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1.
目的 提高对慢性粒—单核细胞白血病(CMML)合并Sweet综合征的认识。方法回顾性分析1例CMML合并皮肤疱疹患者资料,取皮肤活检进行病理检测,并给予DA、CAG方案等化疗。结果病理诊断为CMML合并Sweet综合征,单用皮质激素治疗效果不佳,CAG方案使CMML获得完全缓解,皮疹得到有效控制。3个月后Sweet综合征复发,CMML进展为急性单核细胞白血病。结论CMML合并Sweet综合征罕见,高度提示短期内进展为急性白血病。  相似文献   

2.
OBJECTIVE: To study the cytologic features and role of fine needle aspiration biopsy (FNAB) in the diagnosis of extramedullary leukemia. STUDY DESIGN: Forty-one cases of extramedullary leukemia diagnosed by FNAB were analyzed along with their detailed clinical and hematologic features. RESULTS: Common sites of leukemic involvement were lymph nodes (34), skin (4), orbit (1), eyelid (1) and breast (1). The most common variety of leukemia was chronic myeloid in the chronic phase (17). Twenty-six patients were referred to the cytology clinic for FNAB as the initial screening test. In the majority of fresh cases, leukemia was not the first possibility considered, and FNAB played an important diagnostic role. No gross discrepancy was noted in any of the cases. CONCLUSION: FNAB is helpful in the diagnosis of extramedullary involvement by leukemia because of the good morphologic detail of blasts and other granulocytic cells. However, for more accurate subclassification of a hematologic disorder, other hematologic investigations are mandatory.  相似文献   

3.
Donor leukocyte infusions (DLI) are an effective therapy for patients who relapse with leukemia after bone marrow transplantation (BMT). Severe graft-versus-host disease and prolonged periods of pancytopenia compromise the success of this treatment in a substantial number of patients. We used filgrastim-mobilized peripheral blood progenitor cells (PBPCs), in some cases preceded by cytoreductive therapy, to circumvent some of the problems associated with DLI. Eleven patients (median age 41 years) received a total of 20 donor cell infusions. Their diagnosis was CML in hematological (two patients) or cytogenetic relapse (two patients), six patients suffered from acute myeloid leukemia (AM; n = 5) or Philadelphia chromosome-positive acute lymphoblastic leukemia (ALL Ph+). One patient had multiple myeloma (MM). All six patients with acute leukemias received cytoreductive therapy prior to PBPC infusions; three patients with CML were pretreated with IFN alpha. Four of four patients with CML responded to PBPC infusions and currently are in complete clinical and molecular remission for time periods between 1 and 12 months. Six of six patients with acute leukemias achieved a complete remission. All of them relapsed after a median remission duration of 24 weeks (range 11-49 weeks). Three patients relapsed at extramedullary sites (CNS, testes, skin). Four of six acute leukemia patients received further cytoreductive therapy. All patients responded again and are in complete remission for time periods between 14 and 615 days. Two patients with acute leukemias have died due to dissemination of the disease. The patient with MM did not respond and is alive with disease. Severe (grade III) acute GVHD developed in two of 11 patients, three patients developed grade II disease, six patients did not show any signs of GVHD. Extensive chronic GVHD has developed in two cases to date. Patients with chemotherapy prior to PBPC infusion developed neutropenia and thrombocytopenia with a maximum duration of 20 and 14 days, respectively; prolonged periods of neutropenia did not occur. Two patients developed long-lasting thrombocytopenia in spite of PBPC infusion, in one case followed by leukemic relapse. Repeated courses of chemotherapy and PBPC infusion were generally tolerated well; no early deaths due to treatment-related toxicity or GVHD were observed. We conclude that the use of allogeneic PBPC instead of DLI in patients with relapse after BMT is technically feasible and safe. The efficacy of PBPC infusions seems comparable to DLI in patients with CML. Patients with acute leukemias also achieved complete albeit transient remissions. Aggressive chemotherapy followed by PBPC infusions resulted in only limited duration of cytopenia. The usage of PBPC infusion instead of non G-CSF-mobilized donor cells for treatment of relapse after BMT may reduce pancytopenia-related complications and merits further investigation.  相似文献   

