18例毛细胞白血病的临床分析

目的探讨毛细胞白血病(HCL)的临床特点.方法回顾性分析18例HCL患者的临床资料.结果18例患者中,男13例,女5例,中位年龄51.5岁.腹胀是最常见主诉.初诊时脾大17例,肝大5例,淋巴结肿大6例.白细胞数增高6例,减低7例,正常5例.TRAP阳性15例, 阴性3例.网状纤维增多3例,板层复合体(RLC)14例中存在1例, 细胞遗传学改变7例中存在2例.11例单用干扰素治疗, 8例有效,3例无效中2例行脾切除仍有效.干扰素联合脾切除3例有效.结论HCL患者肝脾淋巴结肿大易见, 网状纤维增多不多见, RLC少见, 部分有细胞遗传学异常,干扰素联合脾切除是治疗的有效方法.     

老年骨髓增生异常综合征18例报告

1990年 1月至 1999年 12月 ,我院收治老年骨髓增生异常综合征 (MDS)患者 18例 ,现报告如下。临床资料 :本文男 11例、女 7例 ,年龄 6 0～ 80岁 ;均符合1982年 FAB协作组制订的诊断及分型标准。其中难治性贫血 (RA) 6例 ,难治性贫血伴环形铁粒幼细胞增多 (RAS) 2例 ,原始细胞增多的难治性贫血 (RAEB) 7例 ,慢性粒—单核细胞白血病 (CMML) 1例 ,转变中原始细胞增多的难治性贫血(RAEBT) 2例。其临床特点为 :贫血 15例 ,发热 10例 ,出血 4例 ,皮肤粘膜出血 5例 ,支气管—肺内感染 3例 ,浅表淋巴结肿大 4例 ,胸骨压痛 3例 ,肝脾肿大 2…     

3例确诊的肝脾γδT淋巴瘤临床诊断及特征分析

目的:探讨肝脾γδT细胞淋巴瘤(HSTCL)的临床表现、病理学特征、免疫表型特点。方法:回顾性分析3例肝脾γδT细胞淋巴瘤患者的临床资料并文献复习。结果:男性2例,年龄分别为18岁、62岁;女性1例,23岁;2例男性有肝脾大、B症状、EB病毒感染,其中1例有乙肝病毒感染; 3例均有血象减低、骨髓受累、肝功能异常、乳酸脱氢酶、铁蛋白升高、无淋巴结肿大。3例骨髓细胞学中幼稚细胞明显增多,胞体中等或偏大,胞浆量丰富,胞核类圆形或不规则,染色质细致,核仁明显;免疫分型均表达CD3、CD2、CD7、TCRγδ,部分CD56及CD16阳性,1例初始部分表达CD8,后不表达CD8,3例均不表达CD4、CD5、CD57、TCRαβ; 2例检测到TCRD单克隆重排基因片段,1例未检测到单克隆重排基因片段。3例接受化疗患者,2例因感染死亡,1例行异基因造血干细胞移植随访10个月仍完全缓解。3例患者从有临床症状到确诊时间分别为2个月、5个月、8个月,其中2例确诊到死亡时间分别为7个月、6个月。结论:肝脾γδT淋巴瘤常侵犯骨髓,早期行骨髓细胞流式免疫分型明确诊断;该型淋巴瘤对化疗多耐药,预后差,异基因造血干细胞移植可能是治疗方法。     

干扰素治疗血液病的进展

本文就干扰素治疗血液病的进展作一综述。一、治疗淋巴系统增生性疾病干扰素治疗毛细胞白血病(HCL)几乎100%有效。不仅对伴有脾大或无脾大的HCL有显著疗效,而且对化疗产生的抗药性、切脾治疗后复发以及晚期HCL患者同样可缓解症状。常用剂量为3×10~6U/m~2,肌肉或皮下注射,每日1次或3次,大部分患者一般于治疗后2～5个月内血红蛋白、     

毛细胞白血病的电子显微镜诊断与鉴别诊断(附8例分析)

