老年原发免疫性血小板减少症患者临床特征及预后分析

目的 探讨老年原发免疫性血小板减少症（immune thrombocytopenia,ITP）患者临床特征及影响预后的相关临床因素,为老年ITP研究提供依据。方法 回顾性分析2013年6月至2017年6月山西医科大学第二医院血液科收治入院的原发ITP患者,其中发病年龄≥ 60岁定义为老年ITP,分析患者临床表现及实验室相关检查,总结其临床特点,对于影响治疗效果及预后的危险因素进行统计学分析。结果 177例成人ITP患者中,年龄≥ 60岁的老年ITP有48例,平均发病年龄为（68.17±6.47）岁,男：女性别比为1：1,其中新诊断的ITP 30例（62.5%）,持续性ITP 6例（12.5%）,慢性ITP 12例（25%）。分别给予重组人促血小板生成素注射液、重组人促血小板生成素注射液联合地塞米松、静注丙种球蛋白、静注丙种球蛋白联合地塞米松、大剂量地塞米松治疗,治疗有效患者40例,有效率为83%;中位随访时间9.5个月（0.5~40.0个月）,复发患者12例,复发率为30%,中位复发时间1.0个月（0.5~14.0个月）。老年ITP患者抗核抗体（antinuclear antibody,ANA）阳性率（54.2%）明显高于非老年患者（23.3%）（P<0.05）,出血评分≥ 2分者比例占77.1%明显高于非老年组（出血评分≥ 2分者占49.6%）（P<0.05）。预后方面显示自然杀伤（natural killer,NK）细胞比例减低为影响疾病复发的危险因素（P<0.05）。结论 老年ITP患者出血评分更高,且更易合并高血压、糖尿病等基础疾病,应早期积极预防脑出血、重要脏器出血等合并症的发生。老年ITP更易合并ANA抗体阳性,提示应注意排查继发结缔组织病及其他潜在恶性疾病可能。NK细胞比例减低为疾病复发的危险因素,对于其作用机制有待于进一步研究。     

High doses of dexamethasone in adult patients with idiopathic thrombocytopenic purpura

BACKGROUND: High-dose dexamethasone (DXM) has been used in treatment of patients with idiopathic thrombocytopenic purpura (ITP) who are refractory to other treatments such as prednisone and splenectomy; nevertheless, different studies show variable success rates, this postulated as possibly being due to racial differences. The objective of this study was to determine DXM effectiveness at high doses in Mexican mestizo adult patients diagnosed with ITP with and without splenectomy. METHODS: Nonhospitalized adult patients with ITP were included, eight patients previously splenectomized (group 1) and 11 who had not undergone splenectomy (group 2). Patients received DXM 40 mg/day intravenously (i.v.) during 4 consecutive days every 4 weeks until six cycles were completed. RESULTS: There were no differences between the two groups regarding age (mean 39 vs. 33 years of age) and initial platelet count (M 17 vs. 24 x 10(9)/L). Median evolution time was 84 months for group 1 and 7 months for group 2 (p = 0.002). Of 19 patients, nine achieved a favorable response (FR), six belonged to group 1, and three to group 2 (Fisher p = 0.07). Nevertheless, after 6 months only two group 1 patients and two group 2 patients maintained FR (Fisher exact test p = 1). Patients achieving FR to initiation of second cycle maintained FR at the end of six cycles. CONCLUSIONS: Thus, the previously mentioned high-dose DXM therapy appears to be useful for both patients with ITP with and without splenectomy and high-dose DXM appears to be a good alternative therapy for postsplenectomy and relapse patients. However, duration of FR to treatment was brief; therefore, other treatment plans might be required to achieve longer remission duration. Response was similar to that observed in other studies carried out in different populations; thus, apparently no genetic or racial variations exist. In addition, whether patients not responding after second cycle should continue until completing the 6-month plan or should try a different therapeutic approach must be considered in the treatment plan.     

