毛细胞白血病治疗近况

毛细胞白血病(HCL)是一少见的成人淋巴系统恶性增殖性疾病。虽然脾切除仅可治愈少数有显著脾脏病变的病人,但对改善大部分病人的血细胞计数仍是一有用的治疗措施。1987年前,是以苯丁酸氮芥的联合化疗和去除白细胞法治疗;但近年来两种新的高效制剂α-干扰素(α-IFN)及 2′-去氧助间型霉素(DCF)已被作为治疗 HCL 的第一线药物。脾切除脾切除后,10%的 HCL 患者可获长期的血液学缓解。脾切除适于脾肿大在肋下5cm以上,中性粒细胞计数大于1.5×10~9/L 及骨     

伴BRAF基因突变的毛细胞白血病二例并文献复习

目的 探讨BRAF基因突变在毛细胞白血病(HCL)诊断和治疗中的价值.方法 分析宁夏回族自治区人民医院收治的2例伴BRAF基因突变的HCL患者,并进行相关文献复习.结果 2例患者均检测到BRAF基因突变,该突变在HCL中具有高度特异性,BRAF基因抑制剂在传统治疗复发的患者中能够获得很好的效果.结论 BRAF基因突变对于HCL具有确诊价值,BRAF抑制剂对于HCL有潜在治疗价值.     

干扰素治疗毛细胞白血病进展

近年来,干扰素(Interferon IFN)用于治疗毛细胞白血病(HCL)越来越受到人们的注目。过去,以脾切除、脾照射、化疗、白细胞分离术及骨髓移植等方法治疗HCL,虽取得了一些效果,但都不甚理想。特别对部分脾切除无效和骨髓受抑制的晚期病人,治疗更为困难。1984年Quesada首次报道16例HCL患者,经IFN治疗后,15例获得了显著疗效。嗣后,不少学者做了许多临床和实验研究,均认为IFN是当今治疗HCL的有效药物,其副作用小,使用方便,     

毛细胞白血病8例报告

我院自1976年以来共收治毛细胞白血病(HCL)8例,通过资料分析,现着重对临床表现进行探讨:1 临床资料1.1 一般资料8例中男性5例,女性3例;最大     

BRAF V600E突变毛细胞白血病一例并文献复习

目的 探讨毛细胞白血病(HCL)的临床特点、诊断、鉴别诊断及治疗方案.方法 观察1例确诊的HCL,并进行文献复习.结果 患者为中老年男性,临床主要表现为反复发热、水肿、脾大及血象三系减少,根据血象、骨髓细胞学,结合免疫表型、基因突变检测(BRAF V600E突变阳性)明确诊断.治疗主要以干扰素为主,疗效尚可.结论 HCL为罕见的成熟B淋巴细胞恶性肿瘤,以"毛细胞"骨髓浸润为特点,免疫表型为CD25+、CD103+、CD11c+、CD23-、CD5-,几乎所有经典型HCL患者均存在BRAF V600E突变.用嘌呤类似物及新型疗法治疗,预后较好.     

克拉屈滨治疗毛细胞白血病三例并文献复习

 【摘要】　目的　观察克拉屈滨治疗毛细胞白血病的疗效。方法　3例患者应用克拉屈滨10 mg维持24 h静脉滴注,共治疗3 d或5 d。结果　3例患者中,2例患者疗效均达到完全缓解,1例患者为接近完全缓解。结论　克拉屈滨治疗毛细胞白血病疗效显著,未发现明显的不良反应。     

毛细胞白血病的临床及实验研究

 [摘要] 目的：探讨毛细胞白血病的临床和实验特点及其不同治疗方案的疗效和转归。方法：回顾性分析我院1978～2002年诊断的6例毛细胞白血病患者的临床特征、实验室检查结果及用不同方案治疗的疗效和转归。结果：6例毛细胞白血病首发症状以脾大、贫血、出血多见;2例细胞免疫表型为CD19(+)、CD20(+)、CD25(+)、 HLA-DR(+)、CD5(-)、CD10(-),细胞群为B细胞性;电镜下见毛细胞微绒毛;传统方案化疗、单用干扰素、单纯切脾疗效较差,只能使病情短暂缓解且易复发,联合治疗和氟达拉滨疗效明显优于前者。结论：感染、出血是HCL患者的主要死亡原因,脾切除加干扰素治疗较单纯切脾或干扰素治疗效果好,氟达拉滨治疗HCL疗效肯定,生存期明显改善。     

