29:
220:
Total serum tryptase is elevated in mast cell leukemia. Normal total (alpha + beta) serum tryptase is approximately 6 micro g/L (range 0 to 11 micro g/L). Values of several hundred micro g/L are characteristic of mast cell leukemia. Plasma and urinary histamine levels are frequently elevated in mast
105:
of face and trunk. The typical cutaneous mast cell infiltrates of urticaria pigmentosa are usually not present before, during, or after diagnosis in patients who have mast cell leukemia. Symptoms include abdominal pain, bone pain, and peptic ulcer which are more prevalent than in other subtypes of
138:(CT) scanning is used to look for hepatosplenomegaly and lymphadenopathy. Plain radiography and bone densitometry can be used to assess bone involvement and the presence of osteoporosis. Endoscopy and biopsy can be useful if gut involvement is suspected.
860:
845:
269:
Acute mast cell leukemia is extremely aggressive and has a grave prognosis. In most cases, multi-organ failure including bone marrow failure develops over weeks to months. Median survival after diagnosis is only about 6 months.
225:(HDC) is the enzyme that catalyzes the reaction which produces histamine from histidine. Measurement of histidine carboxylase in the marrow cells of patients with mast cell leukemia is a very sensitive marker of mast cells.
159:
is an enzyme contained in mast cell granules. Mast cell numbers are best estimated by tryptase immunostaining because very poorly granulated cells may stain very weakly if at all for alpha-naphthol chloroacetate esterase.
237:) is important in mast cell function. Immunotherapy with anti-IgE immunoglobulin raised in sheep resulted in a transient decrease in the numbers of circulating mast cells in one patient with mast cell leukemia. Although
642:
Sperr WR, Jordan JH, Fiegl M, et al. (June 2002). "Serum tryptase levels in patients with mastocytosis: correlation with mast cell burden and implication for defining the category of disease".
1527:
101:
Acute mast cell leukemia is a rapidly progressive disorder with leukemic mast cells in blood and in large numbers in marrow. The common signs and symptoms include fever, headache,
875:
540:
Valent P (October 1995). "1995 Mack-Forster Award
Lecture. Review. Mast cell differentiation antigens: expression in normal and malignant cells and use for diagnostic purposes".
1007:
685:
Krauth MT, Agis H, Aichberger KJ, et al. (November 2004), "Histidine carboxylase (HDC) as novel marker of immature neoplastic mast cells in systemic mastocytosis",
241:
has led to brief responses in patients with mast cell leukemia, no firm conclusions as to the efficacy of this treatment are possible. Chemotherapy with combination of
1448:
290:
Lichtman MA, Segel GB (2005). "Uncommon phenotypes of acute myelogenous leukemia: basophilic, mast cell, eosinophilic, and myeloid dendritic cell subtypes: a review".
1158:
1111:
1012:
936:
1307:
1017:
423:
Valent P, Sperr WR, Samorapoompichit P, et al. (July 2001). "Myelomastocytic overlap syndromes: biology, criteria, and relationship to mastocytosis".
1428:
1084:
1593:
1443:
388:
Travis WD, Li CY, Hoagland HC, Travis LB, Banks PM (December 1986). "Mast cell leukemia: report of a case and review of the literature".
768:
Dalton R, Chan L, Batten E, Eridani S (October 1986). "Mast cell leukaemia: evidence for bone marrow origin of the pathological clone".