目的:研究毛细胞白血病(HCL)细胞超微病理结构,总结电子显微镜的诊断和鉴别诊断要点。方法:分离8例HCL患者骨髓有核细胞,透射电镜观察毛细胞超微结构。结果:HCL细胞大多数为圆形或卵圆形,直径8~14μm,核圆,异染色质块状边集;细胞质中高尔基氏体和粗面内质网中度发达,核糖体丰富,糖原较少,5例HCL中有髓样结构。所有病例表面都存在绒毛或突起,其中6例短粗,属分枝型,2例为细长微绒毛,属细长型。8例中只有2例细胞质有核糖体-板层复合结构(RLC)。结论:细胞整体轮廓和表面微绒毛是HCL超微结构诊断的重要特征,核糖体-板层复合结构在HCL细胞中特异性不强。     

毛细胞白血病65例对切脾的反应:对脾重和骨髓受累的估价

毛细胞白血病(hairy cell leukemia,HCL)的特征是全血细胞减少,外周血有明显细胞浆突出的单核细胞,有中度至重度脾大而无明显淋巴腺病。对本病常用的治疗方法之一是脾切除术,据报道对许多病例有效。作者研究了一组施脾切除的HCL患者,旨在寻找脾切除术疗效的预后标志。 65例施脾切除的HCL患者,男54、女11例,年龄30～80岁。患者脾切除前1周内和切脾后2～12周的血细胞计数和脾重量资料齐全,切脾后随访1～3     

小儿急性巨核细胞白血病的诊断

急性巨核细胞白血病是一种特殊类型的白血病 ,被列入了急性髓细胞白血病的Ｍ7型。我们于1 998～ 2 0 0 0年诊断了 5例Ｍ7患者 ,发现其临床表现以及原始巨核细胞的细胞形态学、细胞化学染色、血小板单克隆抗体检查以及病理学活检有区别于其他ＡＭＬ患者及原始细胞的特点。现报告如下。1　资料与方法1 .1　临床资料本组 5例 ,男 3例 ,女 2例。年龄分别为 1岁 6个月、1 0个月、1 2岁、8岁及 3岁 5个月。就诊时有进行性贫血 ,发热 ,无出血 ,3例肝轻度肿大 ,2例脾轻度肿大 ,3例淋巴结肿大 (详见表 1 )。1 .2　检查方法参照ＦＡＢ的诊断标准 ,在光…     

非霍奇金淋巴瘤并发Hunt Ⅰ型综合征1例

1　病例介绍患者 ,女 ,35岁。因发现左颈肿物 1月于 1 999年 7月 7日入院。无发热、盗汗和消瘦。查体 :左颈后触及 3个 1 .0 cm× 0 .5cm大小淋巴结 ,质中 ,无压痛。心肺无异常。肝肋下未及 ,脾肋下 2 .5cm。左颈淋巴结活检报告 :非霍奇金 B细胞型恶性淋巴瘤 ,高分化。骨髓病理检查 :非霍奇金恶性淋巴瘤的骨髓肉芽肿病变 (淋巴 -巨细胞型 ) ,可疑淋巴瘤细胞浸润 ;网状纤维组织增生。血常规 :WBC1 .61×1 0 9/ L,RBC 3.36× 1 0 12 / L ,Hb 1 0 7g/ L,血小板数49.4× 1 0 9/ L。骨髓象 :淋巴细胞比例增多。诊断 :非霍奇金 B细胞型淋巴…     

Ph‘染色体阳性的急性白血病临床疗效观察

大多数急性白血病患者都有染色体异常，Ph＇染色体阳性见于＞90％的慢性粒细胞性白血病，而急性白血病出现Ph’染色体则较少见。我院自开展形态学、免疫学和细胞遗传学的MIC分型以来，有5例急性白血病患者染色体检查出现Ph＇阳性，现报告如下。1病例介绍例1男，37岁，干部。因头晕、乏力伴低热半月余于1995年11月9日入院。体检：轻度贫血貌，全身皮肤及粘膜无出血点及紫级，全身残表淋巴结无肿大，肝右助下3cm，脾左肋下5cm。例2女，58岁，农民。因头晕、乏力伴牙龈出血1个月于1996年4月4日入院。体检：中度贫血貌，全身浅表淋巴结无肿大…     