Helicobacter pylori eradication: a novel therapeutic option in chronic immune thrombocytopenic purpura

OBJECTIVE: To determine whether Helicobacter pylori eradication is an effective treatment for Australian patients with chronic immune thrombocytopenic purpura (ITP). DESIGN, SETTING AND PATIENTS: Retrospective analysis of clinical records of a consecutive series of ITP patients referred to a gastrointestinal surgeon in a tertiary referral hospital for laparoscopic splenectomy between August 2005 and November 2007. MAIN OUTCOME MEASURES: Platelet response (measured at least 3-monthly) following successful H. pylori eradication therapy (confirmed by urea breath test 4 weeks later). RESULTS: Of 16 patients, seven were H. pylori-negative and underwent laparoscopic splenectomy. Nine were H. pylori-positive and successfully underwent H. pylori eradication therapy; five of the nine had an initial platelet response. Four patients had platelet counts > 100 x 10(9)/L (reference range, 140-450 x 10(9)/L) and were off all immunosuppression at 9 months; three had a sustained response beyond 12 months. One patient had an initial response at 3 months (15 x 10(9)/L to 208 x 10(9)/L), but relapsed 4 months after H. pylori eradication and underwent splenectomy with platelet count recovery. The remaining four patients showed no platelet response and subsequently underwent splenectomy. CONCLUSION: Larger prospective studies are needed to fully ascertain the role of H. pylori in Australian patients with ITP. However, H. pylori eradication is simple and safe. H. pylori screening and eradication should be considered before immunosuppression or splenectomy.     

Coronary artery bypass grafting (CABG) in patients with immune thrombocytopenia (ITP): a community hospital experience and review of the literature

We reviewed the records of 51 patients with Immune Thrombocytopenia (ITP) who underwent Coronary Artery Bypass Grafting (CABG) at Charleston Area Medical Center between June 1992 and September 2005. There were 41 males and 10 females with a median age of 68 years (range 49-87). Four patients had a previous splenectomy, one of whom had it performed concomitantly with the CABG. Three patients were on chronic corticosteroids on admission. The median pump time was 114 minutes (range: 42-244 minutes). The median cross-clamp time was 62 minutes (range 22-192 minutes). The median total chest tube drainage postoperatively was 1,346 cc (range: 265-9875cc). The mean preoperative and 24 hour postoperative platelet count was 126,000 (range 58,000-323,000) and 99,000/mm3 (range: 27,000-194,000), respectively. Twenty-one (40%) patients received platelet transfusions. Platelets were given intraoperatively or postoperatively in all but two of those patients. The median number of units of platelets given was 10 (range: 6-52). Twenty-seven (53%) received packed red cells intraoperatively or postoperatively. The median number of red cells given was 2 (range: 1-34). Other hemostatic agents given intraoperatively/ postoperatively included aprotinin (8 patients), aminocaproic acid (10 patients), DDAVP (5 patients), and intravenous gammaglobulin (IgG) in 3 patients. Thirteen patients were given corticosteroids preoperatively with little improvement in platelet count. CABG may be successfully performed in ITP patients with moderate thrombocytopenia (> or = 50,000/mm3) using conventional therapies (e.g., transfusions, IV IgG, hematinics) without the need for preoperative splenectomy or prolongation of hospital stay. However, a prospective study on the ideal management of ITP patients undergoing CABG would be beneficial.     

脾切除治疗成人特发性血小板减少性紫癜的临床观察

目的:研究脾切除对成人内科治疗无效的特发性血小板减少性紫癜(ITP)的治疗效果。方法:对1990～2000年内科治疗无效的51例成人特发性血小板减少性紫癜患者进行脾切除手术。全组患者术后随访0.5～9年,平均46月。结果:术后血小板1周内恢复正常(≥100×10~9/L)者45例,占88.2％;术后2个月随访,总有效率90.2％。对41例患者进行中远期(0.5～9年)随访,总有效率为80.5％,4例复发,复发率9.75％。结论:术后血小板回升较早和回升幅度较高者预后较好。糖皮质激素治疗的效果不影响脾切除疗效。术前PAIgG检测值对预测脾切除疗效无相关性。选择性脾切除术对内科治疗无效的慢性ITP患者是一种安全和有效的治疗方法。     

