系统性红斑狼疮142例临床分析

目的探讨系统性红斑狼疮(SLE)患者的临床特点。方法对142例SLE患者外周血象、实验室免疫检查结果及主要临床表现进行总结分析。结果142例中血象异常者121例,其中贫血79例,血小板减少65例,白细胞减少76例,二系减少者69例,全血细胞减少15例,给予糖皮质激素及(或)免疫抑制剂治疗后血象有显著改善(P<0.01〉。血细胞减少组抗dsDNA、补体C3、C4、24 h尿蛋白、肾损害、浆膜炎与血细胞正常组比较有统计学意义(P<0.05)。结论SLE易并发血液系统损害,表现多样,经糖皮质激素及免疫抑制剂治疗,其外周血和骨髓象均有不同程度的改善。血细胞减少组抗dsDNA、补体C3、C4、24 h尿蛋白、肾损害、浆膜炎与血液系统受累明显相关,补体C3可作为判断SLE病情复发及缓解的一个敏感而可靠的指标。     

系统性红斑狼疮65例血液学异常临床意义分析

目的探讨系统性红斑狼疮(SLE)患者血液学改变特点。方法对2001年1月～2008年12月本院65例SLE患者血常规、血沉进行检测,部分患者行骨髓检查、骨髓铁染色和溶血相关实验。结果65例SLE患者血常规正常34例,一系减低31例,两系减低19例,三系减低9例;血沉增快56例;25例行骨髓检查,增生明显活跃13例,活跃9例,减低3例;19例同时行骨髓铁染色,7例细胞外铁(2+),6例(+),6例阴性;Coomb′s试验阳性8例,7例为温抗体型,1例为冷抗体型。结论SLE常累及造血系统,相关实验室检查异常,血液学异常具有多样性。     

间充质干细胞移植治疗系统性红斑狼疮的研究

系统性红斑狼疮(SLE)是自身免疫性疾病,传统治疗方法主要有糖皮质激素和免疫抑制剂等,由于长期用药部分患者出现继发感染、复发及疗程长、药物不良反应等问题,对难治性、重症SLE效果欠佳。近年来,越来越多的证据表明SLE患者有间充质干细胞(MSCs)功能异常。因此,MSCs移植治疗SLE成为一种新的方法。该文简要介绍了SLE的发病机制、MSCs的免疫学特性以及干细胞移植治疗SLE的现状。     

血细胞减少的系统性红斑狼疮患者骨髓细胞学表现

骨髓作为较常受累的狼疮靶器官已是共识.新近研究表明系统性红斑狼疮(SLE)的发病可能与骨髓造血干细胞异常有关^[1].本研究对31例SLE患者治疗前的骨髓细胞进行回顾性调查,以了解其骨髓象特点及变化.     

母细胞性浆细胞样树突状细胞瘤

本文报告母细胞性浆细胞样树突状细胞瘤(BPDCN)1例。患者男,70岁。反复皮肤瘙痒伴红斑结节1年。皮损组织病理:瘤细胞在真皮内密集,呈结节、团块状浸润,核深染,形态不规则,可见病理性核分裂象。免疫组化:CD4、CD56、CD123、CD43、CD45RA均阳性。骨髓涂片:骨髓增生明显活跃,异常细胞占72.8%,骨髓流式免疫分型:人白细胞DR抗原(HLA-DR):98.6%,CD56:82.3%,CD38:83%,CD4:92%和CD123:94.6%。该文对BPDCN的诊断、鉴别诊断和治疗进行讨论,揭示该病呈高度侵袭性,预后差,生存期短。     

94例系统性红斑狼疮患者的血液及尿液变化

系统性红斑狼疮(SLE)常合并多种血液及尿液异常,易误诊为某些血液系统疾病.为减少SLE的误诊,笔者对2001年1月～2006年6月我院收治的94例SLE患者资料进行了总结分析,现报道如下.     

