Lenalidomide in the management of eosinophilic dermatosis of hematological malignancy

Eosinophilic dermatosis of hematological malignancy is a paraneoplastic skin eruption associated with chronic lymphocytic leukemia and other B‐cell malignancies. It clinically resembles an insect bite reaction and it can precede the symptoms of the hematological malignancy or be related to a more aggressive course. Different treatments have been proposed, but partial response and recurrence are frequent. Herein, we describe a case of eosinophilic dermatosis associated with mantle cell lymphoma with remission after lenalidomide therapy.     

Staphylococcus aureus Bacteremia in Patients with Hematological Malignancies and/or Agranulocytosis

ABSTRACT. A total of 6 253 cases of Staphylococcus aureus bacteremia, including 274 (4.4%) endocarditis cases, were registered in Denmark in the period 1975–1984. Patients with hematological malignancies and/or agranulocytosis accounted for 479 of the bacteremia cases. The incidence of endocarditis in this group of patients was only 0.4% as compared to 4.7% in other patients with staphylococcal bacteremia (p<0.01). The lower incidence of endocarditis complicating bacteremia in these patients may justify a shorter course of therapy than usually recommended for suspected endocarditis. Patients with hematological malignancies and other patients with agranulocytosis had a higher mortality (49 and 46%, respectively) than other patients with S. aureus bacteremia (33%). The highest mortality was found in patients with multiple myeloma (71%, p<0.01), the lowest in patients with acute lymphocytic leukemia (28%, p<0.01). The higher mortality in these patients may indicate that empiric antibiotic regimens in granulocytopenic patients should include a specific anti-staphylococcal agent.     

Outcome of surgery in patients with hematological malignancies: A 12-year retrospective analysis

Background: Surgical intervention in patients with malignant hematological disorders is a major undertaking due to the expected risks of bleeding, infection and poor wound healing. Methods and materials: A retrospective study of patients treated at the Riyadh Armed Forces Hospital, Saudi Arabia between January 1991 and December 2002 was conducted. The results of patients with acute leukemia and lymphoma who underwent surgical procedures (study group) were compared with those of a control group composed of patients with the same spectrum of disorders treated over the same period of time and given the same treatment protocols but never required any surgery. Results: No single death occurred intraoperatively or in the immediate postoperative period due to surgical therapy per se. However, follow up of both groups of patients revealed a shorter long‐term survival and higher rates of relapse and severe invasive infections in the surgical group compared to the control group of patients. The mean survival for the study group was 1871 ± 307 days versus 3094 ± 279 days for the control group of patients (P = 0.0027). Thirty (75%) study patients suffered relapses of their malignant hematological disorders versus 23 (37.1%) control patients. Forty‐five relapses were encountered in the study group of patients (1.5 relapses per relapsed patient) versus 26 relapses in the control group (1.13 relapses per relapsed patient). Various infections occurred in 37 (92.5%) study patients and 32 (51.6%) control patients. Recurrent infections developed in 30 (75%) study patients and 22 (35.5%) control patients (P = 0.00008). Infections causing tissue invasion were encountered in 29 (72.5%) study patients and 22 (35.5%) control patients. Conclusion: Even major surgical procedures can be performed in patients with leukemia or lymphoma provided enough preparatory measures are made to minimize bleeding and infectious complications. Surgery may, however, be associated with long‐term complications such as a high incidence of relapse of the primary malignant hematological disorder and an increased rate of severe and invasive infections.     

自体造血干细胞移植治疗恶性血液病

为探讨恶性血液病的有效治疗方法,应用自体骨髓移植(ABMT)38例,自体外周血造血干细胞移植(ABSCT)13例,自体外周血造血干细胞与自体骨髓联合移植15例。治疗白血病54例,恶性淋巴瘤11例,多发性骨髓瘤1例。外周血造血干细胞采用化疗加多抗甲素或C—CSF动员。移植物采用微波、阿克拉霉素净化处理。结果:两组动员方案均有良好动员效果。ABSCT组及联合移植组造血功能恢复比ABMT组快(P<0.05),合并症少。66例中,45例仍呈持续缓解(CCR),中位CCR时间32(5～98)个月,复发21例。3年无病生存率及复发率分别为68.5％及26.1％。结果表明:自体造血干细胞移植是根治恶性血液病的有效手段。ABSCT及联合移植具有造血功能恢复快,合并症少等优点。微波和阿克拉霉素体外净化移植物是一种简便、有效的净化方法。     

Ellis‐van Creveld syndrome in a patient from Tanzania

We report an African infant with Ellis‐van Creveld (EVC) syndrome. EVC syndrome is a chondral and ectodermal dysplasia with autosomal recessive transmission. The baby presented with polydactyly, short limbs and atrioventricular septal defect, but was withdrawn from clinical follow up for the first year of life. Initial hematological abnormalities could not be explained and normalized later. EVC syndrome was confirmed by genetic analysis that showed two pathogenic mutations in the EVC2 gene, c.653_654del, p.Val218Glyfs*12 in exon 5, and c.2710C>T, p.Gln904* in exon 16. The variant c.653_654del; p.Val218Glyfs*12 in exon 5 has not been described before. Our review of medical literature suggested this is the first molecularly confirmed case of EVC syndrome in sub‐Saharan Africa.     