193例成人免疫性血小板减少症临床分析

目的总结193例成人免疫性血小板减少症（immune thrombocytopenia,ITP）患者的临床特点,分析ITP常用治疗方法的效果和疾病预后。方法回顾性分析首都医科大学附属北京朝阳医院1990年~2009年193例住院ITP患者的临床资料及治疗效果。结果 193例成人ITP患者中,22.8%的患者发病前有感染病史,79.3%的患者多表现为慢性病程。有出血表现者共167例（86.5%）,皮肤淤点、淤斑最多见（77.7%）,5例（2.6%）患者发生脑出血死亡。发生出血患者发病时血小板计数较未出血患者更低（P=0.000）。患者中166例（86.0%）使用了糖皮质激素或以糖皮质激素为基础的联合方案,115例（69.3%）出现治疗反应者。发病时血小板低于20×109/L,治疗效果较好（P=0.011）;女性患者疗效优于男性（P=0.029）;15~40岁较40岁以上患者疗效好（P=0.000）;有出血情况者较无出血者疗效好（P=0.004）;骨髓巨核细胞数量、抗核抗体（nuclear antibody,ANA）阳性与否和疗效无关。46例（23.8%）患者出现治疗相关不良反应,其中12例（6.2%）为转氨酶升高,12例（6.2%）血糖升高,1例（0.52%）因严重肺部感染死亡。结论成人ITP患者慢性过程多见,糖皮质激素仍然是ITP的首选治疗方法,患者性别、年龄和治疗前血小板数量有助于预测治疗效果。ANA阳性的ITP患者应该长期随访,其有可能转化为自身免疫性疾病。     

伴EVI1高表达的中高危急性髓系白血病临床特点及早期治疗效果

目的：探讨EVI1基因阳性的急性髓系白血病（acute myeloid leukemia, AML）患者的临床生物学特点及其对早期化疗疗效的影响。方法： 选择2015年3月至2016年7月在北京大学血液病研究所诊治的361例AML患者病例资料进行回顾性分析,其中33例AML患者EVI1基因阳性,分析比较其临床特征和生物学特征,并比较中危及高危伴EVI1+AML患者的临床与生物学特征及诱导缓解率,分析获得完全缓解（complete remission, CR）的影响因素。32例健康供者进行EVI1/ABL基因水平检测,确定EVI1表达的异常界值。结果：以EVI1/ABL基因定量表达≥8.0%作为EVI1的阳性表达。在361例初发AML中,EVI1+AML患者共33例（9.1%）,其中男性16例,女性17例,中位年龄45（18~67）岁,中位随访期为6.6（0.7~13.2）个月。中危核型17例,包括正常核型9例,1例+8;高危核型14例,包括7例-7/7q-,4例t(v;11q23),3例inv(3)/t(3;3),2例未见分裂象。33例患者1个疗程完全缓解率为42.4%,总CR率为60.6%。按《美国国立综合癌症网络（National Comprehensive Cancer Network,NCCN ）指南》预后分层,分为中危组16例,高危组17例,无低危组患者。中危组与高危组1个疗程CR率分别为68.8%和17.6%（P=0.005）, 总CR率分别为81.3%和41.2%（P=0.032）,复发率为7.7%和14.3%。单因素分析,高危染色体核型对1个疗程CR率及总体CR率均有影响（P=0.004、0.029）。高危组患者病死率显著高于中危组（41.2% vs. 6.3%,P=0.039）, 且总生存（overall survival, OS）显著低于中危组（P=0.012）。结论: EVI1基因在AML中常伴中、高危核型表达,对于AML患者来说可能不是独立的预后因素,伴-7/7q-、t(v;11q23)及inv(3)/t(3;3)等高危染色体核型的预后差,其1个疗程CR率及总CR率、长期生存率低,病死率高,应尽早行异基因造血干细胞移植。     

腹腔镜脾切除术治疗免疫性血小板减少性紫癜——单个教学医院经验

Background  High anatomic location, fragility, and generous blood supply of the spleen makes laparoscopic splenectomy (LS) difficult to master, and few patients need splenectomy for benign disorders. The aim of this research was to assess operative outcomes and hematological results of a large series of patients treated with LS for chronic immune thrombocytopenic purpura (ITP) and to determine which clinical variables predict favorable hematological outcome.