4.
BACKGROUND: Most children with acute lymphoblastic leukemia (ALL) and increasing number of children with acute myelogenous leukemia (AML) are currently cured with conventional chemotherapy. Despite of this success there is a subset of patients with high-risk features at diagnosis who are predisposed to a very high risk of relapse. Relapse of AML and early bone marrow relapse of ALL can not be cured by conventional chemotherapy. Allogeneic hematopoietic stem cell transplantation (HSCT) is therapeutic option in these children with very high-risk acute leukemia. METHODS AND RESULTS: Between XI/1989-XII/1996 33 children with acute leukemia (ALL: 22, AML: 11) underwent an allogeneic HSCT from HLA identical related donors (HLA-identical sibling: 30, twin: 1, other HLA-identical relative: 2) at the 2nd Dept. of Pediatrics, University Hospital Motol. Median age of our group was 9 years (1.5-19 y.), boys (n = 23) clearly dominated over the girls (n = 10). The resource of stem cells was bone marrow in 31 children, bone marrow and peripheral blood progenitor cells (PBPC) and PBPC in one child respectively. Myeloablative conditioning regimen varied, consisting of total body irradiation and chemotherapy in 21 children and chemotherapy in 12 children. HSCT was performed in first complete remission of acute leukemia in 9 children (AML: 7, ALL: 2), in second remission in 14 children (AML: 2, ALL: 12), in third remission in 4 children (ALL: 4). Six children underwent HSCT in first partial remission (n = 1) and in second (n = 4) or third (n = 1) chemoresistant relapse. Seven (21%) children died due to post-transplant complications. Nine (28%) children suffered from clinically significant acute graft-versus-host reaction (GVH) and 15% (4/27) children who survived 100 days post-transplant suffered from chronic GVH disease. Relapse of leukemia was diagnosed in 39% (12/31) children. Fourteen (42%) children are alive and well in continuous remission with median follow-up 42 months. CONCLUSIONS: Allogeneic HSCT can cure children with very high-risk acute leukemia in the situations where conventional chemotherapy fails. Relapse of leukemia and GVH reaction are most important causes of post-transplant morbidity and mortality.  相似文献   

5.
目的 探讨白血病患者骨髓中DLL4、HES1、VEGF-C及VEGFR-2的mRNA表达水平检测的临床意义,为白血病的诊治提供新的思路。方法选取白血病患者59例作为病例组,均根据临床表现、血象、骨髓象、细胞化学染色、细胞遗传学及流式细胞术检查确诊;对照组20例为营养性贫血患者。采用半定量反转录聚合酶链反应( RT-PCR)方法测定DLL4、HES1、VEGF-C、VEGFR-2 mRNA的含量。结果各组初发急性和慢性白血病患者骨髓中DLL4、HES1、VEGF-C、VEGFR-2mRNA的表达与对照组相比均显著升高(P<0.05)。急性白血病缓解后DLL4、HES1、VEGFR-2的mRNA表达高于对照组( P= 0.041、0.016、0.047)。急性髓系白血病(AML)组DLL4与VEGFR-2、HES1与VEGF-C表达呈正相关(r= 0.424、0.472;P=0.030、0.014);慢性淋巴细胞白血病(CLL)组HES1与VEGF-C表达呈正相关(r= 0.997,P=0.042)。急性白血病伴髓外浸润者VEGF-CmRNA的表达高于不伴髓外浸润者(P=0.022)。AML组VEGF-C mRNA的表达与原始细胞数呈正相关(r=0.315,P=0.024)。结论DLL4、HES1、VEGF-C及VEGFR-2在白血病发病中相互作用,促进白血病发展、转移及浸润,且这些因子在不同类型白血病及髓外浸润中的作用存在差异。  相似文献   

6.
目的 评估全反式维甲酸(ATRA)、三氧化二砷(As2O3)、化疗联合治疗初诊急性早幼粒细胞白血病(APL)的疗效和患者不良反应.方法 对40例采用ATRA、As2O3、化疗三联疗法治疗的APL患者的临床资料进行回顾性分析,观察其疗效及不良反应.结果 总体CR率92.5%(37/40),完全缓解所需中位时间27(22~61)d;白细胞≥10×109/L组CR率72.7%(8/11),白细胞<10×109/L组CR率100%(29/29),差异有统计学意义(x2=8.550,P=0.004);治疗过程中无严重不良反应,仅1例发生维甲酸综合征.结论 ATRA、As2O3、化疗联合应用可作为APL患者的首选治疗方案.  相似文献   

