diseases
1214:
About 90–100% of those with POEMS syndrome will experience
564:). Other features of the disease include a tendency toward
2089:
Samaras P, Bauer S, Stenner-Liewen F, et al. (2007).
75:
continue to produce an antibody that can be detected as a
1787:
444:
caused by the invasion and destruction of tissues by the
226:
Chronic inflammatory demyelinating polyradiculoneuropathy
767:, and sensory impairment affecting both large and small
1855:"POEMS Syndrome: Background, Pathophysiology, Etiology"
1992:
Dispenzieri A, Kyle RA, Lacy MQ, et al. (2003).
919:
have been documented in around 95% of patients. Bone
755:
is the most frequent presentation of POEMS syndrome.
2175:
2809:
2788:
2725:
2655:
2582:
2502:
2442:
2365:
2275:
2179:
1994:"POEMS syndrome: definitions and long-term outcome"
1076:is usually minimal, and bulky disease is uncommon.
322:
312:
287:
268:
219:
209:
201:
193:
152:
103:
83:
47:
42:
2040:Brown, Rachel; Ginsberg, Lionel (1 January 2019).
448:' cells. Signs and symptoms of a cancer causing a
242:monoclonal gammopathy of undetermined significance
2035:
2033:
2031:
2029:
1508:immunomodulatory imide drug's mechanism of action
1001:; both IL1 and IL6 have been proven to increase
1639:
1226:. Other skin abnormalities include thickening,
2091:"Treatment of POEMS syndrome with bevacizumab"
1987:
1985:
1949:
1947:
1637:
1635:
1633:
1631:
1629:
1627:
1625:
1623:
1621:
1619:
1583:
1581:
1556:
1554:
1552:
1550:
1333:develops in 15% of patients. Those with
823:, patients with POEMS syndrome often have few
2479:
2339:
1954:Castillo JJ (2016). "Plasma Cell Disorders".
8:
1462:Other treatment regimens are being studied.
27:"POEMS" redirects here. For other uses, see
2486:
2472:
2464:
2346:
2332:
2324:
2176:
1849:
1847:
1845:
1843:
1841:
1839:
1192:tends to be common in women. The cause of
834:may be normal. Other common findings were
94:
39:
2155:
2106:
2065:
2009:
1912:
1819:
1725:
1303:, and gradual visual field constriction.
589:vascular endothelial growth factor (VEGF)
511:symptoms of the disease. PEST stands for
1890:
1888:
1781:
1060:POEMS syndrome has been associated with
613:) often but not always due to increased
1779:
1777:
1775:
1773:
1771:
1769:
1767:
1765:
1763:
1761:
1546:
1371:plasma or serum protein electrophoresis
631:a predisposition to forming blood clots
341:Crow–Fukase syndrome, Takatsuki disease
234:nodular localized cutaneous amyloidosis
2956:Syndromes affecting the nervous system
1901:Hematology Am Soc Hematol Educ Program
1895:Dispenzieri, Angela (1 January 2005).
1703:
1646:Current Hematologic Malignancy Reports
827:. In patients with localized illness,
2132:"Peripheral neuritis in myelomatosis"
1701:
1699:
1697:
1695:
1693:
1691:
1689:
1687:
1685:
1683:
1345:before POEMS syndrome is identified.
1218:. The most common manifestations are
258:amyloid light chains (AL) amyloidosis
7:
1457:autologous stem cell transplantation
1708:Dispenzieri, Angela (23 May 2019).
1474:have been used in combination with
1037:permeability of blood vessels with
893:disorder characterized by elevated
841:and clustering, as well as unusual
1786:Nozza, Andrea (1 September 2017).
1343:chronic myeloproliferative disease
958:Vascular endothelial growth factor
952:Vascular endothelial growth factor
275:, low-dose alkylators, peripheral
246:waldenstrom hypergammaglobulinemia
25:
2735:Bannayan–Riley–Ruvalcaba syndrome
2042:"POEMS syndrome: clinical update"
705:, given its ability to stimulate
584:), very high blood levels of the
1907:(1). ash publications: 360–357.
