伴嗜酸细胞明显增多的慢性粒细胞白血病急粒变1例

1病例介绍 患者,男性,39岁.2002年9月因四肢酸痛,下肢皮疹伴发热20余天入院,既往体健.无嗜生肉、生鱼史及疫水接触史.体检:四肢散在瘀斑,右下肢可见2 cm×2 cm红色斑块,伴瘙痒,无疼痛.     

是慢性嗜酸粒细胞白血病还是髓系／淋系肿瘤伴嗜酸粒细胞增多？

 2008年WHO将有PDGFRA、PDGFRB或PGFR1重排的慢性嗜酸粒细胞白血病（CEL）分类为髓系／淋系肿瘤伴嗜酸粒细胞增多,而将无这些异常但有其他克隆性异常的CEL分类为CEL不另分类（CEL-NOS）。文章阐述了作者的意见。     

急性淋巴细胞白血病伴嗜酸粒细胞增多1例报告

2000年1月我院门诊确诊一例急性淋巴细胞白血病伴嗜酸粒细胞增多(ALLEo)的患者,因病例罕见和细胞形态特殊,故报告如下: 患者,男,12。以心悸、呼吸困难半月余为主诉就诊。重度贫血外貌,皮肤粘膜无出血点,全身浅表淋巴结无肿大,胸骨压痛阳性,心率快,心律不齐,左房室增大,肝、脾肋缘下可触及。胸部正位片见斑片状阴影,心电图示T波倒置。Hb:60g/L,WBC:14.3×10~9/L,BPC:108×10~9/L,原幼淋巴0.11,淋巴0.63,嗜酸0.09,分叶0.17。骨髓增生Ⅱ级,原淋0.22,幼淋0.195。原始细胞大小不等、以小为主,多为高核浆比,染色质较粗、排列紧密,核仁隐显不一,多为2～4个,胞浆蓝色。该类细胞POX(-)、PAS(±)、NSE(-),涂抹细     

慢性粒细胞白血病伴戈谢细胞增多一例

患者男性,45岁,2004年7月21日因发现白细胞高18年就诊。患者18年前因皮肤感染迁延不愈,外院查血常规示白细胞高于40×109/L,血小板和红细胞具体数值不详,骨髓细胞检查示:骨髓有核细胞增生极度活跃,粒∶红=6.5∶1,粒细胞系占0.775,NAP:阴性,考虑为慢性粒细胞白血病(CML),给予白消安、靛玉红交替服用,白细胞控制在10×109/L以下。于1997年10月复查骨髓细胞示:骨髓有核细胞增生明显活跃,粒∶红=5.4∶1,粒系明显增生,占0.76,中幼粒比值明显增高,杆状核粒细胞胞体增大,其他细胞形态大致正常;红细胞系增生偏低,全片见巨核细胞7个,后改用羟基脲…     

腹痛、多发血栓、嗜酸粒细胞增多综合征

以腹痛起病,伴多器官栓塞、嗜酸粒细胞增多病例骨髓检查嗜酸粒细胞比例增高。皮肤活检：嗜酸粒细胞浸润。抗中性粒细胞胞浆抗体阴性,FIP1L1/PDGFRα融合基因阴性的嗜酸粒细胞增多综合征,属骨髓增殖型疾病。骨髓活检及受累组织器官的病理检查对鉴别诊断有重要价值。需重视以腹痛为首发、伴血栓形成的类似病例,如有2个器官受累时需高度警惕该疾病及更多器官受累。     

嗜酸粒细胞白血病诊断标准探讨

目的：探讨嗜酸粒细胞白血病的诊断标准。方法：回顾性分析了４例患者的临床资料,同时对有关文献进行了复习。结果：Ｅｏｌ是嗜酸粒细胞恶性增殖性疾病,随访２例治疗后死亡,１例ＣＲ,１例出出院不详。结论：建议分型诊断标准为：（１）急性嗜酸粒细胞白血病;（１）外周血嗜酸粒细胞持续增高,并出现不成熟嗜酸粒细胞和／或不成熟粒细胞,有进行性血红蛋白和血小板减少;（２）骨髓原始粒细胞≥５％,各阶段嗜酸粒细胞增多, ≥     

慢性粒细胞白血病伴骨髓坏死1例

慢性粒细胞白血病伴骨髓坏死１例许洪志，徐功立，侯林江，张福东，彭翠兰慢性粒细胞白血病（ＣＭＬ）伴骨髓坏死在临床上较少见，近期我院收治１例，现结合文献报告如下。患者男，４４岁，农民，因乏力伴左上腹不适２个月于１９９５年７月１５日首次入院。Ｔ３６．８℃，...     