7.
Second malignancy after childhood neoplasms is a well-known complication. However, frequency differs considerably according to the types of primary neoplasm and the specifics of therapy. Ten patients with a second malignancy after being cured of the primary tumor are described. There were 2 patients with acute lymphoblastic leukemia, one with non-Hodgkin's lymphoma, and one with breast cancer after Hodgkin's disease. Two patients with heritable retinoblastoma developed osteosarcomas in the irradiation field after a latent period of 7 and 14 years respectively. There was another osteosarcoma in a Wilms' tumor survivor. One patient with acute lymphoblastic leukemia developed a secondary AML 10 years after achieving initial remission, and a meningioma was diagnosed in another patient with cured acute lymphoblastic leukemia. One patient died of peritoneal sarcomatosis of unknown origin 20 years after the diagnosis of acute myeloid leukemia. All patients received radiotherapy for the primary neoplasms. Secondary neoplasms in other patients were probably missed because they occurred in adulthood when the patients were transferred to other medical centres. It is impossible to trace these patients because central registration of patients with neoplasms is lacking. It is therefore important to establish a central cancer registry for the whole of Switzerland. Second malignancy after childhood cancer is not a rare event and requires long-term follow-up of patients with neoplasms.  相似文献   

8.
PURPOSE: To assess the non-cutaneous involvement in primary B-cell non-Hodgkin's lymphoma (NHL) of the skin. PATIENTS AND METHODS: Data from 45 patients with B-cell NHL of the skin were retrospectively analysed. The patients were diagnosed on histologic and immunocytochemical grounds between June 1977 and July 1993, and 14 cases were selected for their exclusively cutaneous initial involvement. Initial treatment, response to therapy, disease-free survival characteristics of relapse and therapeutic sequence were evaluated in every case. RESULTS: Cutaneous involvement presented as nodules or patches, on a single location, in 12 cases, or disseminated, in 2 others. No prognostic factor could be identified, and complete remission was attained in all cases. Cutaneous relapse was seen in 7 patients after 4 to 108 months since diagnosis. Extracutaneous dissemination was not seen in any case, and 13 patients are alive and disease-free. A 90 year-old woman died of toxic complications. CONCLUSIONS: The clinical facts reported here confirm the not too aggressive behaviour of certain B-cell cutaneous NHL, probably related with their origin on the skin itself.  相似文献   

9.
BACKGROUND: Maternal reproductive history of fetal loss previously has been reported to be associated with an increased risk of leukemia in subsequent offspring. Data from a Childrens Cancer Group (CCG) case-control study were analyzed to test the hypothesis that this association was dependent on the number of previous fetal losses and age at leukemia diagnosis. METHODS: A case-control study using a large Childrens Cancer Group database examined maternal history of fetal loss as a risk factor for childhood leukemia in subsequent offspring. One thousand seven hundred fifty-three patients with childhood acute leukemia were compared with 839 community control subjects s and 2081 nonleukemia cancer control subjects. RESULTS: A modest increase in risk was found to be associated with a history of fetal loss. Stratification by age at diagnosis of leukemia showed that this association was significant only for those patients diagnosed before 4 years of age and most significant in those patients diagnosed before 2 years of age. When comparing community controls with patients acute lymphocytic leukemia diagnosed before 2 years of age, one previous fetal loss was associated with a five-fold increased risk (P < 0.001) whereas two or more fetal losses were associated with a relative risk of 24.8 (P < 0.001). Similarly, patients with acute myelocytic leukemia diagnosed before 2 years of age demonstrated 5-fold and 12-fold increased risks associated with the previous fetal loss and 2 or more previous fetal losses, respectively. CONCLUSIONS: Childhood acute leukemia occurring at younger ages may be associated with an underlying genetic abnormality or chronic environmental exposure, which can be either lethal to the developing fetus or mutagenic and result in the development of acute leukemia.  相似文献   

10.
A patient with acute myelogenous leukemia, in clinical remission following chemotherapy, presented with three unusual pulmonary nodules. Needle biopsy showed that these were leukemic cells in the interstitium of the lung.  相似文献   

