Park Joonhong, Chae Hyojin, Kim Myungshin, Lim Jihyang, Kim Yonggoo, Lee Jaewook, Chung Nak Gyun, Cho Bin, Kim Hack Ki, Lee Seok, Han Kyungja
Department of Laboratory Medicine, The Catholic University of Korea, College of Medicine, Seoul, Korea.
Korean J Lab Med. 2010 Dec;30(6):525-32. doi: 10.3343/kjlm.2010.30.6.525.
We evaluated the clinical significance of revised 2008 WHO classification needed to diagnose mixed phenotype acute leukemia (MPAL).
A total of 22 MPAL patients, previously diagnosed by applying the scoring system of the European Group for Immunological Classification of Acute Leukemias (EGIL) were reclassified based on the 2008 WHO classification.
In 2008 WHO classification, the number of monoclonal antibodies (mAbs) required for assigning more than one lineage was markedly decreased, from 26 to 11, compared with that of EGIL. Seventeen of the 22 MPAL patients were reclassified as MPAL with following details: 6 MPAL with t(9;22)(q34;q11.2); BCR-ABL1, 1 MPAL with t(v;11q23); MLL rearranged, 7 MPAL, B/Myeloid, not otherwise specified (NOS) and 3 MPAL, T/Myeloid, NOS. Five patients were excluded from MPAL in the revised classification: 4 cytoplasmic myeloperoxidase (cMPO)-negative and 1 CD19-negative. The failure of complete remission achievement and occurrence of relapse were associated with poor prognosis (P=0.0002 and P=0.009, respectively). But the presence of Philadelphia chromosome was not significantly related with patient outcome (P=0.082). One patient with cCD79a, CD20, CD38, cMPO and CD15, whose diagnosis was reclassified from MPAL to AML has survived during the study period.
Because of decreased number of mAbs needed, it is possible that acute leukemia panel is designed to include all mAbs required to diagnose MPAL according to 2008 WHO classification. When diagnosing MPAL, it is critical to figure out positivity in either cMPO or CD19, and AML expressing more than 2 lymphoid antigens are considered as MPAL.
我们评估了2008年世界卫生组织(WHO)修订分类对于诊断混合表型急性白血病(MPAL)的临床意义。
对22例先前应用急性白血病欧洲免疫分类组(EGIL)评分系统诊断的MPAL患者,依据2008年WHO分类进行重新分类。
在2008年WHO分类中,与EGIL相比,用于指定多个谱系所需的单克隆抗体(mAb)数量显著减少,从26种降至11种。22例MPAL患者中有17例被重新分类为MPAL,具体情况如下:6例MPAL伴t(9;22)(q34;q11.2);BCR-ABL1,1例MPAL伴t(v;11q23);MLL重排,7例MPAL,B/髓系,未另行指定(NOS),3例MPAL,T/髓系,NOS。5例患者在修订分类中被排除出MPAL:4例胞质髓过氧化物酶(cMPO)阴性,1例CD19阴性。未达到完全缓解和复发与预后不良相关(分别为P=0.0002和P=0.009)。但费城染色体的存在与患者预后无显著相关性(P=0.082)。1例具有胞质CD79a、CD20、CD38、cMPO和CD15的患者,其诊断从MPAL重新分类为急性髓系白血病(AML),在研究期间存活。
由于所需mAb数量减少,有可能设计急性白血病检测组合以纳入根据2008年WHO分类诊断MPAL所需的所有mAb。诊断MPAL时,确定cMPO或CD19的阳性至关重要,表达超过2种淋巴系抗原的AML应被视为MPAL。