急性巨核细胞白血病三例

急性巨核细胞白血病（ＡＭＫＬ）较少见，１９９０至１９９７年我所共诊断急性白血病１２２７例，其中经形态学、免疫学、电镜确诊ＡＭＫＬ者仅３例。为提高对ＡＭＫＬ的认识，现将我所收治３例报道如下。一、病例和方法１临床资料：３例中男２例，女１例，年龄４４～７...     

小儿急性巨核细胞白血病的诊断

急性巨核细胞白血病是一种特殊类型的白血病 ,被列入了急性髓细胞白血病的Ｍ7型。我们于1 998～ 2 0 0 0年诊断了 5例Ｍ7患者 ,发现其临床表现以及原始巨核细胞的细胞形态学、细胞化学染色、血小板单克隆抗体检查以及病理学活检有区别于其他ＡＭＬ患者及原始细胞的特点。现报告如下。1　资料与方法1 .1　临床资料本组 5例 ,男 3例 ,女 2例。年龄分别为 1岁 6个月、1 0个月、1 2岁、8岁及 3岁 5个月。就诊时有进行性贫血 ,发热 ,无出血 ,3例肝轻度肿大 ,2例脾轻度肿大 ,3例淋巴结肿大 (详见表 1 )。1 .2　检查方法参照ＦＡＢ的诊断标准 ,在光…     

慢性粒细胞白血病急性巨核细胞白血病变1例

慢性粒细胞白血病(CML)急粒变、急淋变较为常见,但急性巨核细胞白血病变较罕见,我院最近收治1例,报道如下.     

急性巨核细胞白血病易见浆细胞岛一例

1病例介绍患者男，50岁，因间断高热20天，于1994年11月10日入院。患者于入院前20天无明显诱因开始发烧，并伴左侧腹股沟淋巴结肿大，体温高达40℃。血象示“三系减少”，为进一步明确诊断转入我院。体检：T39．4℃BP16／11kPa，轻度贫血貌，全身皮肤粘膜无出血，表浅淋巴结无肿大。骨骼压痛（－），肝脾未及。实验室检查：Hb80g／L，WBC1．6×109／L，PLT95×109／L；分类：原始巨核细胞0．20，淋巴细胞0．46，中性分叶核粒细胞0．34。ESR148mm／h，肥达反应阴性，总蛋白60．2g／L，白蛋白30．0g／L球蛋白30．2g／L，白球比0．99，…     

慢性粒细胞白血病急性巨核细胞白血病变1例

慢性粒细胞白血病(CML)急粒变、急淋变较为常见,但急性巨核细胞白血病变较罕见,我院最近收治1例,报道如下.     

慢性粒细胞白血病急变为急性巨核细胞白血病1例

急性巨核细胞白血病(AMLK)占所有急性髓系白血病中发生率的0.5%~1.2%,其中慢性粒细胞白血病(CML)转化为AMLK的比例报道不一。现报道1例以骨髓纤维化(MF)首诊的AMLK,最终通过电镜、免疫分型以及分子生     

急性白血病及其治疗后骨髓巨核细胞的变化

急性白血病(AL)患者骨髓巨核细胞(MK)的观察已见报道,但均反映初发时MK数量及形态改变.AL治疗后MK的情况,文献报道甚少.我们观察了89例AL患者初发及治疗过程中骨髓MK的变化,结果报告如下:1 材料与方法     

急性白血病患者骨髓巨核细胞变化的临床意义

近10年来关于巨核细胞(MK)不减少的急性白血病(AL)很少报道，我们通过2年的观察，发现绝大部分AL初治时表现为MK减少，仍有一小部分表现为MK不减少，这一部分患者在疾病类型、临床表现、化疗效果、预后等与MK减少的AL患者有明显的差异，现将两者的比较总结如下。     

成人急性淋巴细胞白血病预后因素分析

目前,成人急性淋巴细胞白血病（ALL）的治疗依然是我们面临的挑战。虽然过去的几10 a里其疗效有了长足进步,但60岁以下的成人ALL长期存活率仅为30%～40%。根据预后因素危险分层进行个体化治疗是治疗成功的关键,而成人ALL预后因素随着研究的深入逐年变化,随之也带动治疗的更新。对于初诊的成人ALL患者,其预后因素可     