1498:to increase their production of
1295:, brief obscurations of vision,
1291:, however, they may report
661:While the main features of this
582:impaired lung diffusion capacity
369:for some of the disease's major
56:
1914:10.1182/asheducation-2005.1.360
436:The signs and symptoms of most
277:blood stem cell transplantation
2542:Bonnet–Dechaume–Blanc syndrome
1714:American Journal of Hematology
713:, affected by POEMS syndrome.
522:xtravascular volume overload (
318:Median survival of 13.8 years.
1:
2537:Sakati–Nyhan–Tisdale syndrome
2409:Waldenström macroglobulinemia
1080:Extravascular volume overload
68:is usually diagnosed because
2775:Tatton-Brown–Rahman syndrome
2745:Benign symmetric lipomatosis
1602:10.1016/j.ophtha.2010.08.013
1464:Immunomodulatory imide drugs
1341:are often misdiagnosed with
1180:. Men frequently experience
183:capillary leak-like syndrome
2865:Branchio-oto-renal syndrome
2740:Beckwith–Wiedemann syndrome
1788:"Poems Syndrome: An Update"
1426:As reported by Dispenzieri
1325:50% of POEMS patients have
472:and/or the response of the
452:result from the release of
2977:
2837:Zimmermann–Laband syndrome
2789:Laurence–Moon–Bardet–Biedl
2750:Klippel–Trénaunay syndrome
2700:Caudal regression syndrome
2675:Klippel–Trénaunay syndrome
2637:Smith–Lemli–Opitz syndrome
2607:Cornelia de Lange syndrome
2011:10.1182/blood-2002-07-2299
1314:
1277:
1207:
1141:
1083:
1053:
955:
876:
778:
733:
572:, abnormal lung function (
197:5th to 6th decade of life.
26:
2685:Rubinstein–Taybi syndrome
2058:10.1007/s00415-018-9110-6
1968:10.1016/j.pop.2016.07.002
1658:10.1007/s11899-017-0367-0
1566:rarediseases.info.nih.gov
1311:Hematological alterations
1196:is unknown, however
623:serous macular detachment
556:(i.e. increased in blood
171:cardiorespiratory failure
64:
55:
2755:Neurofibromatosis type I
2642:Snyder–Robinson syndrome
2592:1q21.1 deletion syndrome
2532:Saethre–Chotzen syndrome
1126:are more prevalent than
1033:, as well as increasing
849:invade normal marrow in
769:fibre sensory modalities
641:(systolic dysfunction),
627:infiltrative orbitopathy
574:restrictive lung disease
163:restrictive lung disease
2665:Adducted thumb syndrome
2627:Silver–Russell syndrome
1533:R. S. Crow, working in
1523:capillary leak syndrome
1072:in 50–78% of patients.