693:(11 Pt 2), Abstracts of the American Society of Hematology 46th Annual Meeting. December 4–7, 2004, San Diego, California, USA: 272b,
126:
which can lead to serious bleeding. Liver and splenic dysfunction also contributes to hemorrhage. Involvement of the bone can lead to
601:
584:
1168:
929:
1655:
1433:
1344:
1173:
1517:
1285:
1146:
1044:
1625:
1560:
1096:
1022:
1610:
1391:
1327:
1275:
1263:
1034:
1002:
922:
1522:
1337:
1289:
1228:
949:
258:
1413:
1379:
1079:
886:
70:
1106:
222:
135:
1583:
1369:
1253:
1218:
1136:
1069:
992:
107:
74:
62:
1650:
1620:
102:
78:
77:. In a small proportion of cases, acute mast cell leukemia may evolve from a more progressive form of
1542:
1532:
1438:
242:
864:
151:
Cytochemical properties of the leukemic cells must be typical of mast cell derivation (presence of
585:"Expression of Bcl-2 by human bone marrow mast cells and its overexpression in mast cell leukemia"
155:
granules staining with alpha-naphthyl chloroacetate esterase, but not with peroxidase). Mast cell
1401:
793:
667:
565:
119:
1537:
1492:
897:
785:
750:
702:
659:
606:
557:
507:
440:
405:
365:
334:
307:
41:
914:
777:
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732:
694:
651:
596:
549:
497:
489:
432:
397:
299:
93:
represent less than 10% of blood cells, the tumor is called "aleukemic" mast cell leukemia.
1598:
880:
781:
553:
502:
477:
869:
745:
720:
436:
401:
1644:
1193:
1125:
208:. Malignant mast cells overexpress the anti-apoptosis gene, bcl-2. A mutation called
152:
698:
671:
569:
1508:
1497:
797:
493:
254:
127:
28:
891:
1477:
963:
238:
902:
303:
854:
261:
is an option, although no experience exists concerning responses and outcome.
250:
246:
131:
115:
86:
46:
981:
111:
90:
706:
663:
610:
511:
444:
311:
789:
754:
561:
409:
369:
1466:
1207:
1058:
736:
156:
50:
837:
602:
10.1002/(SICI)1096-8652(199903)60:3<191::AID-AJH4>3.0.CO;2-Y
338:
122:
is characteristic. The mast cells release also many anticoagulants like
1316:
1242:
945:
123:
655:
1423:
972:
957:
849:
85:
criteria includes the requirement for a prevalence of 20% neoplastic
354:"[Acute mast cell leukemia disclosed by vasomotor flushing]"
353:
1358:
189:
185:
181:
583:
CerverĂł C, Escribano L, San Miguel JF, et al. (March 1999).
110:. These former symptoms are due to release of a substance called
205:
197:
193:
177:
173:
169:
918:
234:
201:
82:
721:"Eosinophilic leukaemia with trisomy 8 and double gammopathy"
1528:
Adult type of generalized eruption of cutaneous mastocytosis
719:
Ribeiro I, Carvalho IR, Fontes M, et al. (July 1993).
352:
Le Cam MT, Wolkenstein P, Cosnes A, et al. (1997).
811:
Hoffman R, Benz E, Shattil S, Furie B, Cohen H (2004).
168:
The leukemic cells usually are strongly positive for
827:
118:
mast cells. Enlargement of the liver and spleen, or
1607:
1580:
1573:
1551:
1506:
1474:
1465:
1410:
1388:
1366:
1357:
1324:
1315:
1306:
1272:
1250:
1241:
1215:
1206:
1186:
1155:
1133:
1124:
1093:
1066:
1057:
1031:
989:
980:
971:
956:
831:
81:. The diagnosis of acute mast cell leukemia by the
40:
21:
1449:Refractory cytopenia with multilineage dysplasia
69:but can, rarely, evolve from transformation of
1112:Accelerated phase chronic myelogenous leukemia
525:Hoffbrand AV, Catovsky D, Tuddenham E (2005).
930:
456:
454:
325:Daniel MT, Flandrin G, Bernard J (1975). "".
257:as for acute myeloid leukemia has been used.
8:
631:. Cancer Treatment & Research. Springer.
285:
283:
36:Peripheral blood showing mast cell leukemia.