结节性非化脓性脂膜炎合并骨髓增生异常综合征一例

我科曾收治1例确诊为结节性非化脓性脂膜炎(Weber-Chrislian脂膜炎)患者,经糖皮质激素及硫唑嘌呤治疗后痊愈。20个月后患者因贫血待查行骨髓穿刺,发现骨髓明显病态造血,诊断为骨髓增生异常综合征(MDS)。     

结节性类天疱疮七例临床病理分析

目的 回顾分析结节性类天疱疮的临床特点。方法 回顾分析7例结节性类天疱疮患者的性别、发病年龄、临床表现及治疗和随访情况。结果 7例结节性类天疱疮患者中,女4例、男3例,发病年龄中位值59岁。临床表现以痒疹样皮损、结节为主,伴或不伴水疱,瘙痒症状明显,确诊前均误诊为结节性痒疹、湿疹。所有患者病理表现均有表皮增生肥厚,可见表皮下裂隙,真皮乳头胶原增生,浅层血管周围淋巴细胞、嗜酸性粒细胞浸润;直接免疫荧光检查基底膜带IgG和C3呈线状阳性,间接免疫荧光有2例阳性。7例联合使用糖皮质激素和免疫抑制剂治疗有效。结论 结节性类天疱疮临床易误诊,免疫病理检查有利于诊断,糖皮质激素和免疫抑制剂治疗有效。     

45例大疱性类天疱疮初次住院患者临床回顾性分析

目的总结45例初次住院的大疱性类天疱疮患者的临床特点及治疗经验。方法分析2009年8月—2014年12月45例住院治疗的大疱性类天疱疮患者临床表现、组织病理、免疫病理、治疗方案及并发症等方面的情况。结果 45例住院患者86.67%为老年人,均表现为瘙痒性紧张性大疱,普通组织病理表现为表皮下水疱,直接免疫荧光检查见IgG和(或)C3在基膜带呈线状沉积。6例患者转科或自动出院,入院时均存在低白蛋白血症。余39例患者好转或临床治愈出院,总有效率为86.67%。39例患者中9例单纯使用糖皮质激素治疗,30例糖皮质激素联合免疫抑制剂治疗(主要为甲氨蝶呤)。单纯糖皮质激素治疗组的住院天数显著少于糖皮质激素联合免疫抑制剂治疗组(P0.05)。糖皮质激素联合使用免疫抑制剂治疗感染发生率更高(P0.05)。结论系统使用糖皮质激素是大疱性类天疱疮患者住院治疗的首选,视病情联合免疫抑制剂治疗可以增加疗效,但感染发生率会增加,要警惕低白蛋白血症的危险性。     

儿童系统性红斑狼疮并发深部真菌感染56例临床分析

正系统性红斑狼疮(systemic lupus erythematosus,SLE)是一种累及全身多个系统的自身免疫性结缔组织疾病,糖皮质激素类药物和免疫抑制剂药物的广泛应用,明显提高了SLE患儿的生存率,但也因此降低了患儿的免疫力,使感染概率显著增加,尤其是机会性真菌感染,且出现真菌感染时易误诊为狼疮累及脏器,丧失早期诊断及治疗时机。为探讨儿童SLE并发深部真菌感染的临床特点及相关因素,我们对56例并发深部真菌     

儿童 SLE 53 例分析

目的 探讨儿童SLE的临床及实验室特点。方法 对53例儿童SLE的临床资料进行回顾性分析。结果 儿童SLE患者存在明显性别差异,男女比例为1 ∶ 9.6。年龄7 ~ 14岁,中位数为12岁。首发症状以皮疹最为常见,占41.51%;其次为发热占20.75%;关节痛占20.75%。系统损害中最常受累的是血液系统,占84.90%;肾脏占60.38%,神经系统也易受累,占18.87%。血液、肾、神经系统受累最常见的表现分别为贫血、蛋白尿、癫痫。实验室检查ANA阳性率最高,占90.57%,其次为抗dsDNA抗体,占67.92%。男女在发病年龄、入院评分、出院评分、糖皮质激素起始剂量、出院剂量、住院时间等方面差异均无统计学意义。结论 儿童SLE临床表现多样,倾向于多系统受累。应提高警惕,早期诊断积极治疗。     

127例系统性红斑狼疮患者超声心动图特点的临床研究

目的:探讨系统性红斑狼疮(systemic lupus erythematosus,SLE)患者的超声心动图特点。方法:回顾性分析2004年1月至2011年6月确诊的127例SLE患者的超声心动图检查资料。结果:114例(89.76%)SLE患者存在心脏损害,其中瓣膜反流发生率最高,占76.38%(97/127),以二尖瓣、三尖瓣反流最为常见;其次为心脏结构改变24.41%(31/127)和心包积液23.62%(30/127)。重度活动SLE患者的左室射血分数减低的发生率明显高于轻中度活动的SLE患者(χ2=6.00,P<0.05),病程≥5年的SLE患者肺动脉高压的发生率明显高于病程<5年的SLE患者(χ2=4.68,P<0.05)。结论:系统性红斑狼疮患者心脏改变发生率高,且随着病情变化,超声心动图显示瓣膜功能也呈现易变性,早诊断和合理治疗可逆转早期心血管改变。     