Target cell of leukemic transformation in acute megakaryoblastic leukemia

Acute megakaryoblastic leukemia (AMkL) is a newly defined acute leukemia in which the differentiation of proliferating blasts is arrested at the megakaryocytic precursor stage. In order to clarify whether a target cell of leukemic transformation in AMkL is a cell committed to megakaryocytic lineage, or a multipotential stem cell, we examined AMkL patients with regard to: a) the presence of myelodyplastic features in residual erythroid and granulocytic cells, b) coexistence of myeloperoxidase (MPO)-positive blasts with megakaryoblasts, and c) the presence of the same chromosomal abnormality in erythroid and granuloid colony-forming cells as seen in megakaryoblasts. Regarding the former two items, results were compared with those from megakaryoblastic crisis of chronic myelocytic leukemia (CML-MkBC) and transient myeloproliferative disorder in Down syndrome (DS-TMD), which are thought to be multipotential stem cell disorders. Among 18 patients with AMkL, three, all complicating myelofibrosis, had marked myelodysplastic changes of erythroid series and/or granulocytic series. In 4 out of 7 patients with CML-MkBC, 5 out of 8 patients with DS-TMD, and 7 out of 18 patients with AMkL, MPO-positive blasts, even though rare, were observed in addition to PPO-positive blasts. All except one of these patients with AMkL also showed complicating myelofibrosis. In one case of AMkL with myelofibrosis, chromosomal analysis of cultured cells of individual colonies revealed that all the analysable metaphases from both CFU-GM and BFU-E had the same chromosomal abnormality as megakaryoblasts. This study has clarified that a considerable proportion of AMkL cases, particularly those with complicating myelofibrosis or showing acute myelofibrosis, arise against the background of a multipotential stem cell disorder, even if blasts are exclusively megakaryocytic in phenotype.     

高白细胞急性髓系白血病的临床分析

目的:探讨高白细胞急性髓系白血病(HAML)患者的临床特征、预后并与同期非高白细胞急性髓系白血病(NHAML)患者进行比较。方法:观察随访66例HAML(WBC≥100×109/L)病例(非M3型),分析并发症、早期死亡(early death,ED)、治疗反应、远期预后等,并与同期随机选择的202例NHAML(WBC<100×109/L)病例(非M3型)进行比较。结果:①HAML病例发生低钾血症、出凝血异常、感染、肺白细胞淤滞、脾脏肿大、淋巴结肿大等并发症的概率高于NHAML病例(P<0.05)。HAML病例FAB分型以M4/M5型为主。②HAML病例ED 4例(6.1%),ED与出凝血异常(P<0.01)、肺白细胞淤滞(P<0.01)、CNS白细胞淤滞症状(P<0.01)相关。③HAML病例CR率66.7%,且M4/M5型CR率低。HAML病例3年DFS率为45.7%,3年OS率为32.5%。完全缓解(CR)率与年龄、是否为M4/M5型相关(P<0.05)。3年无病生存率(DFS)与是否>50岁及染色体分组相关(P<0.01)。3年总生存率(OS)与是否CR相关(P<0.05)。NHAML病例CR率为90...     

Granulocytic sarcoma of megakaryoblastic differentiation in the lymph nodes terminating as acute megakaryoblastic leukemia in a case of chronic idiopathic myelofibrosis persisting for 16 years

Abstract: A 43‐yr‐old Japanese woman presented with mild anemia, leukocytosis and splenomegaly in May 1984. Splenomegaly and anemia gradually progressed. Sixteen years later, in October 2000, she developed inguinal lymphadenopathy. Biopsy of the lymph node revealed infiltration of blasts, megakaryocytes, fibroblasts and myeloid cells. Large blasts with basophilic cytoplasm with cytoplasmic projections appeared in the peripheral blood. These blasts were negative in peroxidase stain, positive in acid phosphatase and weakly positive in periodic acid‐Schiff stain. Immunohistochemical staining with monoclonal antibodies revealed that these blasts were positive with anti‐CD41 (glycoprotein IIb/IIIa) and negative with other monoclonal antibodies. So diagnosis of granulocytic sarcoma in megakaryoblastic transformation from chronic idiopathic myelofibrosis was made. A cytogenetic study revealed that bone marrow cells were 46,XX del(13)(q?) initially and additional abnormalities including der(5,5,11)(q11;q13)ins(5;?)(q11;?) were found when she developed megakaryoblastic transformation. Granulocytic sarcoma of megakaryoblastic transformation from chronic idiopathic myelofibrosis is a rare event. Immunophenotyping with monoclonal antibody for CD41(glycoprotein IIb/IIIa) confirmed the diagnosis.     