872:
825:monoclonal plasma cells
790:monoclonal gammopathies
745:sensorimotor neuropathy
450:paraneoplastic syndrome
365:. The name POEMS is an
262:guillain-Barré syndrome
34:Paraneoplastic syndrome
29:Poetry (disambiguation)
2946:Neurological disorders
2801:Laurence–Moon syndrome
2597:Aarskog–Scott syndrome
2552:Baller–Gerold syndrome
2495:Congenital abnormality
2445:hypergammaglobulinemia
2148:10.1136/bmj.2.4996.802
2108:10.3324/haematol.11315
1804:10.4084/mjhid.2017.051
1389:Differential diagnosis
1164:is the most prevalent
909:Sclerotic bone lesions
845:appearances. Atypical
786:Plasma cell dyscrasias
781:Plasma cell dyscrasias
743:, distal, symmetrical
707:blood vessel formation
663:paraneoplastic disease
578:pulmonary hypertension
534:, and lower extremity
337:osteosclerotic myeloma
221:Differential diagnosis
167:pulmonary hypertension
2796:Bardet–Biedl syndrome
2680:Nail–patella syndrome
2572:Pierre Robin sequence
2512:Acrocephalosyndactyly
1395:peripheral neuropathy
1208:Further information:
1178:adrenal insufficiency
1132:pericardial effusions
1084:Further information:
775:Plasma cell dyscrasia
619:cystoid macular edema
615:intracranial pressure
543:clerotic bone lesions
488:, as well as certain
175:progressive inanition
2727:Overgrowth syndromes
2388:Plasma cell leukemia
2046:Journal of Neurology
1256:Raynaud's phenomenon
1116:pericardial effusion
1098:Pericardial effusion
788:are a group of
570:blood clot formation
532:pericardial effusion
431:lasma cell dyscrasia
142:erectile dysfunction
18:Crow–Fukase syndrome
2715:VACTERL association
2433:Primary amyloidosis
2421:Heavy chain disease
2355:Immunoproliferative
1519:monoclonal antibody
1349:Laboratory findings
1234:, and infiltrating
884:Castleman's disease
873:Castleman's disease
2941:Endocrine diseases
2670:Holt–Oram syndrome
2562:Goldenhar syndrome
2522:Carpenter syndrome
2276:External resources
1857:. 16 October 2021.
1363:bone marrow biopsy
1236:livedo reticularis
1230:, acquired facial
1174:glucose metabolism
1166:endocrine disorder
1027:skin abnormalities
725:Mandatory criteria
506:Signs and symptoms
468:by the syndrome's
254:raynaud phenomenon
215:Clinical Criteria.
159:Multiorgan failure
146:testicular atrophy
2928:
2927:
2825:Feingold syndrome
2612:Dubowitz syndrome
2602:Cockayne syndrome
2527:Pfeiffer syndrome
2461:
2460:
2405:Macroglobulinemia
2321:
2320:
1727:10.1002/ajh.25495
1449:alkylating agents
1365:to detect clonal
1220:hyperpigmentation
1200:may have a role.
1170:thyroid anomalies
1144:Endocrine disease
1009:normally targets
879:Castleman disease
609:(swelling of the
593:Castleman disease
440:are due to their
330:
329:
211:Diagnostic method
122:hyperpigmentation
37:Medical condition
16:(Redirected from
2968:
2909:Donohue syndrome
2885:Timothy syndrome
2765:Proteus syndrome
2760:Perlman syndrome
2622:Robinow syndrome
2567:Moebius syndrome
2488:
2481:
2474:
2465:
2453:Cryoglobulinemia
2384:Multiple myeloma
2348:
2341:
2334:
2325:
2177:
2170:
2169:
2159:
2130:Crow RS (1956).
2127:
2121:
2120:
2110:
2086:
2080:
2079:
2069:
2037:
2024:
2023:
2013:
1989:
1980:
1979:
1951:
1942:
1941:
1939:
1937:
1916:
1897:"POEMS Syndrome"
1892:
1883:
1882:
1880:
1879:
1873:rarediseases.org
1865:
1859:
1858:
1851:
1834:
1833:
1823:
1783:
1756:
1755:
1729:
1705:
1678:
1677:
1641:
1614:
1613:
1585:
1576:
1575:
1573:
1572:
1558:
1500:interferon gamma
1357:for neuropathy,
1158:endocrinopathies
1152:Endocrine system
1124:peripheral edema
1112:pleural effusion
1104:peripheral edema
1094:Pleural effusion
1086:Peripheral edema
816:dysfunction. On
792:in which normal
683:myeloma proteins
528:pleural effusion
470:neoplastic cells
456:factors such as
238:multiple myeloma
230:cryoglobulinemia
99:
98:
66:Multiple myeloma
60:
40:
21:
2976:
2975:
2971:
2970:
2969:
2967:
2966:
2965:
2931:
2930:
2929:
2924:
2897:Marfan syndrome
2881:Keutel syndrome
2869:CHARGE syndrome
2853:Fraser syndrome
2811:
2810:Combined/other,
2805:
2784:
2780:Weaver syndrome
2721:
2651:
2647:Turner syndrome
2632:Seckel syndrome
2617:Noonan syndrome
2578:
2498:
2492:
2462:
2457:
2438:
2361:
2352:
2322:
2317:
2316:
2271:
2270:
2188:
2174:
2173:
2142:(4996): 802–4.
