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Original Article

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Blood Res 2013; 48(1):

Published online March 31, 2013

https://doi.org/10.5045/br.2013.48.1.31

© The Korean Society of Hematology

Current routine practice and clinico-pathological characteristics associated with acute promyelocytic leukemia in Korea

Sunhyun Ahn1, Joon Seong Park2, Seong Hyun Jeong2, Hyun Woo Lee2, Jun Eun Park3, Mi Hyang Kim4, Yang Soo Kim5, Ho Sup Lee5, Tae Sung Park6, Eunkyoung You6, Insoo Rheem7, Joowon Park7, JI Young Huh8, Myung Seo Kang8, and Sung Ran Cho1*

Author Info. +

1Department of Laboratory Medicine, Ajou University School of Medicine, Suwon, Korea.

2Department of Hematology-Oncology, Ajou University School of Medicine, Suwon, Korea.

3Department of Pediatrics, Ajou University School of Medicine, Suwon, Korea.

4Department of Laboratory Medicine, Kosin University Gospel Hospital, Busan, Korea.

5Department of Internal Medicine, Kosin University Gospel Hospital, Busan, Korea.

6Department of Laboratory Medicine, School of Medicine, Kyung Hee University, Seoul, Korea.

7Department of Laboratory Medicine, Dankook University College of Medicine, Cheonan, Korea.

8Department of Laboratory Medicine, CHA Bundang Medical Center, CHA University, Seongnam, Korea.

Correspondence to : Correspondence to Sung Ran Cho, M.D., Ph.D. Department of Laboratory Medicine, Ajou University School of Medicine, San-5, Wonchun-dong, Yeoungtong-gu, Suwon 443-721, Korea. Tel: +82-31-219-5780, Fax: +82-31-219-5778, sungran@ajou.ac.kr

Received: August 24, 2012; Revised: November 28, 2012; Accepted: February 13, 2013

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

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Background

Acute promyelocytic leukemia (APL) can be life threatening, necessitating emergency therapy with prompt diagnosis by morphologic findings, immunophenotyping, cytogenetic analysis, or molecular studies. This study aimed to assess the current routine practices in APL and the clinico-pathologic features of APL.

Methods

We reviewed the medical records of 48 Korean patients (25 men, 23 women; median age, 51 (20-80) years) diagnosed with APL in 5 university hospitals between March 2007 and February 2012.

Results

The WBC count at diagnosis and platelet count varied from 0.4 to 81.0 (median 2.0)×109/L and 2.7 to 124.0 (median 54.5)×109/L, respectively. The median values for prothrombin time and activated partial thromboplastin time were 14.7 (11.3-44.1) s and 29 (24-62) s, respectively. All but 2 patients (96%) showed a fibrin/fibrinogen degradation product value of >20 µg/mL. The D-dimer median value was 5,000 (686-55,630) ng/mL. The t(15;17)(q22;q12 and PML-RARA fusion was found in all patients by chromosome analysis and/or multiplex reverse transcriptase-polymerase chain reaction (RT-PCR), with turnaround times of 8 (2-19) d and 7 (2-13) d, respectively. All patients received induction chemotherapy: all-trans retinoic acid (ATRA) alone (N=11, 26%), ATRA+idarubicin (N=25, 58%), ATRA+cytarabine (N=3, 7%), ATRA+idarubicin+cytarabine (N=4, 9%).

Conclusion

Since APL is a medical emergency and an accurate diagnosis is a prerequisite for prompt treatment, laboratory support to implement faster diagnostic tools to confirm the presence of PML-RARA is required.

Keywords Acute promyelocytic leukemia, PML-RARA, Immunophenotyping, Cytogenetic analysis, All-trans retinoic acid

Article

Original Article

Blood Res 2013; 48(1): 31-34

Published online March 31, 2013 https://doi.org/10.5045/br.2013.48.1.31

Copyright © The Korean Society of Hematology.

Current routine practice and clinico-pathological characteristics associated with acute promyelocytic leukemia in Korea

Sunhyun Ahn1, Joon Seong Park2, Seong Hyun Jeong2, Hyun Woo Lee2, Jun Eun Park3, Mi Hyang Kim4, Yang Soo Kim5, Ho Sup Lee5, Tae Sung Park6, Eunkyoung You6, Insoo Rheem7, Joowon Park7, JI Young Huh8, Myung Seo Kang8, and Sung Ran Cho1*

1Department of Laboratory Medicine, Ajou University School of Medicine, Suwon, Korea.