622:
620:
1577:
1471:
1363:
1321:
1312:
1247:
1212:
1130:
1063:
986:
977:
968:
937:
923:
915:
828:
383:
381:
379:
27:
18:
813:Hematology: Basic Principles and Practice
744:
600:
501:
200:) are absent. The cells usually express
1429:Refractory anemia with excess of blasts
279:
212:mutation is detected in most patients.
815:(4th ed.). Churchill Livingstone.
61:is an extremely aggressive subtype of
478:"Red-brown skin lesions and pruritus"
184:. Characteristically, basophil (e.g.
7:
1594:Acute panmyelosis with myelofibrosis
1444:Paroxysmal nocturnal haemoglobinuria
89:in marrow and 10% in blood. If the
782:10.1111/j.1365-2141.1986.tb04133.x
554:10.1111/j.1365-2362.1995.tb01948.x
476:Cather JC, Menter MA (July 2000).
14:
1169:Juvenile myelomonocytic leukemia
1434:Chromosome 5q deletion syndrome
1345:Acute megakaryoblastic leukemia
1174:Chronic myelomonocytic leukemia
1085:Myeloid dendritic cell leukemia
699:10.1182/blood.V104.11.4755.4755
629:Rare Hematological Malignancies
1518:Diffuse cutaneous mastocytosis
494:10.1080/08998280.2000.11927693
1:
1286:Chronic eosinophilic leukemia
1045:Chronic neutrophilic leukemia
437:10.1016/S0145-2126(01)00040-6
402:10.1016/s0025-6196(12)62636-6
1626:Biphenotypic acute leukaemia
1561:Xanthelasmoidal mastocytosis
463:Holland Frei Cancer Medicine
461:Kufe D, et al. (2000).
1003:Acute myeloblastic leukemia
1672:
1523:Erythrodermic mastocytosis
1338:Essential thrombocythaemia
1290:Hypereosinophilic syndrome
644:Int. Arch. Allergy Immunol
529:(5th ed.). Blackwell.
465:(5th ed.). BC Decker.
304:10.1016/j.bcmd.2005.08.006
950:haematological malignancy
482:Proc (Bayl Univ Med Cent)
259:Stem cell transplantation
73:into the more aggressive
35:
26:
527:Postgraduate Haematology
192:) and monocyte markers (
71:chronic myeloid leukemia
22:Acute mast cell leukemia
1107:Philadelphia chromosome
627:Ansell SM, ed. (2008).
223:Histidine decarboxylase
136:computerized tomography
1656:Acute myeloid leukemia
108:acute myeloid leukemia
75:acute myeloid leukemia
63:acute myeloid leukemia
1621:Primary myelofibrosis
1553:Systemic mastocytosis
1498:Systemic mastocytosis
358:Ann Dermatol Venereol
79:systemic mastocytosis
1543:Solitary mastocytoma
1533:Urticaria pigmentosa
1439:Sideroblastic anemia
737:10.1136/jcp.46.7.672
542:Eur. J. Clin. Invest
292:Blood Cells Mol. Dis
243:cytosine arabinoside
65:that usually occurs
327:Nouv Rev Fr Hematol
1488:Mast cell leukemia
1402:Polycythaemia vera
1380:Erythroleukemia/M6
1264:Acute eosinophilic
233:Immunoglobulin E (
120:hepatosplenomegaly
97:Signs and symptoms
59:Mast cell leukemia
1638:
1637:
1634:
1633:
1569:
1568:
1538:Mast cell sarcoma
1493:Mast cell sarcoma
1461:
1460:
1457:
1456:
1424:Refractory anemia
1353:
1352:
1302:
1301:
1298:
1297:
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1202:
1201:
1182:
1181:
1120:
1119:
1053:
1052:
912:
911:
656:10.1159/000059404
56:
55:
16:Medical condition
1663:
1614:
1587:
1578:
1574:Multiple/unknown
1554:
1511:
1481:
1472:
1417:
1395:
1373:
1364:
1331:
1322:
1313:
1279:
1257:
1248:
1229:Acute basophilic
1222:
1213:
1162:
1140:
1131:
1100:
1073:
1064:
1038:
996:
987:
978:
969:
939:
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916:
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531:
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31:
19:
1671:
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1666:
1665:
1664:
1662:
1661:
1660:
1641:
1640:
1639:
1630:
1608:
1603:
1599:Myeloid sarcoma
1581:
1565:
1552:
1547:
1507:
1502:
1475:
1453:
1411:
1406:
1389:
1384:
1367:
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1273:
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1251:
1233:
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1198:
1178:
1156:
1151:
1134:
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1094:
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1067:
1049:
1032:
1027:
990:
961:
952:
943:
913:
908:
907:
840:
826:
821:
820:
810:
809:
805:
770:Br. J. Haematol
767:
766:
762:
725:J. Clin. Pathol
718:
717:
713:
684:
683:
679:
641:
640:
636:
626:
625:
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582:
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577:
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524:
523:
519:
475:
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459:
452:
422:
421:
417:
390:Mayo Clin. Proc
387:
386:
377:
351:
350:
346:
324:
323:
319:
289:
288:
281:
276:
267:
231:
221:cell leukemia.
218:
166:
149:
144:
99:
17:
12:
11:
5:
1669:
1667:
1659:
1658:
1653:
1643:
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1632:
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1557:
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1504:
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1426:
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1376:
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1351:
1350:
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1341:
1340:
1334:
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1319:
1310:
1304:
1303:
1300:
1299:
1296:
1295:
1293:
1292:
1282:
1280:
1270:
1269:
1267:
1266:
1260:
1258:
1245:
1239:
1238:
1235:
1234:
1232:
1231:
1225:
1223:
1210:
1204:
1203:
1200:
1199:
1197:
1196:
1190:
1188:
1184:
1183:
1180:
1179:
1177:
1176:
1171:
1165:
1163:
1153:
1152:
1150:
1149:
1143:
1141:
1128:
1122:
1121:
1118:
1117:
1115:
1114:
1109:
1103:
1101:
1091:
1090:
1088:
1087:
1082:
1076:
1074:
1061:
1055:
1054:
1051:
1050:
1048:
1047:
1041:
1039:
1029:
1028:
1026:
1025:
1020:
1015:
1010:
1005:
999:
997:
984:
975:
966:
954:
953:
944:
942:
941:
934:
927:
919:
910:
909:
906:
905:
894:
883:
872:
857:
841:
836:
835:
833:
832:Classification
825:
824:External links
822:
819:
818:
803:
776:(2): 397–406.
760:
711:
677:
634:
616:
589:Am. J. Hematol
575:
548:(10): 715–20.
532:
517:
468:
450:
431:(7): 595–602.
415:
396:(12): 957–66.
375:
344:
317:
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1230:
1227:
1226:
1224:
1221:
1220:
1214:
1211:
1209:
1205:
1195:
1194:Histiocytosis
1192:
1191:
1189:
1185:
1175:
1172:
1170:
1167:
1166:
1164:
1161:
1160:
1154:
1148:
1145:
1144:
1142:
1139:
1138:
1132:
1129:
1127:
1126:Myelomonocyte
1123:
1113:
1110:
1108:
1105:
1104:
1102:
1099:
1098:
1092:
1086:
1083:
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985:
983:
979:
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947:
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917:
904:
900:
899:
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893:
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888:
884:
882:
878:
877:
873:
871:
867:
866:
862:
858:
856:
852:
851:
847:
843:
842:
839:
834:
830:
823:
814:
807:
804:
799:
795:
791:
787:
783:
779:
775:
771:
764:
761:
756:
752:
747:
742:
738:
734:
730:
726:
722:
715:
712:
708:
704:
700:
696:
692:
688:
681:
678:
673:
669:
665:
661:
657:
653:
650:(2): 136–41.