Methods  LS was successfully performed for 154 patients with chronic ITP from September 1999 to April 2009 at the First Affiliated Hospital of Sun Yat-sen University. Operative outcomes were assessed retrospectively. Long-term follow-up data were obtained from outpatient medical records and phone interviews. Clinical and laboratory variables (including gender, age, disease duration before surgery, previous response to steroids, preoperative platelet count, and postoperative peak platelet count) were evaluated by univariate analysis to identify potential predictors of hematological outcome. Multivariate Logistic regression model was used to determine independent predictors of hematological outcome.

Results  One patient died from subphrenic abscess and postoperative sepsis. The overall major morbidity rate was 8.4%. None of the patients required a second surgery for complications. Of the 127 patients available for a mean follow-up of 43.6 months (range 9–114 months), the overall initial response (i.e., at two months after LS) and long-term response to LS were achieved in 89.0% and 80.3%, respectively. Five patients (3.9%) developed pneumonia 3–35 months after LS. Univariate analysis showed a significant difference in mean age between responders (29.1 years) and nonresponders (38.8 years; P <0.05). Patients who responded to steroid therapy had better hematological outcome than those who did not respond (P <0.05). Compared to nonresponders, responders to LS had a significantly higher postoperative peak platelet count (404×10⁹/L versus 213×10⁹/L, P <0.001). Multivariate Logistic regression analysis identified postoperative peak platelet count as the only independent predictor of favorable response to LS ( P <0.001).

Conclusions  LS is a safe and effective treatment for chronic ITP. Postoperative peak platelet count may serve as a major predictor of long-term response.

     

重症特发性血小板减少性紫癜21例临床分析

目的分析重症特发性血小板减少性紫癜（ITP）治疗的临床疗效及预后转归。方法对21例重症ITP患者初始给予糖皮质激素＋静脉免疫球蛋白±血小板输注的联合治疗方案;对幽门螺杆菌感染阳性的患者给予抗HP根治治疗;对多种治疗措施不能持久符合切脾指征的患者行切脾术;观察治疗后临床出血症状及血小板计数的变化。结果 21例联合方案治疗初始完全反应约占90%,无效约占10%;4例幽门螺杆菌检测阳性的患者给予抗HP治疗,2例有效;4例切脾患者3例完全反应。结论初始联合治疗方案对患者度过危险期,改善出血症状疗效显著;对ITP患者应常规行幽门螺杆菌检测;切脾治疗ITP效果肯定。     

异基因外周血干细胞移植治疗高危白血病

目的　探讨异基因外周血干细胞移植(allo-PBSCT)治疗高危白血病的疗效。方法　25例高危白血病患者,中位年龄34（5.5～52）岁,接受了HLA配型相合同胞供者外周血干细胞移植,其中急性白血病（AL）15例,（第一次完全缓解1例,为Ph染色体阳性,第二次及以上完全缓解期7例,复发7例,包括2例异基因骨髓移植(BMT)后复发;慢性粒细胞性白血病（CML）4例;（第2次慢性期、加速期、急变期、BMT后复发各1例）;骨髓增生异常综合征(MDS)6例,移植物抗宿主病（GVHD）预防方案采用经典环孢霉素（CsA）加氨甲蝶呤（MTX）。结果　所有患者均植活,中性粒细胞数恢复至≥0.5×109/L和血小板数≥20×109/L的中位时间分别为移植后14(10～18) d和11（7～45） d。发生II度及以上急性GVHD13例,包括1例III度GVHD,未发生IV度急性GVHD。23例可评估患者中16例(70%)诊断慢性GVHD。移植相关死亡率为16%,复发6例,4例经回输供者淋巴细胞获得再次缓解。19例患者无病存活,中位随访时间为304（94～1 963） d。2年总生存率、无病生存率及复发率分别为64%、58%及25%。结论　allo-PBSCT可治疗高危白血病患者,降低移植后复发率,延长无病生存。对有高危因素的血液系统恶性肿瘤患者,选择PBSCT替代骨髓移植更有优势。     

