Atypical chronic myeloid leukemia is clinically distinct from unclassifiable myelodysplastic/myeloproliferative neoplasms

Sa A. Wang, Robert P. Hasserjian, Patricia S. Fox, Heesun J. Rogers, Julia T. Geyer, Devon Chabot-Richards, Elizabeth Weinzierl, Joseph Hatem, Jesse Jaso, Rashmi Kanagal-Shamanna, Francesco C. Stingo, Keyur P. Patel, Meenakshi Mehrotra, Carlos Bueso-Ramos, Ken H. Young, Courtney D. Dinardo, Srdan Verstovsek, Ramon V. Tiu, Adam Bagg, Eric D. HsiDaniel A. Arber, Kathryn Foucar, Raja Luthra, Attilio Orazi

Research output: Contribution to journalArticle

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Abstract

Atypical chronic myeloid leukemia (aCML) is a rare subtype of myelodysplastic/ myeloproliferative neoplasm (MDS/MPN) largely defined morphologically. It is, unclear, however, whether aCML-associated features are distinctive enough to allow its separation from unclassifiableMDS/MPN(MDS/MPN-U) .Tostudy these 2 rare entities, 134 patient archives were collected from 7 large medical centers, of which 65 (49%) cases were further classified asaCMLand the remaining 69 (51%) asMDS/MPN-U. Distinctively,aCML was associated with many adverse features and an inferior overall survival (12.4 vs 21.8 months, P 5 .004) and AML-free survival (11.2 vs 18.9 months, P 5 .003). The aCML defining features of leukocytosis and circulating myeloid precursors, but not dysgranulopoiesis, were independent negative predictors. Other factors, such as lactate dehydrogenase, circulating myeloblasts, platelets, and cytogenetics could further stratify MDS/ MPN-U but not aCML patient risks. aCML appeared to have more mutated RAS (7/20 [35%] vs 4/29 [14%]) and less JAK2p.V617F (3/42 [7%] vs 10/52 [19%]), but was not statistically significant. Somatic CSF3R T618I (0/54) and CALR (0/30) mutations were not detected either in aCML or MDS/MPN-U. In conclusion, within MDS/MPN, the World Health Organization 2008 criteria for aCML identify a subgroup of patients with features clearly distinct from MDS/MPN-U. The MDS/MPN-U category is heterogeneous, and patient risk can be further stratified by a number of clinicopathological parameters.

Original languageEnglish (US)
Pages (from-to)2645-2651
Number of pages7
JournalBlood
Volume123
Issue number17
DOIs
StatePublished - Apr 24 2014

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Leukemia, Myeloid, Chronic, Atypical, BCR-ABL Negative
Platelets
L-Lactate Dehydrogenase
Neoplasms
Health
Granulocyte Precursor Cells
Survival
Leukocytosis
Cytogenetics
Blood Platelets

ASJC Scopus subject areas

  • Hematology
  • Biochemistry
  • Cell Biology
  • Immunology

Cite this

Wang, S. A., Hasserjian, R. P., Fox, P. S., Rogers, H. J., Geyer, J. T., Chabot-Richards, D., ... Orazi, A. (2014). Atypical chronic myeloid leukemia is clinically distinct from unclassifiable myelodysplastic/myeloproliferative neoplasms. Blood, 123(17), 2645-2651. https://doi.org/10.1182/blood-2014-02-553800

Atypical chronic myeloid leukemia is clinically distinct from unclassifiable myelodysplastic/myeloproliferative neoplasms. / Wang, Sa A.; Hasserjian, Robert P.; Fox, Patricia S.; Rogers, Heesun J.; Geyer, Julia T.; Chabot-Richards, Devon; Weinzierl, Elizabeth; Hatem, Joseph; Jaso, Jesse; Kanagal-Shamanna, Rashmi; Stingo, Francesco C.; Patel, Keyur P.; Mehrotra, Meenakshi; Bueso-Ramos, Carlos; Young, Ken H.; Dinardo, Courtney D.; Verstovsek, Srdan; Tiu, Ramon V.; Bagg, Adam; Hsi, Eric D.; Arber, Daniel A.; Foucar, Kathryn; Luthra, Raja; Orazi, Attilio.

In: Blood, Vol. 123, No. 17, 24.04.2014, p. 2645-2651.