11.
BACKGROUND AND METHODS: A characteristic of acute myeloid leukemia is the frequent ability of the leukemic cells to sustain their own proliferation in vitro. To determine the clinical importance of this property, we measured the uptake of tritiated thymidine by leukemic cells in serum-free and cytokine-free cultures as a means of determining the rate of spontaneous proliferation in 114 patients with newly diagnosed acute myeloid leukemia. Proliferation was then classified according to three quantitative levels of activity and related to overall survival and to treatment outcome (the response to treatment, the actuarial probability of relapse, and disease-free survival) in 91 patients who were treated with chemotherapy to induce remission. RESULTS: Of the 114 patients, 37 had low, 39 had intermediate, and 38 had high levels of proliferation. The probability of survival at three years was 36 percent among patients with low levels of proliferative activity and 3 percent among those with high levels (P < 0.001). Among the patients treated with chemotherapy, those with low rates of proliferative activity had a 68 percent rate of complete remission and a 49 percent probability of remaining free of relapse, whereas those with high rates of proliferative activity had only a 39 percent rate of complete remission (P = 0.04) and an 11 percent probability of remaining in complete remission (P = 0.009). The probability of disease-free survival at three years among the patients in complete remission after chemotherapy was 49 percent among those with low rates of proliferative activity and 9 percent among those with high rates (P = 0.004). Accordingly, patients with low rates of proliferative activity also had a significantly higher rate of overall survival (44 percent vs. 4 percent; P = 0.002). Patients whose cells had intermediate levels of proliferation in vitro had intermediate rates of survival, relapse, and disease-free survival. CONCLUSIONS: The capacity of leukemic blasts for autonomous proliferation is associated with highly aggressive acute myeloid leukemia.  相似文献   

12.
目的 寻找复发难治性前体淋巴细胞白血病/淋巴瘤诱导缓解的有效方法.方法 以CAG预激方案联合左旋门冬酰胺酶(L-Asp)和泼尼松(PDN)诱导治疗6例复发难治性前体淋巴细胞白血病/淋巴瘤和1例急性杂合细胞白血病.结果 6例患者完全缓解(CR),1例部分缓解(PR),总有效率100%(7/7),CR率85.7%(6/7).患者不良反应轻,均可耐受.结论 CAG联合L-Asp和PDN是复发难治性前体淋巴细胞白血病/淋巴瘤值得尝试的诱导化疗方案.  相似文献   

13.
Children and young adults less than 18 years of age with acute nonlymphocytic leukemia who remained in long term bone marrow and extramedullary remission for two years or longer since starting maintenance were compared to the remaining responders for the following characteristics: cell type, sex, age at diagnosis, race, pretreatment, white blood count, length of time from start to induction therapy to achievement of an M1 marrow, marrow rating at day 56 of therapy, marrow rating at the start of maintenance therapy, and specific study. Forty-eight patients of a group of 333 qualified as having long term remission (14.4%). Multivariant analysis indicated that patients between the ages of 3 and 10 years (p = 0.003) as well as the length of time to achieve an M1 marrow from the start of treatment (p = 0.03) were the only characteristics associated with achievement of a long term remission. Maintenance therapy was discontinued in 15 patients from 2.5 to 4.8 years after start of maintenance and all patients remained in bone marrow remission of periods from 0+ to 3.0+ years after stopping treatment. Of the 33 who have remained on a continuous maintenance therapy 12 have had bone marrow relapses. These data confirm the prognostic value of age and length of time to achieve remission during induction in acute nonlymphocytic leukemia and suggest that there may be no significant benefit from maintenance therapy continued beyond 2 years for patients in their initial remission.  相似文献   