自体造血干细胞移植治疗恶性血液病

目的　评价自体造血干细胞移植 (AHSCT)治疗恶性血液病的疗效。　方法　用 AHSCT治疗白血病及恶性淋巴瘤患者共 5 8例 ,年龄 31.9± 10 .5 (14～ 5 8)岁。其中急性非淋巴细胞白血病 (ANL L ) 30例 (CR1 2 4例 ,CR2 5例 ,CR31例 ) ,急性淋巴细胞白血病 (AL L ) 18例 (CR1 13例 ,CR2 3例 ,CR32例 ) ,慢性粒细胞白血病 (CML ) 3例 (均获血液学缓解 ) ,恶性淋巴瘤 7例 (CR4例 ,PR3例 )。预处理化疗方案选用以下药物中任意两种或三种联合 :阿糖胞苷 3～ 4g/ m2 ,环磷酰胺 4～ 6 g/ m2 ,鬼臼乙叉苷 (VP- 16 ) 0 .5～ 1.0 g/ m2 。除 5例联合全身骨髓照射 (剂量为7～ 8Gy) ,其余均单用化疗。　结果　所有患者移植后均重建造血 ,移植相关死亡 1例 (1.72 % )。ANL L、AL L CR1期移植者 3年无病生存率分别为 5 2 .4%± 4.2 %和 46 .1%± 3.5 % ,复发率分别为 37.7%± 4.5 %和 40 .5 %±6 .7%。 10例 CML 和恶性淋巴瘤患者中 ,除 1例 期恶性淋巴瘤患者复发 ,余均获持续完全缓解。　结论　为降低白血病和恶性淋巴瘤的复发率 ,提高患者的无病生存期 ,无造血干细胞供者的 CR1 期白血病及恶性淋巴瘤患者应积极行 AHSCT。     

全身照射加足叶乙甙联合自体造血干细胞移植治疗非霍奇金淋巴 …

目的 评价全身照射（ＴＢＩ）加足叶乙甙（ＶＰ－１６）作为自体造血干细胞移植（ＡＨＳＣＴ）的预处理方法治疗非霍奇金淋巴瘤（ＮＨＬ）的疗效和安全性。方法 ２４例诱导治疗缓解的中高度恶性ＮＨＬ进行ＡＨＳＣＴ,其中２３例为首次缓解,１例为第３次缓解。自体骨髓移植（ＡＢＭＴ）１０例,自体外周血干细胞移植（ＡＰＢＳＣＴ）１４例,采用ＴＢＩ８００（７００－８５０）ｃＧｙ／Ｖｐ－１６７５７（２３－１１４０）ｍｇ／     

长期生存复发转移性乳腺癌患者的临床病理特征和预后因素分析

目的分析复发转移后生存5年以上乳腺癌患者的临床病理特征和预后因素。方法1996年1月至2002年12月就诊、确诊时为Ⅰ-Ⅲ期、在之后的治疗随访过程中出现复发转移,且复发转移后生存期长于5年的乳腺癌患者72例,分析其临床病理特征、预后因素及治疗情况。结果全组患者复发转移时中位年龄46岁,原发肿瘤≤5cm者80.5%;腋窝淋巴结转移0～3个者69.5%;受体阳性者74.5%;以软组织转移为主者69.4%;中位无病生存期为31个月。一线治疗后完全缓解率为42.5%,中位完全缓解持续时间达26个月。COX多因素分析显示,腋窝淋巴结转移数目是总生存期（P=0.03）和复发转移后生存期（P=0.04）的独立预后因素。结论复发转移后长期生存的乳腺癌患者通常具有更多有利的临床病理指标并且在复发转移后接受了合理的综合治疗。腋窝淋巴结转移数目是独立的预后因素,有长期预测总生存和复发转移后生存期的价值。     