2129:
2128:
2124:
2088:
2087:
2083:
2039:
2038:
2027:
2004:(7): 2496–506.
1991:
1990:
1983:
1953:
1952:
1945:
1935:
1933:
1894:
1893:
1886:
1877:
1875:
1867:
1866:
1862:
1853:
1852:
1837:
1798:(1): e2017051.
1785:
1784:
1759:
1707:
1706:
1681:
1643:
1642:
1617:
1587:
1586:
1579:
1570:
1568:
1560:
1559:
1548:
1543:
1531:
1424:
1391:
1351:
1323:
1317:Thrombocythemia
1315:Main articles:
1313:
1282:
1276:
1212:
1206:
1154:
1140:
1100:
1082:
1070:lymphadenopathy
1058:
1052:
1047:
960:
954:
911:
905:vascular type.
886:(CD) is a rare
881:
875:
870:
812:lambda limited
783:
777:
738:
732:
727:
719:
667:myeloma protein
659:
601:
599:Common features
562:red blood cells
508:
486:myeloma protein
466:immunoglobulins
273:Corticosteroids
93:
38:
35:
32:
23:
22:
15:
12:
11:
5:
2974:
2972:
2964:
2963:
2961:Genodermatoses
2958:
2953:
2951:Rare syndromes
2948:
2943:
2933:
2932:
2926:
2925:
2923:
2922:
2921:
2920:
2918:Fryns syndrome
2912:
2900:
2888:
2872:
2856:
2840:
2828:
2815:
2813:
2807:
2806:
2804:
2803:
2798:
2792:
2790:
2786:
2785:
2783:
2782:
2777:
2772:
2770:Sotos syndrome
2767:
2762:
2757:
2752:
2747:
2742:
2737:
2731:
2729:
2723:
2722:
2720:
2719:
2718:
2717:
2712:
2707:
2702:
2687:
2682:
2677:
2672:
2667:
2661:
2659:
2653:
2652:
2650:
2649:
2644:
2639:
2634:
2629:
2624:
2619:
2614:
2609:
2604:
2599:
2594:
2588:
2586:
2580:
2579:
2577:
2576:
2575:
2574:
2569:
2564:
2559:
2554:
2546:
2545:
2544:
2539:
2534:
2529:
2524:
2519:
2517:Apert syndrome
2508:
2506:
2500:
2499:
2493:
2491:
2490:
2483:
2476:
2468:
2459:
2458:
2456:
2455:
2449:
2447:
2440:
2439:
2437:
2436:
2424:
2412:
2396:
2391:
2381:
2375:
2373:
2363:
2362:
2358:immunoglobulin
2353:
2351:
2350:
2343:
2336:
2328:
2319:
2318:
2315:
2314:
2303:
2301:POEMS syndrome
2292:
2280:
2279:
2277:
2273:
2272:
2269:
2268:
2257:
2246:
2235:
2220:
2205:
2189:
2184:
2183:
2181:
2180:Classification
2172:
2171:
2122:
2101:(10): 1438–9.
2081:
2052:(1): 268–277.
2025:
1981:
1962:(4): 677–691.
1943:
1884:
1860:
1835:
1757:
1720:(7): 812–827.