Case of Pediatric Bullous Systemic Lupus Erythematosus Treated with Intravenous Immunoglobulin

This is a case report of a 16‐year‐old girl recently diagnosed with systemic lupus erythematosus (SLE) who presented with multiple blisters on the face, hands, arms, legs, trunk, and vaginal and oral mucosa. Skin biopsy was consistent with bullous SLE (BSLE). Dapsone is often the first‐line treatment option for BSLE, but the patient's history of anemia and leukopenia and long‐term immunosuppression requirement for her systemic symptoms raised concerns about dapsone and bone marrow toxicity, especially hemolytic anemia and agranulocytosis. She was started on intravenous immunoglobulin (IVIG), 2 g/kg divided over 3 days, with significant improvement in her cutaneous symptoms. IVIG is a treatment option for BSLE patients in whom agents such as dapsone are contraindicated.     

Myelodysplastic syndrome following treatment of malignant melanoma with vincristine, ACNU, and dacarbazine

Therapy-related myelodysplastic syndrome is a rare adverse effect in melanoma patients elicited by chemotherapy. We report a case of myelodysplastic syndrome following treatment of malignant melanoma with alkylating agents. Peripheral blood showed a remarkable suppression of three cell lineages, and the bone marrow was slightly hypercellular. However, no morphological abnormalities were detected in the peripheral blood or the bone marrow, and chromosomal analysis was normal.     

Leukaemic dissemination of Merkel cell carcinoma in a patient with systemic lupus erythematosus

We describe an unusual bone‐marrow metastasis of Merkel cell carcinoma (MCC) arising in the right cheek of a 73‐year‐old woman with systemic lupus erythematosus (SLE) and Sjögren’s syndrome, who had been treated with oral prednisolone and methotrexate for 10 years. Seven months after wide local excision followed by local irradiation, the patient presented with thrombocytopaenia. Her bone marrow had been completely replaced by metastatic MCC cells, and metastatic cytokeratin 20‐positive cells were also identified in the peripheral blood. To our knowledge, in the English literature, only six cases have been described previously of MCC bone‐marrow involvement. Of these six cases, four were immunosuppressed, similar to our case. The high incidence of MCC in immunosuppressed patients such as those with SLE has been discussed previously. We consider that immunosuppression might be associated with bone‐marrow metastasis, which is a rare form of MCC.     

Long-term extracorporeal photoimmunotherapy for treatment of chronic cutaneous graft-versus-host disease: observations in four patients.

BACKGROUND: Chronic cutaneous graft-versus-host disease (GvHD) can arise as a late complication after allogeneic bone marrow transplantation. Patients with extensive disease to date require intensive early and long-term immunosuppression; however, treatment is often insufficient. Since the beneficial effects of phototherapy for chronic cutaneous GvHD are well known, extracorporeal photoimmunotherapy (ECP) was also tried with some success for a few single patients with this disease. OBJECTIVE: The long-term effect of ECP was evaluated in 4 patients with therapy-resistant severe chronic cutaneous GvHD after allogeneic bone marrow transplantation. METHODS: Four patients were treated with monthly sessions of ECP over a period of 16-40 months. Disease severity was assessed by a semiquantitative score adapted from the literature including extent of skin area involved, rigidity of the skin, involvement of joints and immunosuppressive drug consumption. RESULTS: In all patients, a favorable response was observed after 6-12 treatment cycles. One patient had a complete response, 2 patients had a partial response, and 1 patient had a minor response after treatment. In 2 patients, immunosuppressive medication started before initiating ECP could be reduced or completely withdrawn under ECP. Peripheral blood lymphocyte immunophenotyping revealed reduction of CD3+ CD4+ T cells in 3 patients and of elevated CD3+ CD8+ and CD57+ CD8+ T cell subsets in 2 patients. Conclusion: ECP is effective in treating severe chronic cutaneous GvHD. ECP possibly exerts its effects by reducing the number of CD8+ suppressor/cytotoxic T cells, the presumptive effector cells of GvHD. ECP is well tolerated with essentially no side effects and allows reducing the dosage of immunosuppressive agents.     