14.
The purpose of the study was to evaluate the feasibility and efficacy of high-dose cytarabine-anthracycline consolidation chemotherapy followed by autologous transplantation of chemotherapy/rHuG-CSF-mobilized peripheral blood progenitor cells for adult patients with acute myelogenous leukemia in first remission. Fifty-nine consecutive patients (median age 45, range 18-69) with acute myelogenous leukemia in first remission were enrolled on a study of high-dose cytarabine-mitoxantrone consolidation chemotherapy used as a method of in vivo purging for the purpose of autologous peripheral blood progenitor cell transplantation. A median of 7 x 10(8) peripheral blood mononuclear cells/kg were infused 1 day after preparative conditioning with 11.25 Gy total body irradiation and cyclophosphamide (120 mg/kg). Forty-six patients received myeloablative chemo-radiotherapy followed by the infusion of chemotherapy/rHu-G-CSF-mobilized autologous peripheral blood progenitor cells. The median time to both neutrophil and platelet recovery from transplant was 15 days (range, 11-36 and 5-253+ days, respectively). After a median follow-up of 27 months, 31 patients remain alive with 27 in complete remission. Median remission duration for all eligible patients is 12 months, and actuarial leukemia-free survival at 3 years is 42 +/- 14%. The actuarial risk of relapse is 54 +/- 15%. Toxicity of autologous peripheral blood progenitor cell transplant included treatment-related death in two patients and grade III/IV organ toxicity in six. Advanced age was a negative prognostic factor for leukemia-free survival. Our results demonstrate that autologous transplantation of chemotherapy-mobilized peripheral blood progenitor cells is feasible in an unselected population of adult patients with acute myelogenous leukemia in first remission producing improved leukemia-free survival with minimal toxicity.  相似文献   

15.
BACKGROUND: The occurrence of fatal or nearly fatal pulmonary insufficiency in 5 of 22 pediatric patients with relapsed acute myelogenous leukemia (AML) treated with high dose cytosine arabinoside (Ara-C) at St. Jude Children's Research Hospital, Memphis, Tennessee, and institutions affiliated with the Pediatric Oncology Group (POG) is reported. METHODS: Cytosine arabinoside (1.0-1.5 g/m2/day) was given as a 5-day continuous infusion to all patients. Four patients with persistent leukemia received a second 3- or 5-day course. The POG protocol included the administration of granulocyte colony stimulating factor for the priming of myeloblasts. Diagnostic criteria for pulmonary insufficiency included noncardiogenic pulmonary edema with exclusion of underlying cardiorespiratory, infectious, or metabolic conditions. Autopsy material also was reviewed. RESULTS: Of the 22 patients 5 died (23%), including 2 who received a second course of Ara-C as a result of pulmonary insufficiency that developed at a median of 8 days (range, 3-38 days) after the first course. Three patients died despite intubation and pressor support. Two patients were managed successfully with colloids, diuresis, and oxygen by face mask; remission was achieved in both. The postmortem examination of one patient disclosed airless lungs, profound pulmonary edema, and subpleural nodules, but no evidence of leukemia. CONCLUSION: Pulmonary insufficiency from high dose Ara-C varies in severity and may be fatal. It may occur during or after treatment. Awareness of this potential complication, careful attention to fluid status, and aggressive supportive care may optimize outcome.  相似文献   

16.
17.
BACKGROUND: Erythroderma may result from different causes, but a proportion remains undetermined (idiopathic erythroderma). Patients with idiopathic erythroderma have often been regarded to have a pre-Sézary syndrome because some of these patients have developed a cutaneous T-cell lymphoma during follow-up. OBJECTIVE: The aim of this study was to investigate if this was true for our group and also if it is possible to identify further which patients are at high risk of developing cutaneous T-cell lymphoma. METHODS: We analyzed clinical and follow-up data and reviewed the skin histopathology of all patients who were diagnosed with idiopathic erythroderma in our clinic between 1977 and 1994. RESULTS: Twenty-eight patients, 16 males and 12 females, were diagnosed with idiopathic erythroderma. This is 27% of the patients who were diagnosed with erythroderma in our clinic, during this period. During the median follow-up of 33 months, 35% of the patients went into complete remission and 52% showed partial remission. Three patients (13%), all females, had persistent chronic erythroderma. Two of the latter group progressed to cutaneous T-cell lymphoma, i.e. 1 to Sézary syndrome and 1 to mycosis fungoides. CONCLUSION: Based on our results we conclude that only patients with persistent chronic idiopathic erythroderma, which is a minority, have an increased risk of developing cutaneous T-cell lymphoma and therefore need a close and long-term follow-up.  相似文献   

