白细胞清除术联合化疗24例高白细胞急性白血病的疗效观察

目的观察白细胞清除术联合化疗对高白细胞急性白血病的治疗效果。方法白细胞清除术联合化疗治疗24例高白细胞急性白血病患者。结果 24例高白细胞急性白血病患者经白细胞清除术后化疗,缓解率达63．3％,早期死亡率4．5％。结论白细胞清除术联合化疗治疗高白细胞急性白血病是目前较先进的一种方法。     

白细胞单采术在高白细胞急性白血病诱导治疗中的作用

目的 探讨白细胞单采术联合化疗在高白细胞白血病（HLAL）治疗中的作用及注意事项。方法 使用CS-3000血细胞分离机对37例高白细胞急性白血病患者进行白细胞单采,术后进行化疗,观察疗效,并与同期HLAL单纯化疗的疗效相比较。结果 白细胞单采术后患者外周血白细胞数显著下降,在进行化疗后比单用化疗疗效好。结论 白细胞单采能迅速降低白细胞数,提高化疗缓解,是治疗高白细胞白血病的重要辅助方法。     

白血病细胞清除术加用联合化疗治疗高白细胞急性白血病

白血病细胞清除术加用联合化疗治疗高白细胞急性白血病蔡大利高峰肖卫国翟明高白细胞急性白血病（ＨＬＡＬ）病情凶险，早期病死率高，易合并脑出血及成人呼吸窘迫综合征（ＡＲＤＳ），化疗缓解率极低［１，２］。采用血细胞分离机进行白细胞清除术加用联合化疗已有少数报...     

白细胞清除术加联合化疗治疗高白细胞白血病(附四例报告)

高白细胞白血病系指外周血白细胞异常增多的急、慢性白血病。由于白细胞过高(均>100×10~9/L),常伴有高粘滞血症,易发生颅内出血及呼吸窘迫综合征,导致短期内迅速死亡。治疗反应差,缓解率低,预后恶劣.为此,我们采用白细胞清除并同时进行联合化疗治疗高白细胞白血病4例,取得了初步疗效.介绍如下:临床资料     

白细胞剔除术加联合化疗治疗高白细胞白血病疗效观察及护理对策

鉴于高白细胞白血病(HLAL)患者血液循环中自细胞数过高，常规化疗常引起肿瘤溶解综合征和DIC，影响治疗及预后，2000年1月～2003年2月，我院对11例HLAL患者行白细胞剔除术，术后行联合化疗，取得较好疗效，现报告如下。     

白细胞单采在高白细胞性白血病中的治疗作用

治疗性白细胞单采术是指去除患者循环血液中异常增多的白细胞,以减少其对机体的致病作用,是一种见效迅速、安全的辅助治疗手段。1997年8月~2005年5月,我们应用白细胞单采联合化疗治疗高白细胞性白血病(hyperleukocytic leukemia,HLL)40例,效果较好,现报道如下。资料与方法一般资料40例均系我院住院患者,按标准[1]诊断白血病,就诊时WBC均>100×109/L。高白细胞急性白血病(hyperleukocytic acute leukemia,HAL)25例(男14、女11),中位年龄35(17~65)岁,其中急性淋巴细胞性白血病(acute lymphoblastic leukemia,ALL)6例,急性非淋巴细胞性白…     

维甲酸诱导治疗急性早幼粒细胞白血病并发高白细胞血症的临床分析

目的:探讨全反式维甲酸(ATRA)诱导治疗急性早幼粒细胞白血病(APL)并发高白细胞血症的临床特点及治疗的时机.方法:回顾性分析99例初治时白细胞不高的APL患者的ATRA诱导治疗过程.比较高白细胞血症的预防性用药组(44例,白细胞数≤10×109/L时加用化疗药物)和治疗性用药组(55例,白细胞数>10×109/L时加用化疗药物)的临床特点.结果:预防组在白细胞峰值、高白细胞持续时间、白细胞升高倍数上均显著低于治疗组(P<0.05),而血液制品输注量,院内感染、多部位出血事件及维甲酸综合征(RAS)发生率无显著性差异(P>0.05).结论:在ATRA诱导治疗APL过程中,尽早联用化疗药物可以明显降低白细胞峰值,缩短高白细胞持续时间,同时不增加血液制品输注量、院内感染及多部位出血事件发生率.     

