Blood Res 2021; 56(2):
Published online June 30, 2021
https://doi.org/10.5045/br.2021.2021040
© The Korean Society of Hematology
Correspondence to : Stephen E. Langabeer
Cancer Molecular Diagnostics, Trinity Translational Medicine Institute, St. James’s Hospital, Dublin, D08 W9RT, Ireland
E-mail: slangabeer@stjames.ie
This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.
TO THE EDITOR: Emergence of a
A 64-year-old female with a long standing history of systemic lupus erythematosus presented in 2002 with lethargy, tiredness and a cough. Full blood count showed anemia (Hb, 11.6 g/dL), neutropenia (0.2×109/L) and a normal platelet count (243×109/L). Bone marrow aspirate was predominantly infiltrated with Sudan Black-positive myeloblasts consistent with AML FAB type M1. The karyotype was normal with no evidence of splenomegaly identified. A recovery thrombocytosis of 903×109/L was noted after three courses of cytarabine and daunorubicin but was felt to be reactive and resolved. Review of archival bone marrow aspirates showed no morphological evidence of an MPN at this time. The patient relapsed in 2004 with 25% CD13/CD33/CD117+ myeloblasts in the bone marrow aspirate, normal spleen size and a normal karyotype. The patient was subsequently treated with two courses of FLAG-Ida (fludarabine, cytarabine, G-CSF, idarubicin) achieving complete remission with no rebound thrombocytosis. Blood counts were stable for more than a twelve years until 2017 when a slowly rising Hb and HCT accompanied by a microcytosis were noted and sustained until the present (peak Hb, 16.8 g/dL; peak HCT, 0.517; nadir MCV, 68.1 fL) (Fig. 1).
The first case reported of PV developing from a background of AML was described in the pre-
Table 1 Features of four cases of polycythemia vera emerging while in long-term remission from acute myeloid leukemia.
Case reference | Age at AML diagnosis | Sex | AML type | AML treatment | Time to PV from AML diagnosis | ||
---|---|---|---|---|---|---|---|
Antonioli | 60 | M | M2 | Flu/AraC/Ida ×1 Ida/Etop ×1 AutoSCT | 7 yr | Not detected | 28% |
Belotti | 71 | M | M4 | AraC/Ida/Etop ×1 Ida/AraC ×2 AraC ×2 | 7 yr | Not done | Positive |
Portell | 59 | F | M2 | Ida/AraC ×1 AraC ×3 | 5 yr | 2% | 91% |
This case | 64 | F | M1 | AraC/Daun ×8 Flu/AraC/Ida ×2 | 18 yr | Not detected | 17% |
Abbreviations: AML, acute myeloid leukemia; AraC, cytarabine; AutoSCT, autologous stem cell transplantation; Daun, daunorubicin; Etop, etoposide; Flu, fludarabine; Ida, idarubicin; M1, acute myeloid leukemia with minimal maturation; M2, acute differentiated myeloid leukemia; M4, acute myelomonocytic leukemia; VAF, variant allele frequency.
While pre-clinical models of PV are valuable, standardized tools for dissecting the molecular mechanisms of the disease [13], unusual cases such as that described herein further emphasize the underlying clonal complexity of the development of
No potential conflicts of interest relevant to this article were reported.
Blood Res 2021; 56(2): 121-123
Published online June 30, 2021 https://doi.org/10.5045/br.2021.2021040
Copyright © The Korean Society of Hematology.
Stephen E. Langabeer1, Derick W. O’Flynn2, Mary R. Cahill2
1Cancer Molecular Diagnostics, St. James’s Hospital, Dublin, 2Department of Haematology, Cork University Hospital, Cork, Ireland
Correspondence to:Stephen E. Langabeer
Cancer Molecular Diagnostics, Trinity Translational Medicine Institute, St. James’s Hospital, Dublin, D08 W9RT, Ireland
E-mail: slangabeer@stjames.ie
This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.