成人T细胞急性淋巴细胞白血病治疗及预后分析

目的 探讨成人T细胞急性淋巴细胞白血病的治疗与预后。方法 回顾性研究2003年1月1日至2017年6月30日中国医学科学院血液病医院淋巴瘤中心收治的68例初治成人T细胞急性淋巴细胞白血病(T-ALL)患者,分析其疗效及预后。结果 68例成人T-ALL患者中位年龄23(14~60)岁,男性多见。第1疗程后完全缓解(CR)率73%,皮质T-ALL的CR率最高,其他T-ALL其次,早期前体T淋巴细胞白血病最低,分别为100%、73%、54%(χ ²=5.712,P=0.058);年龄>35岁病例组首疗程CR率低于≤35岁病例组(40%比79%,χ ²=6.364,P=0.012),性别、白细胞计数各组的首疗程CR率比较差异无统计学意义。第2疗程后总CR率93%。化疗、自体造血干细胞移植(auto-SCT)、异基因造血干细胞移植(allo-SCT)中位无进展生存时间分别为10个月、24个月、未达到(P=0.002)。5年总生存率为25%,化疗、auto-SCT、allo-SCT中位OS分别为24、34、30个月(P=0.007),5年总生存率分别为9%、33%、38%(P=0.037)。将性别、年龄、白细胞计数、达CR时间>4周、T亚型等因素进行多因素分析,结果显示白细胞计数≥100×10 ⁹/L是影响无复发生存时间的危险因素(风险比2.540,95%CI=1.058~6.099,P=0.037)。结论 成人T-ALL患者预后差,造血干细胞移植可能改善患者预后。     

部分性脾栓塞术治疗难治性ITP(附5例报告)

目的探讨部分性脾栓塞术(partial splenic embolization,PSE)治疗难治性特发性血小板减少性紫癜(idiopathic thrombocytopenia purpura,ITP)的临床疗效。方法对5例难治性ITP患者进行部分性脾栓塞治疗,观察和随访其临床疗效。结果5例病人中4例完全缓解,缓解率达80%;1例短期有效而复发,后行脾切除治疗。全部病例(包括脾切除术1例)血象基本恢复正常,随访至今均无复发。结论PSE不仅保留了脾脏的免疫功能,且手术创伤小,安全性好,并发症少,临床疗效满意,是治疗难治性ITP安全可靠的方法。     

慢性特发性血小板减少性紫癜患者Ⅰ类和Ⅱ类T细胞特点的研究

目的探讨慢性特发性血小板减少性紫癜（ITP）患者Ⅰ类和Ⅱ类T细胞的特点。方法采取30例慢性ITP患者外周血及8例慢性ITP患者和6例遗传性球形红细胞增多症患者的脾细胞（接受脾切除的患者），应用流式细胞仪技术检测辅助T细胞（Th）CD4^＋’和细胞毒性T细胞（Tc1）CD8^＋胞质内细胞因子干扰素γ（IFN-γ）／白细胞介素（IL）-4的比例；应用实时定量聚合酶链反应（PCR）检测T-bet和GATA-3 mRNA的表达水平。结果疾病活动期患者外周血Th1／Th2和Tc1／Tc2的比例（25．62和30．23）显著高于正常对照组（8．29和12．58，均P〈0．01）；缓解期患者外周血Th1／Th2和Tc1／T02的比例（9．86和10．10）与正常对照组相比，差异无统计学意义。ITP患者脾细胞Th1／Th2的比例（41．46）显著高于对照组（16．30，P〈0．01）；Tc1／T02的比例（35．80）高于对照组（16．88），但差异无统计学意义。疾病活动期患者外周血以及ITP患者脾细胞GATA-3mRNA水平明显低于对照组（是对照组的0．20和0．34倍，均P〈0．05），缓解期患者与对照组相比，差异无统计学意义（是对照组的0．97倍）。ITP患者外周血及脾细胞T-bet mRNA的表达水平与对照组相比，差异无统计学意义（是对照组的1．17和1．04倍）。结论ITP是Ⅰ类T细胞占优势的自身免疫性疾病。促进Ⅰ类T细胞模式向Ⅱ类T细胞转化可能为ITP患者提供一种潜在的免疫治疗方式。     