649:
645:
638:
635:
630:
623:
621:
617:
612:
608:
603:
598:
594:
590:
586:
579:
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419:
416:
411:
407:
403:
399:
395:
391:
384:
382:
380:
376:
371:
367:
363:
360:(in French).
359:
355:
348:
345:
340:
336:
333:(3): 319–32.
332:
329:(in French).
328:
321:
318:
313:
309:
305:
301:
298:(3): 370–83.
297:
293:
286:
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164:Tumor markers
163:
161:
158:
154:
153:metachromatic
147:Cytochemistry
146:
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60:
52:
48:
45:
43:
39:
34:
30:
25:
20:
1651:Rare cancers
1609:
1582:
1509:Mastocytosis
1487:
1476:
1412:
1390:
1368:
1326:
1274:
1252:
1217:
1157:
1135:
1095:
1068:
1033:
991:
964:granulocytes
896:
885:
874:
859:
844:
812:
806:
773:
769:
763:
731:(7): 672–3.
728:
724:
714:
690:
686:
680:
647:
643:
637:
628:
595:(3): 191–5.
592:
588:
578:
545:
541:
535:
526:
520:
488:(3): 297–9.
485:
481:
471:
462:
428:
424:
418:
393:
389:
364:(9): 621–2.
361:
357:
347:
330:
326:
320:
295:
291:
268:
255:mitoxantrone
232:
219:
216:Biochemistry
209:
167:
150:
130:. Abdominal
128:osteoporosis
100:
66:
58:
57:
1478:Mastocytoma
245:and either
239:splenectomy
1645:Categories
962:and other
898:DiseasesDB
274:References
251:daunomycin
247:idarubicin
132:ultrasound
116:neoplastic
91:mast cells
87:mast cells
47:Hematology
982:Myelocyte
948:-related
425:Leuk. Res
265:Prognosis
229:Treatment
142:Diagnosis
112:histamine
42:Specialty
1467:CFU-Mast
1208:CFU-Baso
1059:Monocyte
707:15562525
672:42677668
664:12065914
611:10072109
570:23872236
512:16389403
445:11377685
312:16203163
157:tryptase
103:flushing
51:oncology
1317:CFU-Meg
1243:CFU-Eos
1080:AMoL/M5
946:Myeloid
892:D007946
881:M9742/3
798:9763026
790:3096368
755:8157759
562:8557056
503:1317061
410:3095598
370:9739925
124:heparin
67:de novo
1023:APL/M3
973:CFU-GM
958:CFU-GM
796:
788:
753:
746:501401
743:
705:
670:
662:
609:
568:
560:
510:
500:
443:
408:
368:
337:
310:
180:, and
1359:CFU-E
1187:Other
1159:MD-MP
903:30102
876:ICD-O
870:207.8
855:C94.3
794:S2CID
687:Blood
668:S2CID
566:S2CID
339:54900
253:, or
190:CD123
186:CD11b
182:CD117
114:from
887:MeSH
865:9-CM
786:PMID
751:PMID
703:PMID
660:PMID
607:PMID
558:PMID
508:PMID
441:PMID
406:PMID
366:PMID
335:PMID
308:PMID
206:CD25
204:and
198:CD15
194:CD14
178:CD68
174:CD33
170:CD13
49:and
1584:AML
1370:AML
1308:MEP
1254:AML
1219:AML
1137:AML
1097:CML
1070:AML
993:AML
861:ICD
846:ICD
778:doi
741:PMC
733:doi
695:doi
691:104
652:doi
648:128
597:doi
550:doi
498:PMC
490:doi
433:doi
398:doi
362:124
300:doi
235:IgE
210:KIT
202:CD2
134:or
83:WHO
1647::
1611:MP
1414:MD
1392:MP
1328:MP
1276:MP
1147:M4
1035:MP
1018:M2
1013:M1
1008:M0
901::
890::
879::
868::
853::
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774:64
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492::
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435::
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400::
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314:.
302::
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