20例高白细胞性白血病白细胞单采后观察

高白细胞性白血病是指外周血白细胞 >1 0 0×1 0 9/L患者 ,属高危型白血病 ,易发生颅内出血、脑栓塞等严重并发症 ,病死率高 (早期死亡率高 )。笔者对 2 0例高白细胞性白血病行白细胞单采治疗进行了观察 ,现报道如下。1　材料与方法1 .1 　 主要材料与设备美国 Baxter公司产 Cs30 0 0血细胞分离机、Cs30 0 0专用一次性全封闭管道 ( Kit4R2 2 30 )、CIS- F82 0血细胞计数仪 (日本株式会社产品 )、1 0 %的葡萄糖酸钙 (扬州中保制药有限公司 )、1次性 50 ml大空针 (威海医用高分子厂 )。1 .2 　 临床资料行白细胞单采患者 2 0例 ,男 1 3例 …     

MCS单采去除白细胞治疗高白细胞白血病的护理体会

高白细胞白血病(HLL)是在少数急性或慢性粒细胞白血病急变时,外周血白细胞大于100×109/L.异常增高的外周血白细胞导致血液黏滞度增加,血流缓慢,加重组织缺氧,白血病细胞比正常细胞大且僵硬,变形性差,易在小血管形成微血栓或凝块,造成微循环障碍.白细胞单采去除术可迅速去除患者体内白细胞,减轻患者病征,为下一步药物化疗打下基础.笔者2005年1月～2006年6月对15例HLL患者采用MCS血细胞分离机单采去除患者白血病细胞治疗,取得了满意的治疗效果.现将单采治疗过程中护理体会报告如下.     

高白细胞急性白血病50例分析

目的　研究高白细胞急性白血病 (HAL )的临床特点。方法　对 5 0例 HAL进行临床回顾性分析 ,同时选择 5 0例非高白细胞急性白血病 (HAL )作对照组。结果　 HAL起病急骤、病程<1月 ,脏器出血、髓外浸润的发生率、白细胞瘀滞综合征及化疗后肿瘤溶解综合征、早期死亡率较对照组高。结论　对 HAL降低血液粘滞度、化疗前、化疗中硷化尿液及水化治疗 ,以降低早期死亡率     

Correlation between expression of CD56/NCAM and severe leukostasis in hyperleukocytic acute myelomonocytic leukaemia

OBJECTIVE: The possible contribution of surface molecules to the development of leukostasis syndrome in hyperleukocytic acute myeloid leukaemia (AML) was assessed by routine immunophenotyping and grading of the probability of clinical leukostasis. METHODS: Fifty-three patients (23 women, 30 men, median age 59 yr) with hyperleukocytic AML [white blood count (WBC) above 50 x 10(9)/L] were graded for the probability of clinical leukostasis according to the severity of neurologic, pulmonary and other symptoms possibly caused by leukostasis using a recently published scoring system. Age, WBC, absolute blast count, haemoglobin, cytogenetic risk group, infection, relative CD56 expression and absolute count of CD56 positive blasts were analyzed in multivariate stepwise backward logistic regression analysis. RESULTS: In patients with acute monocytic leukaemia (AML M4/M5) the absolute count of leukaemic blasts expressing CD56/NCAM was highly associated with the development of symptoms graded as highly probable leukostasis and all three patients succumbing to early death were CD56 positive. Only the absolute count of CD56 positive blasts was a significant predictor of risk of severe leukostasis (P = 0.020). This was not found in AML without monocytic involvement (AML M1, M2, M3v). CONCLUSIONS: The expression of CD56/NCAM, a surface marker used in routine immunophenotyping of AML, may help to predict severe and potentially fatal leukostasis in hyperleukocytic acute myelomonocytic leukaemia. These results emphasize the usefulness of this four-stage clinical grading scale for analysing the factors, which lead to severe leukostasis in hyperleukocytic patients. We extend previous findings that the mechanisms of leukostasis are different depending on the involvement of the monocytic lineage.     