Research output: Contribution to journalArticle

Wang, SA, Hasserjian, RP, Fox, PS, Rogers, HJ, Geyer, JT, Chabot-Richards, D, Weinzierl, E, Hatem, J, Jaso, J, Kanagal-Shamanna, R, Stingo, FC, Patel, KP, Mehrotra, M, Bueso-Ramos, C, Young, KH, Dinardo, CD, Verstovsek, S, Tiu, RV, Bagg, A, Hsi, ED, Arber, DA, Foucar, K, Luthra, R & Orazi, A 2014, 'Atypical chronic myeloid leukemia is clinically distinct from unclassifiable myelodysplastic/myeloproliferative neoplasms', Blood, vol. 123, no. 17, pp. 2645-2651. https://doi.org/10.1182/blood-2014-02-553800
Wang, Sa A. ; Hasserjian, Robert P. ; Fox, Patricia S. ; Rogers, Heesun J. ; Geyer, Julia T. ; Chabot-Richards, Devon ; Weinzierl, Elizabeth ; Hatem, Joseph ; Jaso, Jesse ; Kanagal-Shamanna, Rashmi ; Stingo, Francesco C. ; Patel, Keyur P. ; Mehrotra, Meenakshi ; Bueso-Ramos, Carlos ; Young, Ken H. ; Dinardo, Courtney D. ; Verstovsek, Srdan ; Tiu, Ramon V. ; Bagg, Adam ; Hsi, Eric D. ; Arber, Daniel A. ; Foucar, Kathryn ; Luthra, Raja ; Orazi, Attilio. / Atypical chronic myeloid leukemia is clinically distinct from unclassifiable myelodysplastic/myeloproliferative neoplasms. In: Blood. 2014 ; Vol. 123, No. 17. pp. 2645-2651.
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title = "Atypical chronic myeloid leukemia is clinically distinct from unclassifiable myelodysplastic/myeloproliferative neoplasms",
abstract = "Atypical chronic myeloid leukemia (aCML) is a rare subtype of myelodysplastic/ myeloproliferative neoplasm (MDS/MPN) largely defined morphologically. It is, unclear, however, whether aCML-associated features are distinctive enough to allow its separation from unclassifiableMDS/MPN(MDS/MPN-U) .Tostudy these 2 rare entities, 134 patient archives were collected from 7 large medical centers, of which 65 (49{\%}) cases were further classified asaCMLand the remaining 69 (51{\%}) asMDS/MPN-U. Distinctively,aCML was associated with many adverse features and an inferior overall survival (12.4 vs 21.8 months, P 5 .004) and AML-free survival (11.2 vs 18.9 months, P 5 .003). The aCML defining features of leukocytosis and circulating myeloid precursors, but not dysgranulopoiesis, were independent negative predictors. Other factors, such as lactate dehydrogenase, circulating myeloblasts, platelets, and cytogenetics could further stratify MDS/ MPN-U but not aCML patient risks. aCML appeared to have more mutated RAS (7/20 [35{\%}] vs 4/29 [14{\%}]) and less JAK2p.V617F (3/42 [7{\%}] vs 10/52 [19{\%}]), but was not statistically significant. Somatic CSF3R T618I (0/54) and CALR (0/30) mutations were not detected either in aCML or MDS/MPN-U. In conclusion, within MDS/MPN, the World Health Organization 2008 criteria for aCML identify a subgroup of patients with features clearly distinct from MDS/MPN-U. The MDS/MPN-U category is heterogeneous, and patient risk can be further stratified by a number of clinicopathological parameters.",
author = "Wang, {Sa A.} and Hasserjian, {Robert P.} and Fox, {Patricia S.} and Rogers, {Heesun J.} and Geyer, {Julia T.} and Devon Chabot-Richards and Elizabeth Weinzierl and Joseph Hatem and Jesse Jaso and Rashmi Kanagal-Shamanna and Stingo, {Francesco C.} and Patel, {Keyur P.} and Meenakshi Mehrotra and Carlos Bueso-Ramos and Young, {Ken H.} and Dinardo, {Courtney D.} and Srdan Verstovsek and Tiu, {Ramon V.} and Adam Bagg and Hsi, {Eric D.} and Arber, {Daniel A.} and Kathryn Foucar and Raja Luthra and Attilio Orazi",
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T1 - Atypical chronic myeloid leukemia is clinically distinct from unclassifiable myelodysplastic/myeloproliferative neoplasms

AU - Wang, Sa A.