组织胞浆菌病四例

[例 1]患者男 ,4 8岁。因畏寒发热 2 0余日入院。入院体检 :体温 3 9℃ ,脉搏 10 2次 /ｍｉｎ ,消瘦 ,浅表淋巴结不肿大 ,心肺未见异常 ,肝肋下 7ｃｍ ,脾肋下 2ｃｍ ,质均中 ;血沉2 8ｍｍ/ｈ ;Ｂ超示肝脾肿大 ;骨髓检查于网状细胞内查到组织荚膜胞浆菌 ,余检查未见异常。诊断 :播散型组织胞浆菌病 ,给予氟康唑、大蒜液和伊曲康唑治疗 1个月后体温降至3 8℃以下 ,4 0ｄ后体温正常 ,药物减量。抗真菌药治疗 3个月后复查Ｂ超肝脾无异常而出院。[例 2 ]患者男 ,3 4岁。持续高热 2个多月 ,全身散在出血点 3ｄ入院。曾于外院疑诊为流行性感冒、恶…     

Splenic abscesses due to Mycobacterium tuberculosis in patients with AIDS

Among 60 patients with AIDS seen at our institution, two had splenic abscesses due to Mycobacterium tuberculosis without pulmonary tuberculosis. In both cases splenic abscess was the first manifestation of AIDS; the patients had prolonged fever and had lost weight and experienced malaise; slight hepatomegaly was noted in both instances and peripheral lymphadenopathy in one. Chest radiography gave normal results in one case and showed hilar lymphadenopathy in the other. Ultrasonographic findings were characteristic: homogeneous hepatomegaly and splenomegaly, with multiple filling defects of variable size. Diagnosis required splenectomy in one case and biopsy of cervical lymph nodes in the other. In both cases Ziehl-Neelsen staining gave positive results; M. tuberculosis grew from a culture of splenic tissue of one patient and from a culture of lymph nodal tissue of the other. There was a rapid response to antituberculous therapy. Splenic tuberculosis seems to be a distinct extrapulmonary entity in patients with AIDS. Ultrasonographic images are useful for diagnosis and follow-up.     

Splenectomy in chronic lymphocytic leukaemia.

In a retrospective study it was endeavoured to evaluate the effects of splenectomy in chronic lymphocytic leukaemia (CLL) characterised by splenomegaly. The material comprises 42 patients subjected to the operation in the course of the past 20 years. In the majority the spleen weighed more than 1000 g. The main indication for splenectomy was anaemia, while in 9 cases it was thrombocytopenia and in 14 cases hypercatabolism. Splenectomy is followed by a pronounced increase in the venous haemoglobin level and platelet count to higher values which have been recorded for up to 3 years after the procedure. In cases where data were available, there has been weight gain and a falling basal metabolic rate. Splenectomy is effective especially in cases predominated exclusively by splenomegaly, but even in cases with marked extrasplenic manifestations, splenectomy often greatly reduces the need for prednisone and cytostatics. Increasing hepatomegaly and lymphomas were not more common after splenectomy than in a control series, and the incidence of infections was not increased after the operation. For comparison, 37 non-splenectomised patients with splenomegaly were assessed. X-radiation of the spleen seems to be insufficient, since usually it has to be repeated. Splenomegaly does not decrease spontaneously and rarely after treatment with prednisone/cytostatics. The findings indicate that splenectomy of patients with CLL and increasing splenomegaly should be performed more often and presumably also earlier than recommended in the literature.     

Splenectomy in Chronic Lymphocytic Leukaemia

In a retrospective study it was endeavoured to evaluate the effects of splenectomy in chronic lymphocytic leukaemia (CLL) characterised by splenomegaly. The material comprises 42 patients subjected to the operation in the course of the past 20 years. In the majority the spleen weighed more than 1000 g. The main indication for splenectomy was anaemia, while in 9 cases it was thrombocytopenia and in 14 cases hypercatabolism. Splenectomy is followed by a pronounced increase in the venous haemoglobin level and platelet count to higher values which have been recorded for up to 3 years after the procedure. In cases where data were available, there has been weight gain and a falling basal metabolic rate. Splenectomy is effective especially in cases predominated exclusively by splenomegaly, but even in cases with marked extrasplenic manifestations, splenectomy often greatly reduces the need for prednisone and cytostatics. Increasing hepatomegaly and lymphomas were not more common after splenectomy than in a control series, and the incidence of infections was not increased after the operation. For comparison, 37 non-splenectomised patients with splenomegaly were assessed. X-radiation of the spleen seems to be insufficient, since usually it has to be repeated. Splenomegaly does not decrease spontaneously and rarely after treatment with prednisone/cytostatics. The findings indicate that splenectomy of patients with CLL and increasing splenomegaly should be performed more often and presumably also earlier than recommended in the literature.     