Grading of symptoms in hyperleukocytic leukaemia: a clinical model for the role of different blast types and promyelocytes in the development of leukostasis syndrome

OBJECTIVE: Patients with hyperleukocytic leukaemia were graded according to the severity of symptoms possibly caused by leukostasis to evaluate the effectiveness of therapy and to test the relative contribution of blast type and count of blasts and promyelocytes in the development of leukostasis syndrome. METHODS: Ninety-five patients (59 male, 36 female, median age 52 yr) with hyperleukocytic leukaemia [leukocytes above 50 x 10(9)/L, 48 acute myeloid leukaemia (AML), 31 chronic myeloid leukaemia (CML), 13 acute lymphoblastic leukaemia (ALL), three chronic myelomonocytic leukaemia (CMML)] were grouped according to the presence or absence and severity of neurologic, pulmonary and other symptoms into four categories (no, possible, probable and highly probable leukostasis syndrome). Age, white blood count (WBC), haemoglobin, blast count and total of blasts plus promyelocytes of these groups were compared by Mann-Whitney U-test. RESULTS: Patients with myeloid leukaemia (AML M1/M2, CML) which scored as highly probable leukostasis showed significantly higher WBC (P = 0.011), lower haemoglobin (P = 0.004), higher peripheral blast counts (P = 0.004) and higher total of peripheral blasts plus promyelocytes (P < 0.001) compared with the lower probability groups. In leukaemia involving the monocytic lineage (AML M4/M5, CMML) no significant differences were found in any of these factors between patients with highly probable leukostasis and the other patients. CONCLUSIONS: Our results show that a four-stage clinical grading scale is a valuable tool for analysing hyperleukocytic patient populations and evaluate the effectiveness of therapy more precisely. We further demonstrate that the mechanisms of leukostasis are different in myeloid leukaemia as compared with leukaemia with involvement of the monocytic lineage.     

Leukapheresis and cranial irradiation in patients with hyperleukocytic acute myeloid leukemia: no impact on early mortality and intracranial hemorrhage

To assess the role of leukapheresis and cranial irradiation in reducing the incidence of intracranial hemorrhage (ICH) and early death in patients with hyperleukocytic acute myeloid leukemia (AML) and the impact of such treatment on survival. This study retrospectively analyzed the records of 75 patients with hyperleukocytic AML who had a white cell count over 100,000/microL. All patients had de novo AML except for two with therapy-related AML. Various factors were assessed for their impact on morbidity and mortality, particularly the role of pre-induction leukapharesis and cranial irradiation. The most significant risk factors for ICH were the presence of two or more symptoms of leukostasis (odds ratios [OR] 10.6, 95% CI: 2.67-42.02; P = 0.001) and respiratory distress (OR 5.41, 95% CI: 1.44-20.32, P = 0.012). The most significant risk factors for early death were age >or= 65 (OR 4.21, 95% CI: 1.45-12.21, P = 0.008), respiratory failure (OR 3.34, 95% CI: 1.24-9.50, P = 0.018), and two or more symptoms (OR 3.50 95% CI: 1.16-10.52, P = 0.026). Neither leukapheresis nor cranial irradiation were significantly associated with a decreased incidence of ICH (P = 0.349 and 0.378, respectively). Leukapheresis had no significant influence on early death (P = 0.367). The median survival patients receiving no pretreatment was 10.50 months (range 2.58-18.42) and for those receiving pretreatment 1.50 months (range 0.10-3.16; log-rank test, P = 0.062). Leukapheresis and cranial irradiation do not improve survival or decrease the incidence of ICH in adults with hyperleukocytic AML.     

Hyperleukocytosis, leukostasis and leukapheresis: practice management

Hyperleukocytosis, arbitrarily defined in acute leukemia as a white blood cell count greater than 100,000/mL, often is associated with increased morbidity and mortality in patients with leukemic processes. It can induce leukostasis, tumor lysis syndrome and disseminated intravascular coagulopathy and has significant prognostic implications with or without one of these clinical complications. The main sites that tend to be injured from the obstructions are the central nerve system and lungs. Despite characteristic clinical presentations, the diagnosis of leukostasis is rarely made with high confidence. The main goal of the management of hyperleukocytosis and/or leukostasis is to reduce the white blood cell count before starting induction chemotherapy. The cytoreduction can be achieved by either leukapheresis and/or hyroxyurea. The technical aspects, complications and efficacy of leukapheresis are discussed in the current article.     