35例慢性粒-单核细胞白血病细胞形态学特征和染色体核型分析

 目的　进一步认识慢性粒-单核细胞白血病（CMML）细胞形态学和染色体核型特征。方法　回顾分析35例CMML患者的血象、骨髓象、细胞化学铁染色,18例染色体核型分析。结果　35例患者外周血白细胞（WBC）＞10.0×109／L 27例（87.3 %）,血红蛋白（Hb）浓度减低33例（94.3 %）,血小板（Plt）＜100×109／L 23例（65.7 %）。成熟单核细胞绝对值在（1.18～44.2）×109／L范围内。易见幼稚粒细胞者33例（94.3 %）,可见幼稚单核细胞者9例（25.7 %）。骨髓增生极度活跃或明显活跃28例（80 %）。分类中粒系+单核系≥0.70者20例（57.1 %）,可见幼稚单核细胞24例（68.6 %）。表现为一系或两系以上病态造血26例（74.3 %）。18例患者染色体核型分析,染色体异常5例（14.3 %）。结论　该组CMML的患者,血象以WBC增高、Hb和Plt同时减低明显增多为特点。易见幼稚粒细胞,单核细胞绝对值明显增高。骨髓增生异常综合征（MDS）伴单核细胞增多（MDS-CMML）的患者明显比具有骨髓增殖性疾病特征（MPD-CMML）的患者多见。CMML的患者染色体有核型异常,但无特异性,Ph染色体阴性。     

青黄胶囊治疗慢性嗜酸性粒细胞白血病1例

1 病例介绍 患者,男,31岁,汉族.主因乏力、消瘦4年,间断咳嗽、气促2年,浮肿6个月,面瘫7天于2002年1月10日入我院.缘于1998年10月因头晕、乏力,进行性消瘦就诊于湖南省人民医院,查血常规WBC15.2×109/L HGB 103 g/LPlt189×109/L,嗜酸性粒细胞比例增高为64%;B超示:肝脾肿大;骨穿示:有核细胞增生明显活跃,粒系嗜酸性粒细胞各阶段比值均明显增高,占59%.考虑:"嗜酸性粒细胞增多症".     

Cardiac tamponade in chronic myelomonocytic leukemia: a case report.

Serous effusion is a rare complication of chronic myelomonocytic leukemia. We present a patient with chronic myelomonocytic leukemia who developed a massive pericardial effusion with cardiac tamponade as early manifestations. Numerous clumps of monocytes were observed in the effusion. The patient obtained rapid relief following the intravenous administration of etoposide (100 mg daily for 5 days). Few reports have documented details of such a case.     

A case of sudden hearing loss during treatment with decitabine for chronic myelomonocytic leukemia

Chronic myelomonocytic leukemia (CMML) was initially classified in the category of myelodysplastic syndrome (MDS), but it is now categorized by the 2001 World Health Organization (WHO) classification in a separate nosological group of MDS. Unlike chronic myeloid leukemia (CML), the bone marrow morphology in CMML demonstrates prominent dysplastic changes in at least two of the three myeloid lineages. A 73-year-old male patient was brought to division of hematology for evaluation of leukocytosis. He was diagnosed with CMML and treated with decitabine. The hearing impairment had arisen during the third cycle of decitabine. To our knowledge, this is the first case that idiopathic sudden hearing loss (SHL) occured in CMML patients during treatment with decitabine.     

Chronic myelomonocytic leukemia coexisting with B-cell chronic lymphocytic leukemia

Coexistence of B-cell chronic lymphocytic leukemia (B-CLL) and chronic myelomonocytic leukemia (CMML) is an unusal event, and to our knowledge, only four such cases have been reported in the literature. We report a 68-year-old white woman in whom these two diseases were diagnosed concomitantly. The diagnosis was made on the basis of peripheral blood count, morphology and immunophenotyping, and bone marrow cytology and histology. Interphase FISH analysis detected a 13q14.3 deletion in lymphocytes nuclei and no such abnormality in monocytes nuclei. The PCR analysis of IgH gene rearrangement in the bone marrow, as well as the peripheral blood lymphocytes, showed two different monoclonal IgH configurations as the result of biallelic clonal rearrangement of IgH genes suggesting an origin of lymphocytes from B-cell progenitors. The patient was originally treated with prednisone 1 mg/kg/day because of progressive significant thrombocytopenia, without improvement. Subsequently, she received one course of cladribine (2-CdA). Significant reduction of lymphocytes in the peripheral blood was observed. However, rapid increase of monocytes was seen shortly after the 2-CdA treatment. Subsequently, she received hydroxyurea (1.5 g/day) without hematological improvement. The patient died in January 2003, three months after diagnosis because of progression of both leukemias and associated pneumonia. Possible etiopathogenic relationship between both disorders is discussed.     

Angiolymphoid hyperplasia with eosinophilia — A case report

Angiolymphoid hyperplasia with eosinophilia (ALHE) is a distinctive vascular tumor presenting as isolated or grouped papules or plaques or nodules over skin in head and neck. We, hereby, present a case of ALHE affecting the base of tongue-a rare site of involvement.     