TO THE EDITOR: Emergence of a
A 64-year-old female with a long standing history of systemic lupus erythematosus presented in 2002 with lethargy, tiredness and a cough. Full blood count showed anemia (Hb, 11.6 g/dL), neutropenia (0.2×109/L) and a normal platelet count (243×109/L). Bone marrow aspirate was predominantly infiltrated with Sudan Black-positive myeloblasts consistent with AML FAB type M1. The karyotype was normal with no evidence of splenomegaly identified. A recovery thrombocytosis of 903×109/L was noted after three courses of cytarabine and daunorubicin but was felt to be reactive and resolved. Review of archival bone marrow aspirates showed no morphological evidence of an MPN at this time. The patient relapsed in 2004 with 25% CD13/CD33/CD117+ myeloblasts in the bone marrow aspirate, normal spleen size and a normal karyotype. The patient was subsequently treated with two courses of FLAG-Ida (fludarabine, cytarabine, G-CSF, idarubicin) achieving complete remission with no rebound thrombocytosis. Blood counts were stable for more than a twelve years until 2017 when a slowly rising Hb and HCT accompanied by a microcytosis were noted and sustained until the present (peak Hb, 16.8 g/dL; peak HCT, 0.517; nadir MCV, 68.1 fL) (Fig. 1).
The first case reported of PV developing from a background of AML was described in the pre-
Table 1 . Features of four cases of polycythemia vera emerging while in long-term remission from acute myeloid leukemia..
Case reference | Age at AML diagnosis | Sex | AML type | AML treatment | Time to PV from AML diagnosis | ||
---|---|---|---|---|---|---|---|
Antonioli | 60 | M | M2 | Flu/AraC/Ida ×1 Ida/Etop ×1 AutoSCT | 7 yr | Not detected | 28% |
Belotti | 71 | M | M4 | AraC/Ida/Etop ×1 Ida/AraC ×2 AraC ×2 | 7 yr | Not done | Positive |
Portell | 59 | F | M2 | Ida/AraC ×1 AraC ×3 | 5 yr | 2% | 91% |
This case | 64 | F | M1 | AraC/Daun ×8 Flu/AraC/Ida ×2 | 18 yr | Not detected | 17% |
Abbreviations: AML, acute myeloid leukemia; AraC, cytarabine; AutoSCT, autologous stem cell transplantation; Daun, daunorubicin; Etop, etoposide; Flu, fludarabine; Ida, idarubicin; M1, acute myeloid leukemia with minimal maturation; M2, acute differentiated myeloid leukemia; M4, acute myelomonocytic leukemia; VAF, variant allele frequency..
While pre-clinical models of PV are valuable, standardized tools for dissecting the molecular mechanisms of the disease [13], unusual cases such as that described herein further emphasize the underlying clonal complexity of the development of
No potential conflicts of interest relevant to this article were reported.
Table 1 . Features of four cases of polycythemia vera emerging while in long-term remission from acute myeloid leukemia..
Case reference | Age at AML diagnosis | Sex | AML type | AML treatment | Time to PV from AML diagnosis | ||
---|---|---|---|---|---|---|---|
Antonioli | 60 | M | M2 | Flu/AraC/Ida ×1 Ida/Etop ×1 AutoSCT | 7 yr | Not detected | 28% |
Belotti | 71 | M | M4 | AraC/Ida/Etop ×1 Ida/AraC ×2 AraC ×2 | 7 yr | Not done | Positive |
Portell | 59 | F | M2 | Ida/AraC ×1 AraC ×3 | 5 yr | 2% | 91% |
This case | 64 | F | M1 | AraC/Daun ×8 Flu/AraC/Ida ×2 | 18 yr | Not detected | 17% |
Abbreviations: AML, acute myeloid leukemia; AraC, cytarabine; AutoSCT, autologous stem cell transplantation; Daun, daunorubicin; Etop, etoposide; Flu, fludarabine; Ida, idarubicin; M1, acute myeloid leukemia with minimal maturation; M2, acute differentiated myeloid leukemia; M4, acute myelomonocytic leukemia; VAF, variant allele frequency..