Pulmonary leukostasis: role of perfusion lung scan in diagnosis and follow up

A patient with hyperleukocytic myelomonocytic leukemia who presented to the emergency room with sudden pleuritic chest pain and dyspnea is reported. Clinical manifestations included dyspnea tachypnea and hyperventilation. Blood gas analysis revealed hypoxemia, hypocarbia, and respiratory alkalosis. Chest X ray was normal, and perfusion lung scan revealed a diffuse vascular occlusive pattern compatible with pulmonary leukostasis. The patient underwent immediate leukapheresis with subsequent mitigation of symptoms. A second perfusion lung scan showed evidence of significant improvement. To our knowledge this is the first published case of hyperleukocytosis presenting with pulmonary leukostasis that was successfully diagnosed and followed by serial perfusion lung scan.     

Leukocytoreduction for Acute Leukemia

Abstract: Both in children and adults, acute leukemia may present with extremely high blast counts; a phenomenon known as hyperleukocytosis. Respiratory failure, intracranial bleeding, and severe metabolic abnormalities frequently occur in acute hyperleukocytic leukemias (AHLs) and are the primary determinants of the high early mortality (20% to 40%) observed. The process leading to these complications has long been known as leukostasis, but the biological mechanisms underlying its development and progression have remained unclear. Traditionally, leukostasis has been attributed to overcrowding of leukemic blasts in the microcirculation, and its treatment has focused on prompt leukocytoreduction. However, it is becoming increasingly evident that leukostasis results from the adhesive interactions between leukemic blasts and the endothelium; a mechanism that none of the current therapies directly addresses. The endothelial damage associated with leukostasis is likely to be mediated by cytokines released in situ and by subsequent migration of leukemic blasts in the perivascular space. The adhesion molecules displayed by the leukemic blasts and their chemotactic response to the cytokines in the vascular microenvironment are probably more important in causing leukostasis than the cell number. This may explain why leukostasis may develop in some patients with AHL and not in others, and why some patients with acute leukemia without hyperleukocytosis (<50,000 blasts/mm³) develop leukostasis and respond to leukocytoreduction. Leukapheresis effectively reduces the blast count in many patients with AHL and is routinely used for immediate leukocytoreduction. However, the most appropriate use of leukapheresis in acute leukemia remains unclear, and the procedure may not prevent early death more efficiently than fluid therapy, hydroxyurea, and prompt induction chemotherapy. The use of cranial irradiation remains very controversial and is not generally recommended. The identification of the adhesion molecules, soluble cytokines, and chemotactic ligand‐receptor pairs mediating endothelial cell damage in AHL should become a priority if better outcomes are desired.     

Pulmonary leukostasis as the single worst prognostic factor in patients with acute myelocytic leukemia and hyperleukocytosis

Patients with acute myelocytic leukemia and hyperleukocytosis have a poor prognosis due to vascular leukostasis and infiltration in the central nervous system and lungs. The clinical records of all patients with a new diagnosis of acute myelocytic leukemia and initial white blood cell count greater than 100,000 X 10(9)/liter admitted to the Mount Sinai Medical Center between the years 1974 and 1983 were examined. Forty-three patients were identified, 22 of whom had clinical and/or pathologic evidence of leukostasis of the central nervous system and/or lung. All patients received induction chemotherapy with daunorubicin and a continuous infusion of cytosine arabinoside. Thirty-five patients also underwent therapeutic leukapheresis prior to induction chemotherapy. The overall remission induction rate in these 43 patients was 51 percent. Fifteen patients had lung leukostasis; the remission rate for these patients was 27 percent (three complete remissions, one partial remission), as compared with a remission rate of 64 percent (18 of 28) for those without pulmonary leukostasis (chi 21 = 5.53; p = 0.02). Thirteen patients had central nervous system infiltration; the remission rate for these patients was 46 percent (five complete remissions, one partial remission), as compared with 53 percent (16 of 30) for patients without central nervous system involvement (chi 21 = 0.19; p = 0.67). The median survival of 21 patients without leukostasis was 10.8 months, as compared with 15.4 months for seven patients with central nervous system involvement and no lung leukostasis and 0.2 months for 15 patients with pulmonary leukostasis (chi 22 = 19.9; p less than 0.001). Thus, pulmonary leukostasis was found to be the single worst prognostic factor in this group of patients.     