恶性血液病合并血流感染患者病原菌分布及其预后影响因素

 目的 分析恶性血液病合并血流感染患者的病原学特征及其预后影响因素。方法 选取2016年1月-2022年5月遵义医科大学附属医院血液内科的恶性血液病合并血流感染的住院患者为研究对象。根据患者发生血流感染30 d内的治疗结局分为生存组和死亡组。分析患者的病原学特征及预后情况,并采用单因素及logistic回归分析影响恶性血液病合并血流感染预后的危险因素。结果 共纳入185例患者,基础疾病以急性白血病为主(125例,67.6%)。共分离197株病原菌,革兰阴性菌109株(55.3%),其中大肠埃希菌55株(27.9%);革兰阳性菌86株(43.7%),其中人葡萄球菌24株(12.2%);真菌2株(1.0%)。大肠埃希菌中产超广谱β-内酰胺酶(EBSLs)菌株28株(50.9%);肺炎克雷伯菌中产EBSLs菌株2株(10.0%);耐甲氧西林的人葡萄球菌、表皮葡萄球菌、金黄色葡萄球菌的检出率分别为70.8%、71.4%、36.4%。单因素分析表明,年龄≥70岁、粒细胞缺乏持续时间≥7 d、未合理使用抗菌药物、合并心功能不全、合并急性肾功能不全、感染性休克、肺部感染患者的30天病死率较高,差异均有统计学意义(均P<0.05)。多因素logistic回归分析表明,粒细胞缺乏持续时间≥7 d[OR=3.306,95%CI(1.224~8.927)]、合并心功能不全[OR=6.291,95%CI(1.930~20.508)]、合并急性肾功能不全[OR=8.419,95%CI(2.198~32.241)]、感染性休克[OR=22.150,95%CI(3.639~134.806)]均为恶性血液病合并血流感染患者30天内死亡的独立危险因素(均P<0.05)。结论 恶性血液病合并血流感染最常见的病原菌中,革兰阴性菌以大肠埃希菌为主,革兰阳性菌以人葡萄球菌为主。影响恶性血液病合并血流感染患者预后的危险因素较多,缩短粒细胞缺乏持续时间,改善心功能不全、肾功能不全,积极控制感染性休克是减少恶性血液病合并血流感染患者30天内死亡的有效措施。     

自体外周血干细胞动员和采集的研究

目的　探索理想的血液肿瘤外周血干细胞动员方案。方法　选择 15例恶性血液病患者 ,14例应用米托蒽醌 (2 0mg/m2 ) 阿糖胞苷 (8g/m2 ) 粒细胞集落刺激因子 (G -CSF ,惠尔血 30 0 μg/d ,从白细胞 <1× 10 9/L开始至采集完成 ) (MAG) ;1例多发性骨髓瘤应用环磷酰胺 (4 g/m2 ) G -CSF(CG)。用CS - 30 0 0Plus血细胞分离机采集单个核细胞。处理血量约 2 0 0ml/kg ,历时 3～ 4h。直至CD34 细胞数 >4× 10 6/kg为止。 结果　 14例用MAG方案者中 ,一次采集即采到CD34 细胞 2× 10 6/kg以上者 11例 (79% ) ,其中 9例 (6 4% ,9/14)达 4× 10 6/kg以上。通过测定采集日外周血中CD34 细胞数可预测一次采集足量干细胞的可能性 (P <0 .0 0 1)。 2例部分缓解者动员后获完全缓解 ,1例Ph阳性小克隆和 1例AML1/ETO基因在动员后转阴。移植 14例可评价者中 10例存活至今 (3～ 5年 ) ,4例 (Ph 杂白、L2和L3型急淋、急单各 1例 )复发死亡。结论　MAG具有抗瘤和动员双重作用。外周血CD34 细胞数与采集成功与否相关性佳     

Saporin-S6: A Useful Tool in Cancer Therapy

Thirty years ago, the type 1 ribosome-inactivating protein (RIP) saporin-S6 (also known as saporin) was isolated from Saponaria officinalis L. seeds. Since then, the properties and mechanisms of action of saporin-S6 have been well characterized, and it has been widely employed in the construction of conjugates and immunotoxins for different purposes. These immunotoxins have shown many interesting results when used in cancer therapy, particularly in hematological tumors. The high enzymatic activity, stability and resistance to conjugation procedures and blood proteases make saporin-S6 a very useful tool in cancer therapy. High efficacy has been reported in clinical trials with saporin-S6-containing immunotoxins, at dosages that induced only mild and transient side effects, which were mainly fever, myalgias, hepatotoxicity, thrombocytopenia and vascular leak syndrome. Moreover, saporin-S6 triggers multiple cell death pathways, rendering impossible the selection of RIP-resistant mutants. In this review, some aspects of saporin-S6, such as the chemico-physical characteristics, the structural properties, its endocytosis, its intracellular routing and the pathogenetic mechanisms of the cell damage, are reported. In addition, the recent progress and developments of saporin-S6-containing immunotoxins in cancer immunotherapy are summarized, including in vitro and in vivo pre-clinical studies and clinical trials.