Philadelphia chromosome-negative chronic myelogenous leukemia and chronic myelomonocytic leukemia

Philadelphia chromosome (Ph)-negative chronic myelogenous leukemia (CML) and chronic myelomonocytic leukemia (CMML) are heterogeneous disorders characterized by various degrees of proliferation, dysplasia, maturation arrest, and monocytosis. In this article, the clinical, laboratory, molecular, and therapeutic aspects of the disease entities are reviewed.     

Oral idarubicin in patients with late chronic phase chronic myelogenous leukemia or chronic myelomonocytic leukemia

Patients with chronic myelogenous leukemia (CML) who have failed or cannot receive interferon alpha (IFN-alpha) based regimens or patients with advanced chronic myelomonocytic leukemia (CMML) have very limited current therapeutic options. Hence, there is a need to develop new strategies for these patients. This study was undertaken to determine the efficacy and toxicity of a chronic low-dose oral idarubicin regimen in these patients as positive data has been generated on this agent in shorter schedules given to patients with other hematological malignancies. Eighteen patients were treated on study. The starting dose of oral idarubicin was 2 to 5 mg/m2 daily depending in initial WBC count. This dose was escalated in the absence of Grade 4 myelosuppression or Grade 3 or 4 extramedullary toxicity. Oral idarubicin was given daily for 28 days followed by a 21 day break off treatment in repeated cycles until there was evidence of disease progression or intolerable toxicity. The dose of idarubicin was adjusted, at 2-week intervals, by 25% to maintain a white blood cell (WBC) count between 2 and 4x10(9)/L and a platelet count of >75x10(9)/L. The dose was reduced by 25% for grade 2 extramedullary toxicity and held until toxicity resolved to grade 2 or better for grade 3 toxicity. Oral idarubicin was then restarted at 75% of the initial dose. Five out of 14 CML patients achieved a complete hematologic remission. No CMML patient responded (median survival 3 months). The overall median survival was 24 months. CML patients had a median survival of 28 months. Major toxicities (myelosuppression, gastrointestinal, cardiac) were infrequent with a median cumulative dose of 1110 mg/m2 (range 54-9750). Five patients have received oral idarubicin for > 1 year with no overt cardiotoxicity, reaching median cumulative dose of 2756 mg/m2 (range 2550-9750) which is higher than those documented in prior studies. We conclude that oral idarubicin is sufficiently safe and active to warrant phase II studies investigating it as part of interferon-based regimens in patients with advanced CML.     

Activity of azacitidine in chronic myelomonocytic leukemia

BACKGROUND:

Hypomethylating drugs are useful in the management of myelodysplastic syndrome (MDS). Two of these drugs, azacitidine and decitabine, have received FDA approval for the treatment of MDS and chronic myelomonocytic leukemia (CMML). However, phase 2 and 3 studies that assessed these agents in MDS included only a small number of patients with CMML. The objective of this study was to evaluate the efficacy and safety of azacitidine in the treatment of CMML.

METHODS:

The records of thirty‐eight patients diagnosed with CMML and treated with azacitidine at our institution were reviewed. Azacitidine was administered at 75 mg/m²/day for 7 days or 100 mg/m²/day for 5 days every 4 weeks. Patients who received at least 1 cycle of the drug were considered evaluable for response.

RESULTS:

Response was assessed by the modified International Working Group (IWG) criteria. The overall response rate was 39% (14 of 36); complete response (CR) rate was 11% (4 of 36); partial response (PR) rate was 3% (1 of 36); hematologic improvement (HI) was 25% (9 of 36). The median overall survival was 12 months. There was a statistically significant overall survival advantage in responders compared with nonresponders: 15.5 months versus 9 months, respectively (P = .04). Treatment was generally well tolerated. One of 2 patients had complete resolution of a skin rash that was due to monocytic infiltration.

CONCLUSIONS:

Azacitidine is active in the treatment of CMML. The therapy‐associated toxicity is acceptable. Our results support further investigation of azacitidine in CMML, particularly in combination with other agents. Cancer 2011;. © 2010 American Cancer Society.     

Pericardial effusion in chronic myelomonocytic leukemia (CMML): a case report and review of the literature

Pericardial effusion is a rare but potentially life-threatening complication of chronic myelomonocytic leukemia. A case of a 70-year-old female with CMML is reported, who developed a severe pericardial effusion after an initially stable course of disease. She underwent pericardiocentesis and subsequent intracardial instillation of mitoxantrone with poor response. Subxiphoid pericardiotomy was successfully performed. Leukocytosis did not respond to hydroxyurea. Because of decreasing performance status of the patient systemic chemotherapy was not applied and the patient died within 2 weeks. A review of the literature shows that there is no effective treatment for pericardial effusion in CMML, and some common features may be noted.