Eosinophilic pustule smear in the diagnosis of pediatric post-transplant eosinophilic folliculitis

Hematologic-associated eosinophilic pustular folliculitis is a subtype of eosinophilic pustular folliculitis (EPF) which develops in patients with underlying hematological malignancies after treatment with chemotherapy, bone marrow transplant (BMT), or stem cell transplant (SCT). Few cases of hematological-associated EPF have been reported in pediatric patients. Skin biopsy is considered the gold standard for diagnosis. We describe a case in which Wright staining of a pustule smear for eosinophils provided data to rapidly support a clinical diagnosis of hematologic-associated EPF.     

生物制剂治疗系统性红斑狼疮新进展

系统性红斑狼疮是一种表现多样的慢性自身免疫病，可累及全身。糖皮质激素及免疫抑制剂为主的传统药物对部分SLE患者的疗效欠佳，且长期应用毒副作用大。生物制剂的出现为SLE治疗带来新的选择，临床应用中也取得了一定成效。本文就生物制剂在红斑狼疮临床治疗中的新进展作一综述。     

Abnormal hematopoiesis in psoriasis--in vitro studies

Using mononuclear cells (MNC) of patients suffering from psoriasis for production of colony-stimulating factors (CSF) an abnormal colony formation in bone marrow of healthy volunteers was detected (colony-forming assay): Number of colonies per 10(5) bone marrow cells after cultivation with 10(6) MNC of patients suffering from psoriasis vulgaris: 65 +/- 14 (n = 8); psoriasis arthropathica: 63 +/- 11 (n = 8). Control values (10(5) bone marrow cells plus 10(6) MNC of healthy donors): 116 +/- 23 (n = 10). Statistical differences: psoriasis vulgaris: p less than 0.001; psoriasis arthropathica: p less than 0.001. The mechanisms responsible for these abnormalities in hematopoiesis are still unknown. They may be of pathogenic significance in psoriasis.     

Hypersensitivity to dacarbazine in patients with metastatic malignant melanoma

BACKGROUND: Dacarbazine (DTIC) is the first-line chemotherapy for metastatic malignant melanoma without cerebral metastasis. Its clinical and hematological safety is usually good. Hypersensitivity in hepatic failure patients is the most serious side effect described. PATIENTS AND METHODS: This was a retrospective study of the prevalence of hypersensitivity in patients treated with DTIC for metastatic melanoma between 11/01/2002 and 10/31/2003. Hypersensitivity was diagnosed in the event of fever, hypereosinophilia (> 500/mm3) with or without liver dysfunction (> twice pre-therapeutic values). Clinical data, DTIC administration modalities, number of courses and clinical and laboratory safety data were recorded. RESULTS: Twenty patients were included, 11 women and 9 men of median age 58.6 years (22-82 years) with multiple metastases in all cases. DTIC was the first-line treatment for 19 patients, being administered for 4 days to 10 patients and for 1 day to the other 10 patients, depending on their overall health status. Five hypersensitivity-like manifestations were observed, all in the 4-day treatment group. In 3 patients, fever and hypereosinophilia were seen without liver dysfunction at D3 of the second course of treatment. In 2 patients, treatment was stopped after the second course because of disease progression. In the third patient, 4 courses were given with recurrence of symptoms, although the latter were controlled during the fifth course with corticosteroids and antihistamines given 15 minutes before the start of treatment. Two patients experienced severe forms of hypersensitivity with fever, hypereosinophilia, liver dysfunction (cytolysis and cholestasis) and delayed medullar aplasia, after the first and second course respectively. In one patient, bone marrow examination showed a block at the promyelocytic stage consistent with a toxic etiology. Treatment with DTIC was stopped, and all signs regressed with symptomatic treatment. DISCUSSION: Hypersensitivity with DTIC seems to be frequent, being observed in 20% of our patients, with early onset (after the first or second course) and absence of dose-dependence. We describe for the first time two cases of medullar aplasia occurring in association with DTIC hypersensitivity. During phase I studies, the hematologic toxicity of DTIC was moderate, rarely affecting red cells, and was observed with higher doses than those used in metastatic malignant melanoma. We suggest that this aplasia forms part of the signs of hypersensitivity because of the bone marrow morphology, the existence of anemia and concomitant resolution with all the others signs of hypersensitivity. CONCLUSION: Laboratory monitoring (NFS, liver enzymes) is thus justified, particularly after the first and second courses of DTIC. In case of fever and hypereosinophilia without liver dysfunction, DTIC may be continued together with symptomatic treatment. In the event of hepatic dysfunction, and of course severe hematological disorders, potentially fatal complications can occur and treatment must be stopped.