AU - Hasserjian, Robert P.

AU - Fox, Patricia S.

AU - Rogers, Heesun J.

AU - Geyer, Julia T.

AU - Chabot-Richards, Devon

AU - Weinzierl, Elizabeth

AU - Hatem, Joseph

AU - Jaso, Jesse

AU - Kanagal-Shamanna, Rashmi

AU - Stingo, Francesco C.

AU - Patel, Keyur P.

AU - Mehrotra, Meenakshi

AU - Bueso-Ramos, Carlos

AU - Young, Ken H.

AU - Dinardo, Courtney D.

AU - Verstovsek, Srdan

AU - Tiu, Ramon V.

AU - Bagg, Adam

AU - Hsi, Eric D.

AU - Arber, Daniel A.

AU - Foucar, Kathryn

AU - Luthra, Raja

AU - Orazi, Attilio

PY - 2014/4/24

Y1 - 2014/4/24

N2 - Atypical chronic myeloid leukemia (aCML) is a rare subtype of myelodysplastic/ myeloproliferative neoplasm (MDS/MPN) largely defined morphologically. It is, unclear, however, whether aCML-associated features are distinctive enough to allow its separation from unclassifiableMDS/MPN(MDS/MPN-U) .Tostudy these 2 rare entities, 134 patient archives were collected from 7 large medical centers, of which 65 (49%) cases were further classified asaCMLand the remaining 69 (51%) asMDS/MPN-U. Distinctively,aCML was associated with many adverse features and an inferior overall survival (12.4 vs 21.8 months, P 5 .004) and AML-free survival (11.2 vs 18.9 months, P 5 .003). The aCML defining features of leukocytosis and circulating myeloid precursors, but not dysgranulopoiesis, were independent negative predictors. Other factors, such as lactate dehydrogenase, circulating myeloblasts, platelets, and cytogenetics could further stratify MDS/ MPN-U but not aCML patient risks. aCML appeared to have more mutated RAS (7/20 [35%] vs 4/29 [14%]) and less JAK2p.V617F (3/42 [7%] vs 10/52 [19%]), but was not statistically significant. Somatic CSF3R T618I (0/54) and CALR (0/30) mutations were not detected either in aCML or MDS/MPN-U. In conclusion, within MDS/MPN, the World Health Organization 2008 criteria for aCML identify a subgroup of patients with features clearly distinct from MDS/MPN-U. The MDS/MPN-U category is heterogeneous, and patient risk can be further stratified by a number of clinicopathological parameters.

AB - Atypical chronic myeloid leukemia (aCML) is a rare subtype of myelodysplastic/ myeloproliferative neoplasm (MDS/MPN) largely defined morphologically. It is, unclear, however, whether aCML-associated features are distinctive enough to allow its separation from unclassifiableMDS/MPN(MDS/MPN-U) .Tostudy these 2 rare entities, 134 patient archives were collected from 7 large medical centers, of which 65 (49%) cases were further classified asaCMLand the remaining 69 (51%) asMDS/MPN-U. Distinctively,aCML was associated with many adverse features and an inferior overall survival (12.4 vs 21.8 months, P 5 .004) and AML-free survival (11.2 vs 18.9 months, P 5 .003). The aCML defining features of leukocytosis and circulating myeloid precursors, but not dysgranulopoiesis, were independent negative predictors. Other factors, such as lactate dehydrogenase, circulating myeloblasts, platelets, and cytogenetics could further stratify MDS/ MPN-U but not aCML patient risks. aCML appeared to have more mutated RAS (7/20 [35%] vs 4/29 [14%]) and less JAK2p.V617F (3/42 [7%] vs 10/52 [19%]), but was not statistically significant. Somatic CSF3R T618I (0/54) and CALR (0/30) mutations were not detected either in aCML or MDS/MPN-U. In conclusion, within MDS/MPN, the World Health Organization 2008 criteria for aCML identify a subgroup of patients with features clearly distinct from MDS/MPN-U. The MDS/MPN-U category is heterogeneous, and patient risk can be further stratified by a number of clinicopathological parameters.

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