1679:
1615:
1577:
1545:
1544:
1542:
1539:
1530:
1527:
1490:and stimulate
1441:corticosteroid
1423:
1420:
1390:
1387:
1350:
1347:
1339:erythrocytosis
1335:thrombocytosis
1327:thrombocytosis
1312:
1309:
1278:Main article:
1275:
1272:
1228:hypertrichosis
1210:Skin condition
1205:
1202:
1194:endocrinopathy
1168:, followed by
1139:
1138:Endocrinopathy
1136:
1081:
1078:
1054:Main article:
1051:
1048:
1046:
1045:Minor criteria
1043:
995:megakaryocytes
956:Main article:
953:
950:
914:Osteosclerotic
910:
907:
877:Main article:
874:
871:
869:
868:Major criteria
866:
851:osteosclerotic
779:Main article:
776:
773:
761:distal wasting
736:Polyneuropathy
734:Main article:
731:
730:Polyneuropathy
728:
726:
723:
718:
715:
691:interleukin 1β
658:
655:
639:cardiomyopathy
600:
597:
554:erythrocytosis
518:, evidence of
507:
504:
482:immunoglobulin
412:), as is PEP (
403:yeloma protein
352:paraneoplastic
333:POEMS syndrome
328:
327:
324:
320:
319:
316:
310:
309:
291:
285:
284:
270:
266:
265:
223:
217:
216:
213:
207:
206:
203:
199:
198:
195:
191:
190:
156:
150:
149:
114:polyneuropathy
107:
101:
100:
87:
81:
80:
62:
61:
53:
52:
49:
45:
44:
43:POEMS syndrome
36:
33:
24:
14:
13:
10:
9:
6:
4:
3:
2:
2973:
2962:
2959:
2957:
2954:
2952:
2949:
2947:
2944:
2942:
2939:
2938:
2936:
2919:
2916:
2915:
2913:
2910:
2906:
2905:
2901:
2898:
2894:
2893:
2889:
2886:
2882:
2878:
2877:
2873:
2870:
2866:
2862:
2861:
2857:
2854:
2850:
2849:
2845:
2841:
2838:
2834:
2833:
2829:
2826:
2822:
2821:
2817:
2816:
2814:
2808:
2802:
2799:
2797:
2794:
2793:
2791:
2787:
2781:
2778:
2776:
2773:
2771:
2768:
2766:
2763:
2761:
2758:
2756:
2753:
2751:
2748:
2746:
2743:
2741:
2738:
2736:
2733:
2732:
2730:
2728:
2724:
2716:
2713:
2711:
2708:
2706:
2703:
2701:
2698:
2697:
2695:
2691:
2688:
2686:
2683:
2681:
2678:
2676:
2673:
2671:
2668:
2666:
2663:
2662:
2660:
2658:
2654:
2648:
2645:
2643:
2640:
2638:
2635:
2633:
2630:
2628:
2625:
2623:
2620:
2618:
2615:
2613:
2610:
2608:
2605:
2603:
2600:
2598:
2595:
2593:
2590:
2589:
2587:
2585:
2584:Short stature
2581:
2573:
2570:
2568:
2565:
2563:
2560:
2558:
2555:
2553:
2550:
2549:
2547:
2543:
2540:
2538:
2535:
2533:
2530:
2528:
2525:
2523:
2520:
2518:
2515:
2514:
2513:
2510:
2509:
2507:
2505:
2501:
2496:
2489:
2484:
2482:
2477:
2475:
2470:
2469:
2466:
2454:
2451:
2450:
2448:
2446:
2441:
2434:
2430:
2429:
2425:
2422:
2418:
2417:
2413:
2410:
2406:
2402:
2401:
2397:
2395:
2392:
2389:
2385:
2382:
2380:
2377:
2376:
2374:
2372:
2368:
2364:
2359:
2356:
2349:
2344:
2342:
2337:
2335:
2330:
2329:
2326:
2313:
2309:
2308:
2304:
2302:
2298:
2297:
2293:
2291:
2287:
2286:
2282:
2281:
2278:
2274:
2267:
2263:
2262:
2258:
2256:
2252:
2251:
2247:
2245:
2241:
2240:
2236:
2234:
2230:
2229:
2225:
2221:
2219:
2215:
2214:
2210:
2206:
2204:
2200:
2199:
2195:
2191:
2190:
2187:
2182:
2178:
2167:
2163:
2158:
2153:
2149:
2145:
2141:
2137:
2133:
2126:
2123:
2118:
2114:
2109:
2104:
2100:
2096:
2095:Haematologica
2092:
2085:
2082:
2077:
2073:
2068:
2063:
2059:
2055:
2051:
2047:
2043:
2036:
2034:
2032:
2030:
2026:
2021:
2017:
2012:
2007:
2003:
1999:
1995:
1988:
1986:
1982:
1977:
1973:
1969:
1965:
1961:
1957:
1950:
1948:
1944:
1932:
1928:
1924:
1920:
1915:
1910:
1906:
1902:
1898:
1891:
1889:
1885:
1874:
1870:
1864:
1861:
1856:
1850:
1848:
1846:
1844:
1842:
1840:
1836:
1831:
1827:
1822:
1817:
1813:
1809:
1805:
1801:
1797:
1793:
1789:
1782:
1780:
1778:
1776:
1774:
1772:
1770:
1768:
1766:
1764:
1762:
1758:
1753:
1749:
1745:
1741:
1737:
1733:
1728:
1723:
1719:
1715:
1711:
1704:
1702:
1700:
1698:
1696:
1694:
1692:
1690:
1688:
1686:
1684:
1680:
1675:
1671:
1667:
1663:
1659:
1655:
1651:
1647:
1640:
1638:
1636:
1634:
1632:
1630:
1628:
1626:
1624:
1622:
1620:
1616:
1611:
1607:
1603:
1599:
1596:(4): 778–82.
1595:
1591:
1590:Ophthalmology
1584:
1582:
1578:
1567:
1563:
1557:
1555:
1553:
1551:
1547:
1540:
1538:
1536:
1528:
1526:
1524:
1520:
1516:
1511:
1509:
1505:
1504:interleukin 2
1501:
1497:
1493:
1489:
1485:
1481:
1477:
1476:dexamethasone
1473:
1469:
1465:
1460:
1458:
1454:
1450:
1446:
1445:dexamethasone
1442:
1437:
1432:
1429:
1421:
1419:
1417:
1413:
1409:
1404:
1403:interleukin-6
1400:
1396:
1388:
1386:
1384:
1380:
1376:
1372:
1368:
1364:
1360:
1356:
1348:
1346:
1344:
1340:
1336:
1332:
1328:
1322:
1318:
1310:
1308:
1306:
1302:
1299:, large
1298:
1294:
1290:
1286:
1281:
1273:
1271:
1269:
1265:
1261:
1257:
1253:
1249:
1245:
1241:
1237:
1233:
1229:
1225:
1221:
1217:
1211:
1203:
1201:
1199:
1195:
1191:
1187:
1183:
1179:
1176:defects, and
1175:
1171:
1167:
1163:
1159:
1153:
1149:
1148:Endocrinology
1145:
1137:
1135:
1133:
1129:
1125:
1121:
1117:
1113:
1109:
1105:
1099:
1095:
1091:
1087:
1079:
1077:
1075:
1071:
1067:
1063:
1057:
1049:
1044:
1042:
1040:
1036:
1035:microvascular
1032:
1028:
1024:
1020:
1016:
1012:
1008:
1004:
1000:
996:
992:
988:
984:
980:
976:
972:
968:
964:
959:
951:
949:
948:extremities.
