Dutta Pankhi, Sazawal Sudha, Kumar Rajat, Saxena Renu
Department of Haematology, All India Institute of Medical Sciences, New Delhi, India.
Indian J Pathol Microbiol. 2008 Jul-Sep;51(3):437-9. doi: 10.4103/0377-4929.42555.
Acute promyelocytic leukemia (APML) is a well-characterized malignancy with typical clinico-hematological and molecular features. However, Indian data on this malignancy are limited. This study was conducted to determine the clinico-hematological profile of APML in India. Thirty-five patients with APML presenting to Hematology Department, AIIMS, New Delhi, between July 2003 and June 2005 were evaluated for presenting clinical features, hemogram, peripheral smear, bone marrow morphology and cytochemistry. Reverse transcriptase PCR (RT-PCR) for PML-RARalpha was done in all cases. Male-to-female ratio was 0.9:1 (males--17 and females--18) with median age 25 years (range 11-57 years). Presenting features included anemia, bleeding, fever, gum hypertrophy and scrotal ulceration. All cases showed hypergranular abnormal promyelocytes. Median hemoglobin was 6.3 g/dL (range - 3.0-9.0 g/dL), total leukocyte count (TLC) was 33.88 x 10(9) /L (range - 1-170 x 10(9) /L). Platelet count was 28 x 10(9) /L (range - 4-170 x 10(9) /L). All cases were positive for myeloperoxidase and sudan black (SB), whereas 60% cases also showed non specific esterase (NSE) positivity with 40% cases being fluoride sensitive. RT-PCR showed PML-RARalpha in 33/35 cases with the bcr3 isoform being present in 24/33 positive cases (72.7%). The two cases negative for PML-RARalpha showed typical morphology and responded to ATRA. On statistical analysis, no correlation was found between bcr isoform and TLC, platelet count, age sex and early death. Unusual features included gum hypertrophy and scrotal ulceration at presentation and high median presenting TLC (33.8 x 10(9) /L). There was, however, no microgranular variant. Another interesting feature was a high incidence of NSE positivity (60%), which was fluoride sensitive in 40%. Moreover, the bcr3 isoform was significantly overexpressed (72.7%) in comparison to other studies. APML in India has certain unusual features, which may reflect a different biology.
急性早幼粒细胞白血病（APML）是一种具有典型临床血液学和分子特征的特征明确的恶性肿瘤。然而，关于这种恶性肿瘤的印度数据有限。本研究旨在确定印度APML的临床血液学特征。对2003年7月至2005年6月期间就诊于新德里全印度医学科学研究所血液科的35例APML患者进行了评估，包括其呈现的临床特征、血常规、外周血涂片、骨髓形态学和细胞化学检查。所有病例均进行了PML-RARα的逆转录酶聚合酶链反应（RT-PCR）。男女比例为0.9:1（男性17例，女性18例），中位年龄25岁（范围11 - 57岁）。呈现的特征包括贫血、出血、发热、牙龈肥大和阴囊溃疡。所有病例均显示颗粒增多的异常早幼粒细胞。中位血红蛋白为6.3 g/dL（范围 - 3.0 - 9.0 g/dL），白细胞总数（TLC）为33.88×10⁹/L（范围 - 1 - 170×10⁹/L）。血小板计数为28×10⁹/L（范围 - 4 - 170×10⁹/L）。所有病例髓过氧化物酶和苏丹黑（SB）均呈阳性，而60%的病例非特异性酯酶（NSE）也呈阳性，其中40%的病例对氟化物敏感。RT-PCR显示35例中有33例存在PML-RARα，24/33例阳性病例（72.7%）存在bcr3亚型。2例PML-RARα阴性病例表现出典型形态并对全反式维甲酸（ATRA）有反应。经统计学分析，未发现bcr亚型与TLC、血小板计数、年龄、性别及早期死亡之间存在相关性。不寻常的特征包括就诊时牙龈肥大和阴囊溃疡以及较高的就诊时TLC中位数（33.8×10⁹/L）。然而无微颗粒变异型。另一个有趣的特征是NSE阳性发生率较高（60%），其中40%对氟化物敏感。此外，与其他研究相比，bcr3亚型显著过度表达（72.7%）。印度的APML具有某些不寻常的特征，这可能反映了不同的生物学特性。