自体外周血CD34+细胞移植治疗非霍奇金淋巴瘤

目的 采用CD34+细胞体外分选技术对非霍奇金淋巴瘤(NHL)患者进行自体外周血CD34+细胞移植,观察其临床疗效.方法 5例NHL患者,女3例,男2例;中位年龄29岁;T细胞NHL 3例,弥漫大B细胞NHL 1例,真性组织细胞型NHL 1例;临床分期ⅡA 2例,ⅣB 3例;移植前4例处首次完全缓解,1例为部分缓解.采用Clini MACS临床型磁性细胞分离器收集CD34+和CD34-细胞组分,患者于预处理后输注分选的CD34+细胞.结果 Clini MACS体外分选可去除3.3个对数级CD34-细胞;回输CD34+细胞中位数为2.0×106/kg;CD34+回收率为52%,CD34+细胞纯度为86%;移植后白细胞恢复至＞0.5×109/L和血小板＞20×109/L天数(中位数)分别为12 d和19 d.患者总体生存率和无病生存率均为4/5,1年生存率为4/5,估计5年生存率为75%.结论 NHL患者自体外周血CD34+细胞移植后可获得迅速稳定的造血重建,体外CD34+细胞纯化富集后移植可望提高NHL患者自体移植疗效.     

Outcome in Hodgkin's disease: a 20-year cohort of patients.

We reviewed the long-term survival, treatment-related mortality and morbidity of a continuous cohort of patients with Hodgkin's disease diagnosed and staged at the Haematology unit of the Belfast City Hospital between January 1973 and October 1992. The analysis included a comparison of the survival of those patients who were entered into BNLI (British National Lymphoma Investigation) trials compared to those not entered during this 20 year period. In addition univariate and multivariate analysis of prognostic factors was performed. The complete remission rate (CR) was 79.6% with a 15 year survival of 55.3%. On multivariate analysis in which deaths due to active Hodgkin's disease only were considered age > 50 emerged as the most significant prognostic factor (P < 0.0007), the presence of B symptoms also having independent significance (P = 0.008). Trial status did not have any independent prognostic significance. Eighty one deaths occurred: active Hodgkin's disease (50), second malignancy (9), treatment-related (10), unrelated (9), unknown (3). This long-term follow up study provides useful information additional to the data produced by clinical trials which are biased by selection criteria. The occurrence of Haemophilus Influenzae meningitis in a patient 17 years following splenectomy highlights the need for appropriate vaccination of patients splenectomised for Hodgkin's disease.     

HIGH DOSE PULSE DEXAMETHASONE THERAPY IN CHRONIC IDIOPATHIC THROMBOCYTOPENIC PURPURA

We studied the response to pulse high dose dexamethasone (DXM) in 12 consecutive patients of symptomatic chronic idiopathic thrombocytopenic purpura (ITP) who had not responded to the standard forms of therapy. All patients had been exhibited to two or more cycles of prednisolone. Besides this 5 patients had also been given danazol and 4 patients had undergone splenectomy. Six cycles of dexamethasone (40 mg per day for 4 sequential days every 4 weeks) were successfully given in all 12 patients. At the end of the sixth cycle there were 7 (58%) complete responders (CR) [platelet count (PLT)100 × 10⁹ /L], 1 partial responder (PR) [PLT-50-100 × 10⁹/L] and 4 non responders (NR) [PLT < 50 × 107L]. Follow up at 1 year showed 4 (33.3%) patients each in CR, PR and NR group. Side effects noted were increased appetite in 8 (66.6%) patients, hyperalertness in 6 (50%) patients, abdominal discomfort in 5 (41.6%) patients and nausea with vomiting in 2 (16.6%) patients. We were not able to identify any chemical or laboratory prognostic parameter which would allow prediction of a successful outcome of this treatment.KEY WORDS: Dexamethasone, Idiopathic thrombocytopenic purpura