2-CdA in the treatment of hairy cell leukemia: a review of long-term follow-up

Hairy cell leukemia is a rare, indolent, chronic lymphoproliferative disorder characterized by the proliferation of a malignant B-cell clone with irregular cytoplasmic projections. Treatment with purine analogs such as 2-chlorodeoxyadenosine (2-CdA) is associated with excellent remission rates and long-term survival. Although the majority of patients achieve a complete remission (CR), most have minimal residual disease detected by sensitive methods. Despite the long term disease-free survival, approximately 36% of patients relapse and many require further therapy. Repeat administration of 2-CdA is very effective in achieving a second CR. Recently, new agents such rituximab and BL22 were shown to be effective in the treatment of relapsed and refractory disease.     

The value of the Wright-Giemsa stain for diagnosing hairy cell leukemia in body cavity fluids

We compared the value of the Wright-Giemsa stain with the Papanicolaou stain for diagnosing hairy cell leukemia in the body cavity fluids of three patients. The cytological features of the leukemic cells were much clearer on the Wright-Giemsa-stained preparations. This allowed for the accurate diagnosis of leukemic involvement of the body cavity fluids in 3/3 patients using the Wright-Giemsa stain but only 1/3 using the Papanicolaou stain. A tartrate-resistant acid phosphatase stain was used to confirm the diagnosis in each case.     

Sustained long-term remissions with weekly interferon maintenance therapy in hairy cell leukemia

Aim: This study evaluates the efficacy of weekly α‐interferon (IFN) maintenance therapy in hairy cell leukaemia (HCL), a disease that remains incurable. Method: Nine patients (six male, three female, aged 41–69 yrs) with hairy cell leukaemia (HCL) received IFN 3mU s.c. once weekly as long‐term maintenance therapy after achieving optimal clinical and hematological response to initial therapy with thrice weekly IFN. Results: Eight of the nine patients are in a state of sustained response at 3–17 years (median 12 years). Conclusion: Our results are similar to those from three previous studies using long‐term IFN maintenance therapy, bringing the total number of patients in sustained remission to 118. We hope these reports will lead to a multi‐centre, phase III study of IFN maintenance therapy (including pegylated IFN, given less frequently) in HCL patients achieving optimal response to initial therapy, be it IFN or a purine analogue.     

福达拉滨联合环磷酰胺治疗多毛细胞白血病1例并文献复习

目的:探讨多毛细胞白血病（HCL）的临床特点及治疗措施。方法:根据患者的临床表现、骨髓细胞形态学及组织化学染色、免疫分型、电镜检查等进行诊断,采用福达拉滨联合环磷酰胺方案治疗,并综合已有文献简述HCL治疗现状及进展。结果:该例患者为经典型HCL,福达拉滨联合环磷酰胺方案治疗后缓解。结论:HCL患者骨髓涂片发现毛细胞、电镜检查、免疫分型对诊断有重要意义,嘌呤核苷酸类似物对其治疗有较高的缓解率。     

The Hemalog D automated differential counter in the diagnosis of hairy cell leukemia

We studied the peripheral blood of 37 patients with hairy cell leukemia (HCL) prior to (n = 24) or following (n = 19) splenectomy, in the Hemalog D multi-channel white cell differential counter, to investigate whether the apparatus could contribute to the (early) diagnosis of this entity and to the differential diagnosis of HCL from atypical hairy cell leukemia (AHCL; n = 9), chronic lymphocytic leukemia (CLL; n = 21) and leukemic non-Hodgkin lymphoma of low-grade malignancy (LNHL; n = 19). HCL showed almost invariably monocytopenia, neutropenia and an increased percentage of LUC, with a rather typical picture of the X-Y display of the peroxidase channel. The percentage of hairy cells closely correlated with the percentage of the LUC from the Hemalog D. Discriminant analysis using several parameters of the Hemalog D differential count resulted in a complete separation of HCL from CLL, and a fair, although not complete, distinction of HCL from AHCL and LNHL. It was impossible to discriminate between AHCL and LNHL. The most important discriminating (single or combined) parameters were the absolute monocyte count, the TWBC and the absolute neutrophil number. It is concluded that the Hemalog D is a valuable tool in the (early) diagnosis of HCL and in the discrimination between HCL and other leukemic lymphoproliferative disorders.     