947:
943:
939:
935:
930:
926:
922:
918:
915:
908:
906:
904:
900:
896:
895:interleukin-6
892:
889:
888:heterogeneous
885:
880:
867:
865:
863:
862:bony lamellae
859:
855:
852:
848:
844:
843:megakaryocyte
840:
837:
836:megakaryocyte
833:
830:
826:
822:
819:
815:
811:
807:
803:
799:
795:
791:
787:
782:
774:
772:
770:
766:
762:
758:
754:
750:
746:
742:
737:
729:
724:
722:
716:
714:
712:
708:
704:
700:
696:
695:interleukin 6
692:
688:
684:
680:
676:
672:
668:
664:
656:
654:
652:
648:
644:
640:
636:
632:
628:
624:
620:
616:
612:
608:
607:
598:
596:
594:
590:
587:
583:
579:
575:
571:
567:
563:
559:
555:
551:
550:hrombocytosis
549:
544:
542:
537:
533:
529:
525:
521:
517:
515:
505:
503:
500:
498:
495:
491:
487:
483:
479:
475:
474:immune system
471:
467:
463:
459:
455:
451:
447:
443:
439:
434:
432:
430:
425:
424:ndocrinopathy
423:
418:
417:olyneuropathy
416:
411:
409:
404:
402:
397:
396:ndocrinopathy
395:
390:
388:
383:
382:olyneuropathy
381:
376:
372:
368:
364:
360:
356:
353:
350:
346:
342:
338:
335:(also termed
334:
325:
321:
317:
315:
311:
307:
303:
299:
295:
292:
290:
286:
282:
278:
274:
271:
267:
263:
259:
255:
251:
247:
243:
239:
235:
231:
227:
224:
222:
218:
214:
212:
208:
204:
200:
196:
192:
188:
187:renal failure
184:
180:
176:
172:
168:
164:
160:
157:
155:
154:Complications
151:
147:
143:
139:
135:
131:
130:visceromegaly
127:
123:
119:
115:
111:
108:
106:
102:
97:
91:
88:
86:
82:
78:
74:
71:
67:
63:
59:
54:
50:
46:
41:
30:
19:
2902:
2890:
2874:
2858:
2842:
2830:
2818:
2690:Gastrulation
2504:Craniofacial
2426:
2414:
2398:
2379:Plasmacytoma
2305:
2294:
2283:
2259:
2248:
2237:
2222:
2207:
2192:
2139:
2135:
2125:
2098:
2094:
2084:
2049:
2045:
2001:
1997:
1959:
1956:Primary Care
1955:
1934:. Retrieved
1904:
1900:
1876:. Retrieved
1872:
1863:
1795:
1791:
1717:
1713:
1652:(2): 85–95.
1649:
1645:
1593:
1589:
1569:. Retrieved
1565:
1532:
1512:
1472:lenalidomide
1461:
1436:radiotherapy
1427:
1425:
1414:antigen, or
1399:plasma cells
1392:
1385:parameters.
1379:thrombocytes
1367:plasma cells
1352:
1331:polyglobulia
1324:
1321:Polycythemia
1289:asymptomatic
1283:
1260:Nail changes
1244:acrocyanosis
1224:haemangiomas
1216:skin changes
1213:
1204:Skin changes
1186:gynecomastia
1162:Hypogonadism
1155:
1101:
1074:Organomegaly
1066:splenomegaly
1062:hepatomegaly
1059:
1056:Organomegaly
1050:Organomegaly
1019:organomegaly
997:all express
991:plasma cells
961:
912:
882:
847:plasma cells
802:soft tissues
794:plasma cells
784:
739:
720:
660:
657:Pathogenesis
649:levels, and
604:
602:
566:leukocytosis
547:
540:
519:
513:
509:
501:
497:plasma cells
442:mass effects
435:
428:
421:
414:
407:
400:
393:
386:
379:
363:plasma cells
361:of aberrant
357:caused by a
345:PEP syndrome
344:
340:
336:
332:
331:
298:lenalidomide
281:chemotherapy
279:, high-dose
138:gynecomastia
126:hypogonadism
73:plasma cells
2812:known locus
2710:Sirenomelia
2428:light chain
2416:heavy chain
1515:bevacizumab
1468:thalidomide
1431:Mayo Clinic
1397:and clonal
1383:erythrocyte
1305:Papilledema
1301:blind spots
1285:Papilledema
1280:Papilledema
1274:Papilledema
1264:leukonychia
1262:consist of
1254:, and
1232:lipoatrophy
1039:endoneurial
1011:endothelial
1005:synthesis.