2Department of Hematology-Oncology, Ajou University School of Medicine, Suwon, Korea.

3Department of Pediatrics, Ajou University School of Medicine, Suwon, Korea.

4Department of Laboratory Medicine, Kosin University Gospel Hospital, Busan, Korea.

5Department of Internal Medicine, Kosin University Gospel Hospital, Busan, Korea.

6Department of Laboratory Medicine, School of Medicine, Kyung Hee University, Seoul, Korea.

7Department of Laboratory Medicine, Dankook University College of Medicine, Cheonan, Korea.

8Department of Laboratory Medicine, CHA Bundang Medical Center, CHA University, Seongnam, Korea.

Correspondence to: Correspondence to Sung Ran Cho, M.D., Ph.D. Department of Laboratory Medicine, Ajou University School of Medicine, San-5, Wonchun-dong, Yeoungtong-gu, Suwon 443-721, Korea. Tel: +82-31-219-5780, Fax: +82-31-219-5778, sungran@ajou.ac.kr

Received: August 24, 2012; Revised: November 28, 2012; Accepted: February 13, 2013

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Background

Acute promyelocytic leukemia (APL) can be life threatening, necessitating emergency therapy with prompt diagnosis by morphologic findings, immunophenotyping, cytogenetic analysis, or molecular studies. This study aimed to assess the current routine practices in APL and the clinico-pathologic features of APL.

Methods

We reviewed the medical records of 48 Korean patients (25 men, 23 women; median age, 51 (20-80) years) diagnosed with APL in 5 university hospitals between March 2007 and February 2012.

Results

The WBC count at diagnosis and platelet count varied from 0.4 to 81.0 (median 2.0)×109/L and 2.7 to 124.0 (median 54.5)×109/L, respectively. The median values for prothrombin time and activated partial thromboplastin time were 14.7 (11.3-44.1) s and 29 (24-62) s, respectively. All but 2 patients (96%) showed a fibrin/fibrinogen degradation product value of >20 µg/mL. The D-dimer median value was 5,000 (686-55,630) ng/mL. The t(15;17)(q22;q12 and PML-RARA fusion was found in all patients by chromosome analysis and/or multiplex reverse transcriptase-polymerase chain reaction (RT-PCR), with turnaround times of 8 (2-19) d and 7 (2-13) d, respectively. All patients received induction chemotherapy: all-trans retinoic acid (ATRA) alone (N=11, 26%), ATRA+idarubicin (N=25, 58%), ATRA+cytarabine (N=3, 7%), ATRA+idarubicin+cytarabine (N=4, 9%).

Conclusion

Since APL is a medical emergency and an accurate diagnosis is a prerequisite for prompt treatment, laboratory support to implement faster diagnostic tools to confirm the presence of PML-RARA is required.

Keywords: Acute promyelocytic leukemia, PML-RARA, Immunophenotyping, Cytogenetic analysis, All-trans retinoic acid

Table 1 . Demographic & laboratory characteristics of the 48 patients with acute promyelocytic leukemia..

a)While presence of PML-RARA was confirmed in 36 patients, PML-RARA isoform results were available in 25 cases..

Abbreviations: WBC, white blood cell; Hb, hemoglobin; PT, prothrombin time; aPTT, activated partial thromboplastin time; FDP, fibrin/fibrinogen degradation product; NSE, nonspecific esterase; RT-PCR, reverse transcriptase-polymerase chain reaction; ND, not done..

Table 2 . Additional chromosomal abnormalities in APL patients..

a)Two patients had both trisomy 8 and ider(17)(q10)t(15;17)(q22;q21), one patient had trisomy 8 and del(6)(q21q25), and one patient had trisomy 8 and add(6)(q27). b)Other abnormalities include t(2;14)(q11.2;q11.2)del(11)(q23)/t(7;14)(p10;q10),-14/16q-/inv(9)(p12q13)/del(6)(q21q25)/+21/+6(q27)/add(19)(p13.3)..

Table 3 . The lead time (in days) for reporting the results of diagnostic studies and initiating the induction therapy..


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