Multicenter retrospective analysis regarding the clinical manifestations and treatment results in patients with hairy cell leukemia: twenty‐four year Turkish experience in cladribine therapy

In this multicenter retrospective analysis, we aimed to present clinical, laboratory and treatment results of 94 patients with Hairy cell leukemia diagnosed in 13 centers between 1990 and 2014. Sixty‐six of the patients were males and 28 were females, with a median age of 55. Splenomegaly was present in 93.5% of cases at diagnosis. The laboratory findings that came into prominence were pancytopenia with grade 3 bone marrow fibrosis. Most of the patients with an indication for treatment were treated with cladribine as first‐line treatment. Total and complete response of cladribine was 97.3% and 80.7%. The relapse rate after cladribine was 16.6%, and treatment related mortality was 2.5%. Most preferred therapy (95%) was again cladribine at second‐line, and third line with CR rate of 68.4% and 66.6%, respectively. The 28‐month median OS was 91.7% in all patients and 25‐month median OS 96% for patients who were given cladribine as first‐line therapy. In conclusion, the first multicenter retrospective Turkish study where patients with HCL were followed up for a long period has revealed demographic characteristics of patients with HCL, and confirmed that cladribine treatment might be safe and effective in a relatively large series of the Turkish study population. Copyright © 2014 John Wiley & Sons, Ltd.     

Simultaneous presentation of hairy cell leukemia and follicular small cleaved cell lymphoma in a patient with previous diagnosis of renal cell carcinoma

Hairy cell leukemia is a lymphoproliferative disorder of the B lymphoid system that is associated with an increased incidence of second malignancies. Most of these second neoplasma have been solid tumours and few reports of lymphoid malignancies exist. We report here a case of simultaneous presentation of hairy cell leukemia diagnosed in the spleen of a patient under therapy for follicular lymphoma who had an antecendent history of renal cell carcinoma. The hairy cell leukemia and the follicular lymphoma were studied by means of molecular techniques and they were found to contain two different clonal B cell proliferations. This suggests an independent origin of these two B cell neoplasms. As far as we know, this is the first report of the coexistence of hairy cell leukemia and follicular lymphoma.     

Subsets of hairy cell leukemia defined by unique membrane proteins

Leukemic cell membrane proteins of 27 patients with hairy cell leukemia were evaluated by SDS gel electrophoresis in order to identify subsets of these patients and to define molecules for isolation and the development of diagnostic reagents. [³⁵S]methionine metabolic labeling, gel electrophoresis of isolated membranes and fluorography demonstrated about 100 membrane proteins. Variations in the amount of synthesis of two principal proteins, p35 and p15 (35,000 and 15,000 dalton proteins respectively), defined, in relative terms, three subtypes of hairy cell leukemias: (a) p35⁺, p15^? cells, (b) p35^?, p15⁺ cells, and (c) p35^?, p15^? cells. At this time, it is not known whether these subsets represent various related disease entities or stages in the progression of one disorder. Although p35 migrated in the gels with one component of the HLA-DR (p23,30) protein complex, immunoprecipitation studies with anti-p23, 30 serum indicated that it probably was not derived from HLA-DR antigen, which was, in fact, synthesized in addition to p35 on all hairy cells which were tested. Cultured lymphoblastoid cell lines established from the peripheral blood of hairy cell leukemia patients were substantially different in membrane protein patterns from the fresh leukemic cells. In particular, p35 and p15 were minimally expressed by cultured cells.     

The ultrastructural study of non-Hodgkin’s lymphoma cell,chronic lymphocytic and hairy cell leukemia

Non-Hodgkin’s lymphoma cell leukemia (NHLCL), chronic lymphocytic leukemia (CLL) and hairy cell leukemia (HLC) are the diseases very similar to each other. The differential diagnosis is very difficult, especially when there are small lymphoid cells in peripheral blood and bone marrow under light microscope. We have observed 34 cases with electron microscope. The studies were correlated with clinical manifestation, cytology, pathology and immunologic histochemistry. Ultrastructural features strongly indicated the difference in three various diseases, although all the immunologic markers showed B-cell type. It is concluded that electron microscopic examination is of a definite significance in the diagnosis and successful treatment.     

Hairy cell leukemia in father and son

Hairy cell leukemia (HCL) is an uncommon B cell disorder, and familial HCL is rarely encountered among the first degree relatives of HCL patients. A father and son, both of whom developed hairy cell leukemia, is presented in this report. The HLA haplotype shared by the father and son was A2, B18, BW6, CW7, DR3, DR10, and DQ8. Among these haplotypes, HLA A2 and Bw6 have previously been reported.