Chronic active Epstein-Barr virus infection mimicking Henoch-Schönlein purpura

INTRODUCTION: Chronic active Epstein-Barr virus (CAEBV) infection is characterized by chronic or recurrent symptoms for at least 3 months, such as fever, hepatosplenomegaly and lymphadenopathy. The diagnosis is established due to the presence of anti-EBV antibodies or isolation of this infectious agent in affected tissues. Three cases of CAEBV infection mimicking Henoch-Sch?nlein purpura (HSP) were described. CASE 1: Female 3-year old patient with cervical adenomegaly, anemia and fever developed palpable purpura, haematuria and arthritis. CAEBV infection was established by serology test. She received methylprednisolone and acyclovir. She had generalized lymphadenopathy, hepatomegaly, splenomegaly, disseminated intravascular coagulation and deceased. CASE 2: Male 12-year old patient with persistent anemia, lymphadenopathy, hepatomegaly and splenomegaly had CAEBV infection diagnosis by serology test. He developed purpura and arthritis and received methylprednisolone. CASE 3: Male 13-year old patient had purpura, abdominal pain, haematuria, hepatomegaly, splenomegaly, lymphadenopathy, anemia and elevated liver enzymes. The cervical lymph node biopsy was positive to EBV infection. He received methylprednisolone and acyclovir, developing acute fulminant hepatitis and death. DISCUSSION: CAEBV infection mimicking HSP was rarely observed in our population.     

40例毛细胞白血病临床特征分析

目的:研究我国毛细胞白血病(HCL)的临床及实验室检查特点。方法:回顾性总结40例HCL患者的临床及相关实验室检查资料。结果:①脾大是HCL的最主要的症状体征,而全血细胞减少者比例较少,在经典型中也仅占1/3左右;变异型白细胞计数常增高;②电镜检查核糖体-板层复合物的阳性率仅有20%～30%;③多色流式细胞学表型分析发现典型的毛细胞表达CD19、CD20、CD22、CD25、CD103、CD11c和sIgM,是诊断的重要依据;④核苷类似物治疗HCL疗效优于其他治疗,值得推荐;⑤变异型HCL比例较高,表现不典型,且治疗效果差。结论:我国HCL中全血细胞减少者比例较低,电镜核糖体-板层复合物的阳性率低,而毛细胞免疫表型与国外类似,核苷类似物治疗效果也较好。变异型HCL比例高于国外,治疗反应差。     

Interferon is effective in hairy-cell leukaemia

Seventeen patients with hairy-cell leukaemia (HCL) and peripheral cytopenias were given human lymphoblastoid interferon (Wellferon), 3 megaunits daily or 6 megaunits on alternate days intramuscularly, for 4-24 weeks. Twelve of the patients had undergone splenectomy, three had no palpable spleen and had therefore not been offered surgery, and two patients with substantial splenomegaly were given interferon (IFN) as treatment of first choice. Toxic effects were minor except in one patient who experienced a severe form of somnolence syndrome. In all patients hairy cells (HCs) were cleared from the blood and platelet and Hb levels improved in 2-14 weeks. Neutrophils were improved in 14/17 of the patients. In the two patients with splenomegaly, the spleen became impalpable after 5-8 weeks therapy, and haematological improvement occurred at 12-14 weeks. HC infiltration of the marrow was reduced in all patients, but was complete (less than 5%) in only two, both of whom had impalpable spleens. Immunological surface-marker studies confirmed that light-chain-restricted B cells disappeared from the blood in parallel with the clearance of morphological HCs. There was no evidence of HC maturation and no increase in phenotypic NK cells. T cells were moderately reduced and the relatively greater reduction of Leu 2a+ suppressor cells resulted in increased Leu 3a+/2a+ helper/suppressor ratios in 11/17 of the patients. Early experience in the six patients who have stopped IFN suggests that, after an initial further increase in Hb and neutrophil levels, HCs gradually return with slow deterioration of haematological parameters. Interferon is now the treatment of choice for patients becoming cytopenic post-splenectomy or for patients without splenomegaly. IFN is effective first-line therapy in patients with splenomegaly, but further work is needed to establish whether the agent should replace splenectomy in such patients. Some form of maintenance or re-treatment therapy will probably be necessary.     