987:tumor cells
983:macrophages
979:bone tissue
975:Osteoblasts
839:hyperplasia
829:iliac crest
818:iliac crest
814:plasma cell
798:bone marrow
753:hyperpathia
711:lymph nodes
647:vitamin B12
606:Papilledema
410:kin changes
389:rganomegaly
306:bevacizumab
294:Thalidomide
250:scleroderma
194:Usual onset
110:Papilledema
77:paraprotein
48:Other names
2935:Categories
2705:Ectromelia
2250:DiseasesDB
1878:2023-08-16
1571:2023-08-14
1541:References
1416:siltuximab
1252:hyperaemia
1190:Amenorrhea
1142:See also:
1031:neuropathy
965:levels in
899:Neuropathy
891:lymph node
856:, causing
757:Neuropathy
635:joint pain
611:optic disc
516:apilledema
302:bortezomib
289:Medication
134:amenorrhea
2914:Multiple
2497:syndromes
2360:disorders
2285:eMedicine
2261:SNOMED CT
1936:15 August
1923:1520-4391
1812:2035-3006
1752:128361561
1736:0361-8609
1453:melphalan
1422:Treatment
1408:rituximab
1381:, and/or
1297:scotomata
1293:headaches
1182:impotence
925:sclerotic
858:sclerosis
749:allodynia
717:Diagnosis
687:cytokines
675:cytokines
558:platelets
494:malignant
490:cytokines
484:, i.e. a
462:cytokines
446:neoplasms
438:neoplasms
323:Frequency
314:Prognosis
269:Treatment
179:infection
85:Specialty
70:malignant
2694:mesoderm
2557:Cyclopia
2307:Orphanet
2290:derm/771
2266:79268002
2166:13364332
2136:Br Med J
2117:18024383
2076:30498913
2020:12456500
1976:27866585
1931:16304404
1830:28894560
1744:31012139
1674:31324035
1666:28299525
1610:21035860
1496:NK cells
1466:such as
1451:such as
1447:plus an
1443:such as
1268:clubbing
1248:flushing
1240:necrosis
946:proximal
897:levels.
832:biopsies
821:biopsies
765:weakness
741:Subacute
681:and the
651:diarrhea
586:cytokine
478:neoplasm
458:hormones
375:symptoms
355:syndrome
205:Chronic.
202:Duration
105:Symptoms
90:Oncology
2244:D016878
2157:2035359
2067:6342878
1821:5584767
1535:Bristol
1529:History
1492:T cells
1359:CT scan
1128:pleural
1120:Ascites
1108:ascites
1090:Ascites
1041:edema.
923:can be
921:lesions
917:lesions
903:hyaline
860:of the
854:lesions
796:in the
524:ascites
492:by the
476:to the
454:humoral
367:acronym
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967:plasma
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697:, and
645:, low
580:, and
545:, and
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185:, and
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2657:Limbs
2255:29226
2233:273.8
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1998:Blood
1748:S2CID
1670:S2CID
1506:(see
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1023:edema
971:serum
938:spine
929:lytic
747:with
643:fever
536:edema
464:, or
371:signs
359:clone
343:, or
118:edema
2394:MGUS
2367:PCDs
2312:2905
2296:GARD
2239:MeSH
2228:9-CM
2162:PMID
2113:PMID
2072:PMID
2016:PMID
1972:PMID
1938:2023
1927:PMID
1919:ISSN
1905:2005
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963:VEGF
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703:VEGF
699:TNFα
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326:Rare
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