Hyperleukocytosis and leukostasis: common features of childhood chronic myelogenous leukemia

Ten patients under 20 yr of age with the usual (adult) type of chronic myelogenous leukemia (CML) were seen at the University of Rochester Medical Center from 1970 to 1982. The mean white cell count in these 10 patients at presentation was 360,000/microliter, as compared to a mean of 137,000/microliter in 80 CML patients over 20 yr of age seen during the same time interval (p less than 0.02). Analyses of all 90 cases revealed a significant decrease in the average leukocyte count at presentation with increasing age. The childhood cases also had a significantly higher proportion of blood blasts, promyelocytes, and myelocytes than did the adult subjects (p less than 0.01). Signs of leukostasis were present in 12% of adult cases as compared to 60% of the 10 childhood cases, and in these 6 subjects, the mean white cell count was 510,000/microliter. In these 6 patients, leukapheresis and/or chemical therapy was initiated rapidly, and this was followed by complete resolution of the clinical signs of leukostasis. A review of the literature from 1960 to 1982 identified 61 childhood cases that were reported with the usual type of CML. In this group, the frequency of hyperleukocytosis and the distribution of white cell counts corresponded very closely to the 10 cases studied at the University of Rochester. Thus, the usual type of CML presenting in childhood differs from that of adults in that hyperleukocytosis, blood granulocyte immaturity, and leukostatic central nervous system, retinal, and respiratory signs are significantly more common and extreme and merit rapid cytoreductive treatment.     

Hydroxyurea in the prevention of the effects of leukostasis in acute leukemia.

Hydroxyurea was administered orally to prevent the effects of leukostasis in adults with acute leukemia who had peripheral blast cell counts greater than 100,000/cu mm. A single oral dose of 50 to 100 mg/kg was given daily until the absolute blast cell count decreased to less than 100,000/cu mm. Hydroxyurea was effective in rapidly lowering the blast cell count by an average of 50% after one dose in each of ten episodes. No patient developed symptoms or signs of the leukostasis syndrome, and no side effects directly attributable to hydroxyurea were observed. The leukostasis syndrome associated with very high blast cell counts in adults with acute leukemia can be avoided by the use of hydroxyurea in the manner described. This treatment can be particularly useful in the interval before consultation or referral and prior to the cytotoxic effect of definitive induction chemotherapy.     

Therapeutic apheresis in hyperleukocytosis and hyperviscosity syndrome

Therapeutic apheresis (TA), as a way of rapidly eliminating harmful or excessive blood components such as plasma proteins (plasma exchange) or cells (leukapheresis and platelet apheresis), has found broad application in a vast array of hematologic disorders. The most common hematologic indications for TA are leukocytosis in acute leukemias and hyperviscosity syndrome secondary to plasma cell dyscrasia. Leukapheresis is indicated in the initial management of leukostasis in patients with hyperleukocytosis in acute leukemias, particularly myeloid leukemias, or in patients who are at high risk of developing such a complication. Patients with lymphoid malignancies rarely develop leukostasis but may undergo cytoreduction with leukapheresis as prophylaxis for tumor lysis. The use of leukapheresis in acute promyelocytic leukemia is discouraged, given the possibility of increased risk of coagulopathy and bleeding. Similarly, therapeutic plasma exchange (TPE) represents an effective but temporary way of managing hyperviscosity syndrome secondary to immunoglobulin M paraproteins in patients with Waldenstr?m macroglobulinemia. This review provides an overview of the pathophysiology of leukostasis and its management with leukapheresis. The use of TPE in the management of hyperviscosity syndrome is also discussed.