18.
All-trans-retinoic acid in acute promyelocytic leukemia   总被引:1,自引:0,他引:1  
BACKGROUND: All-trans-retinoic acid induces complete remission in acute promyelocytic leukemia. However, it is not clear whether induction therapy with all-trans-retinoic acid is superior to chemotherapy alone or whether maintenance treatment with all-trans-retinoic acid improves outcome. METHODS: Three hundred forty-six patients with previously untreated acute promyelocytic leukemia were randomly assigned to receive all-trans-retinoic acid or daunorubicin plus cytarabine as induction treatment. Patients who had a complete remission received consolidation therapy consisting of one cycle of treatment identical to the induction chemotherapy, then high-dose cytarabine plus daunorubicin. Patients still in complete remission after two cycles of consolidation therapy were then randomly assigned to maintenance treatment with all-trans-retinoic acid or to observation. RESULTS: Of the 174 patients treated with chemotherapy, 120 (69 percent) had a complete remission, as did 124 of the 172 (72 percent) given all-trans-retinoic acid (P=0.56). When both induction and maintenance treatments were taken into account, the estimated rates of disease-free survival at one, two, and three years were 77, 61, and 55 percent, respectively, for patients assigned to chemotherapy then all-trans-retinoic acid; 86, 75, and 75 percent for all-trans-retinoic acid then all-trans-retinoic acid; 75, 60, and 60 percent for all-trans-retinoic acid then observation; and 29, 18, and 18 percent for chemotherapy then observation. By intention-to-treat analysis, the rates of overall survival at one, two, and three years after entry into the study were 75, 57, and 50 percent, respectively, among patients assigned to chemotherapy, and 82, 72, and 67 percent among those assigned to all-trans-retinoic acid (P= 0.003). CONCLUSIONS: All-trans-retinoic acid as induction or maintenance treatment improves disease-free and overall survival as compared with chemotherapy alone and should be included in the treatment of acute promyelocytic leukemia.  相似文献   

19.
OBJECTIVE: To assess the role of ultrasonography and fine needle aspiration cytology (FNAC) in preoperative diagnosis of patients with occult thyroid carcinoma (OTC). STUDY DESIGN: Data on 768 thyroid carcinoma patients receiving primary treatment at Chang Gung Medical Center were retrospectively reviewed. Of these patients, 97 had OTC. To detect small thyroid nodules early and define the characteristics of clinically palpable nodules, thyroid ultrasonography with FNAC were performed on 67 histopathologically proven OTC patients. Analysis for diagnostic value was done for ultrasonography and FNAC. RESULTS: In the 67 patients receiving ultrasonography with FNAC, 23 were preoperatively diagnosed as having papillary thyroid carcinoma and 1 as having follicular carcinoma. The tumor size of these 24 preoperative FNAC-proven OTC was 0.81 +/- 0.23 cm (mean +/- SD). In the remaining patients, 10 presented pictures suspicious for malignancy, with a mean tumor size 0.63 +/- 0.24 cm, and 33 (49.3%) were diagnosed as having benign thyroid lesions in preoperative FNAC. The tumor size in these 33 lesions was 0.58 +/- 0.24 cm. Fifty-seven of the 67 OTC patients received frozen sections. Thirty-eight papillary thyroid carcinomas and four follicular carcinomas were correctly diagnosed on frozen sections. CONCLUSION: Although the rate is not high, high-resolution ultrasonography and FNAC is the best approach to preoperative diagnosis for OTC patients today.  相似文献   

20.
BACKGROUND: Acute lymphoblastic leukemia has a different evolution in children and adults. Complete remission is achieved in 70-85% of the latter, but only 25 to 35% have a prolonged disease-free survival. AIM: To assess the treatment outcome of adults with acute lymphoblastic leukemia in Chile. PATIENTS AND METHODS: A retrospective review of the medical files of 51 adults with acute lymphoblastic leukemia treated according to the national protocol for antineoplastic drugs, in a public hospital of Santiago. RESULTS: Mean age of patients was 35.9 years old and 53% were younger than 20 years old. Complete remission was achieved in 64%. Early mortality occurred in 22%, mostly due to infections. Treatment failure occurred in 16%. Global survival was 17 months and global survival at 5 years was 24%. Prognostic criteria of the protocol, defined a low risk group that had a complete remission of 100%, no early deaths and 5 year disease-free survival of 53%. The group defined as having high risk had a complete remission of 47%, a 29% early mortality and a 5 years disease-free survival of 6%. CONCLUSIONS: Aiming to improve treatment results in acute lymphoblastic leukemia, efforts should be made to improve the management of febrile neutropenic patients and to intensify chemotherapy.  相似文献   

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