老年急性白血病340例临床分析

目的分析老年急性白血病的分布及预后情况。方法选取2009年9月至2013年5月在我院未经治疗的、年龄大于60岁的急性白病患者340例,对其发病年龄、预后危险因素、治疗、生存时间等指标进行分析。结果340例60岁以上老年急性白血病患者中,中位发病年龄68岁,急性淋巴细胞白血病（ALL）24例（7.06%）;在急性髓细胞白血病（AML）314例中,初发AML（de novo AML）250例（79.62%）。急性早幼粒细胞白血病（APL）仅9例。初发AML （非APL）中细胞遗传学和分子生物学预后较好的34例（14.11%）,预后差的61例（25.31%）,随着年龄增加,预后较好的比例降低,而预后差的比例升高。在有生存资料的患者93例中,85例选择了化疗。年龄也对完全缓解（CR）率、总体生存有显著影响。化疗后获得CR的患者生存明显优于未获得CR的患者。结论老年急性白血病患者预后差,如何提高总体生存仍然是一个难题。     

Pure erythroid leukemia subsequent to acute myelomonocytic leukemia: A case report

Rationale:Pure erythroid leukemia is a rare subcategory of acute myeloid leukemia characterized by predominant immature erythroid population. Its occurrence subsequent to acute myelomonocytic leukemia has not been reported before. We reported this rare case to call attention because it may pose a diagnostic challenge.Patient''s concerns:A 54-year-old female patient presented to our hospital in March 2018 with symptoms of easy fatigability.Diagnosis:Bone marrow aspiration was hypercellular showing 67.2% blasts mainly including moderate myeloblasts and monoblasts. There was mild dysplasia with some cells having round, oval, or bizarre nuclei which containing 1 to 3 nucleolus. Erythroid lineage was hypoplasia and mature erythrocytes were generally normal. Conventional cytogenetics of bone marrow cells revealed complex karyotype (44, XX, del (5) (q14q34) del (5) (q14q34), del (14) t (11;14) (q10; q10), −16, del (17), −18[10]).Interventions:The patient was treated with second line chemotherapy but did not respond.Qutcomes:She died of cardiopulmonary failure 19days after starting of therapy.Lessons:This unexpected and relatively uncommon occurrence was associated with a universally rapid and fatal clinical course with survival measured in <2 months despite intensive chemotherapy. We call attention to this rare phenomenon because it may pose a diagnostic challenge.     

Very early onset of an acute myeloid leukemia in an adult patient with B‐cell lymphoblastic leukemia

We report on a case of a 30‐year‐old male with acute B‐lymphoblastic leukemia (B‐ALL) with immunophenotype CD19⁺, CD22⁺, CD20⁺, CD10⁺, with aberrant expression of CD13 and CD117, and IgH gene rearrangements. Three months after treatment with GMALL‐2003 and Ida/FLAG protocols bone marrow showed predominance of blasts with myeloid morphology and phenotype MPO⁺, CD13⁺, CD33⁺, CD64⁺, CD15⁺, CD56⁺, EVI‐1 gene overexpression and lack of IgH rearrangements. The case is the first report of a very early emergence of myeloid leukemia during the induction treatment for B‐ALL in an adult patient. Different pathogenetic mechanisms are discussed – clonal evolution or selection, lineage switch or development of a de novo or therapy‐induced leukemia.     

Molecular lesions in childhood and adult acute megakaryoblastic leukaemia

While acute megakaryoblastic leukaemia (AMKL) occurs in children with (DS-AMKL) and without (paediatric non-DS-AMKL) Down syndrome, it can also affect adults without DS (adult non-DS-AMKL). We have analysed these subgroups of patients (11 children with DS-AMKL, 12 children and four adults with non-DS-AMKL) for the presence of molecular lesions, including mutations and chromosomal abnormalities studied by sequencing and single nucleotide polymorphism array-based karyotyping, respectively. In children, AMKL was associated with trisomy 21 (somatic in non-DS-AMKL), while numerical aberrations of chromosome 21 were only rarely associated with adult AMKL. DS-AMKL was also associated with recurrent somatic gains of 1q (4/11 DS-AMKL patients). In contrast to trisomy 21 and gains of 1q, other additional chromosomal lesions were evenly distributed between children and adults with AMKL. A mutational screen found GATA1 mutations in 11/12 DS-AMKL, but mutations were rare in paediatric non-DS-AMKL (1/12) and adult AMKL (0/4). JAK3 (1/11), JAK2 (1/11), and TP53 mutations (1/11) were found only in patients with DS-AMKL. ASXL1, IDH1/2, DNMT3A, RUNX1 and CBL mutations were not found in any of the patient group studied, while NRAS mutation was identified in two patients with paediatric non-DS-AMKL.