A single cycle of 2-chlorodeoxyadenosine results in complete remission in the majority of patients with hairy cell leukemia.

Twenty-six patients with hairy cell leukemia (HCL) were treated with 2-chlorodeoxyadenosine (2-CdA), a purine analogue resistant to adenosine deaminase, at 0.1 mg/kg/d for 7 days by continuous intravenous infusion. Fifteen patients were previously untreated, while 11 patients had received prior treatment with splenectomy alone (three patients), interferon alpha alone (four), splenectomy, then interferon alpha (two), or splenectomy, interferon alpha, then 2-deoxycoformycin (2-DCF) (two). Sixteen (80%) of 20 patients evaluable at 3 months achieved complete remission (CR), and four (20%) achieved partial remission (PR) following a single cycle of therapy. All four patients in PR had complete recovery of their peripheral blood counts (except one patient whose platelet count remained 84,000/microL), but had residual HCL in the bone marrow (three patients) or residual splenomegaly (one). Patients with bulky adenopathy, massive splenomegaly, and severe pancytopenia responded as well as those with only modest marrow involvement. The three patients with residual marrow disease received a second cycle of 2-CdA, and two have attained CR. Therefore, 18 of 20 (90%) achieved CR with either one or two cycles of therapy. No patient achieving CR has relapsed at a median follow-up of 12 (+/- 2.1) months. Toxicities included myelosuppression and culture-negative fever. A community-acquired pneumonia was the only infectious complication. Since a single cycle of 2-CdA induces sustained CR in the vast majority of patients with minimal toxicity, this agent is emerging as the treatment of choice for all patients with HCL.     

A variant form of hairy cell leukemia resistant to alpha-interferon: clinical and phenotypic characteristics of 17 patients

We describe the clinical and laboratory features of 17 adult patients with a variant form of hairy cell leukemia (HCL-V) studied over the last 7 years. The main findings were: splenomegaly, moderate anemia, thrombocytopenia, and a raised white blood cell count (median 116 x 10(9)/L; range 15 to 482). The circulating lymphoid cells had abundant villous cytoplasm and a round, occasionally bilobed nucleus, with a prominent nucleolus. Monocytopenia, a feature of typical HCL, was not seen; neither was tartrate-resistant acid phosphatase demonstrated in eight cases tested. HCL-V cells had a mature B-cell phenotype: CD19+, CD20+, CD22+, FMC7+, CD11c+, CD10-, CD5-, with light chain isotope restriction in 15 cases. In contrast to typical hairy cells, HCL-V cells were negative with the monoclonal antibodies anti-HC2 and anti- TAC (CD25). Immunoglobulin (Ig) was not detected in two cases and IgG was expressed in the cell membrane of 73% of cases. Bone marrow histology was different from HCL, showing interstitial infiltration by cells clumped together and a moderate amount of reticulin, but the spleen showed the typical red pulp expansion of HCL. HCL-V patients did not respond to splenectomy (5 of 7) or alpha-interferon (7 of 7); 2 of 3 patients had a partial response to 2'deoxycoformycin. The clinical course was benign with 15 patients alive with a median survival greater than 4 years. We confirm that HCL-V is a distinct clinico-pathologic entity with intermediate features between HCL and B-prolymphocytic leukemia.     

Successful treatment with low-dose splenic irradiation for massive splenomegaly in an elderly patient with hairy-cell leukemia

The therapeutic approach to hairy-cell leukemia (HCL) is in some instances still debated. Although management with alpha-interferon and purine analogues is well established, there is an alternative role for therapeutic splenectomy in patients with massive splenomegaly who have failed to respond to systemic therapy. Most patients with HCL will not be suitable for treatment with splenectomy as their ages at diagnosis are high. Here, we report an elderly Japanese HCL patient whose refractory massive splenomegaly responded well to low-dose splenic irradiation.