急性髓系白血病合并慢性淋巴细胞白血病一例报道及文献复习

 目的 探讨急性髓系白血病（AML）合并慢性淋巴细胞白血病（CLL）的临床特点、病因、诊断、治疗及预后。方法 临床诊断1例AML合并CLL,并就相关文献进行复习。结果 患者经MA方案（米托蒽醌10 mg／d第1 ~ 3天,阿糖胞苷150 mg／d第1,3,5,7天,200 mg第2,4,6天）化疗后取得完全缓解,但CD19阳性的淋巴细胞（表达CD20,CD23,SIgM,部分表达CD5,CD22,CD25）仍然存在,于9个月后AML复发未能再次缓解而死亡。结论 AML合并CLL为一种具有特殊生物学特征的罕见疾病,免疫分型和细胞遗传学技术在疾病的诊断和认识中发挥重要作用,治疗应以AML为主。     

B细胞型幼淋巴细胞白血病1例并文献复习

目的:提高对B细胞型幼淋巴细胞白血病（B-PLL）的诊治认识。方法:回顾分析了我院1例B-PLL患者的临床表现、骨髓细胞形态学、免疫分型、不良基因检测等临床资料以及治疗过程,并结合文献进行学习讨论。结果:B-PLL患者初诊时白细胞总数较高,脾肿大;骨髓形态学呈体积中等的幼稚淋巴细胞,核圆、核仁清晰、核染色质浓密、核浆比例低的特点;成熟B细胞免疫表型,免疫球蛋白轻链限制性表达,排除其他淋巴增殖性疾病,诊断为B细胞型幼淋巴细胞白血病。该患者TP53缺失,给予R-CHOP方案治疗,治疗反应差。结论:形态学检查和免疫表型是诊断B-PLL的重要手段,同时需整合临床表现、细胞遗传学等结果综合判断,TP53基因缺失或突变会导致这类患者传统化疗的预后不良。     

Chemotherapy of metastatic Merkel cell carcinoma: case report and review of the literature

Merkel cell carcinoma is a skin cancer that bears histologic, ultrastructural, and immunohistochemical resemblance to small cell lung cancer. Although this tumor is often localized and may be treated with wide excision, metastatic Merkel cell carcinoma does appear in 18%-28% of patients and may be aggressive and lethal. We report a case of metastatic Merkel cell carcinoma who responded to treatment with cisplatin/etoposide. Review of the literature reveals that doxorubicin/cyclophosphamide-based regimens and cisplatin-based regimens have been most commonly studied. Complete and partial responses are often achieved although the duration of response tends to be short. Further investigation of chemotherapeutic regimens similar to those used in small cell lung cancer should be undertaken.     

慢性淋巴细胞白血病/小B细胞淋巴瘤继发急性髓系白血病一例并文献复习

目的 提高对慢性淋巴细胞白血病/小B细胞淋巴瘤(CLL/SLL)继发髓系肿瘤的认识.方法 报道1例CLL/SLL继发急性髓系白血病患者,并对该病的发生率、危险因素及可能的机制进行文献复习.结果 CLL/SLL继发髓系肿瘤患者大多数伴有染色体异常,对接受以氟达拉滨为基础联合化疗的患者出现造血不良特征或血细胞减少应加强评估.结论 CLL/SLL继发髓系肿瘤少见,预后差,临床医师对具有危险因素的患者应提高警惕,争取早诊断、早干预.     

T-cell chronic lymphocytic leukemia: report of a case and review of the literature.

We recently observed a unique case of T-cell type chronic lymphocytic leukemia, documented by cell surface marker analyses. Immunologic tests at the time of diagnosis were as follows: 1) skin tests were negative; 2) in vitro lymphocyte responses to antigens or pokeweed mitogen were absent; 3) in vitro lymphocyte responses to phytohemagglutinin, concanavalin-A or allogeneic cells were low but significant; and 4) stimulating capacity of leukemic T cells were absent. Chromosomal analysis of bone marrow showed a pattern of 45 chromosomes with a marker chromosome. Terminal deoxynucleotidyl transferase activity was very low. Patient responded extremely well to COP (cyclophosphamide-oncovin-prednisone) therapy. Patient died of recurrent transitional cell carcinoma while his leukemia was in complete remission, approximately 20 months after the diagnosis of T-cell CLL.     

异时原发乳腺癌和皮肤Merkel细胞癌1例及文献复习

目的：探讨皮肤Merkel细胞癌的诊断及鉴别诊断、治疗及预后。方法：对1例异时原发乳腺癌和皮肤Merkel细胞癌患者的临床病史、肿瘤组织的病理免疫组化表达及治疗过程进行回顾性分析。结果：本例Merkel细胞癌患者表现为乳腺癌术后3年出现皮肤肿块，无乳腺癌复发和转移证据；免疫组化表达神经内分泌标志物和细胞角蛋白20（CK20）。于术后6月复发，术后4年死亡。结论：Merkel细胞癌罕见且进展快，即使综合治疗后仍有局部复发和远处转移的倾向，预后差。诊断主要依靠病理，免疫组化染色有助于鉴别诊断。临床上需结合病史与第一原发癌的转移相鉴别。     

Acute liver failure because of chronic lymphocytic leukemia: case report and review of the literature

Infiltration of the liver by hematologic malignancies is an uncommon cause of liver failure. B-Cell chronic lymphocytic leukemia (cll) is a usually indolent disease that may infiltrate the liver, but based on a review of the literature, has never been reported to induce acute liver failure. Here, we describe the case of a 78-year-old woman with acute liver failure secondary to infiltration with cll being unresponsive to chemotherapy and causing death. This case is notable because of its atypical presentation and ultimate poor prognosis.     

Merkel cell carcinoma after liver transplantation: a case report

BACKGROUND: Merkel cell carcinoma is a rare and aggressive neuroendocrine skin cancer with a very low incidence in the general population. MCC seems to be common in transplant recipients and 52 cases have been reported in the literature. METHODS AND RESULTS: This report describes a Merkel cell carcinoma which developed in a liver transplant recipient. To our knowledge, this is the second such case reported, as Merkel cell carcinoma most commonly occurs after kidney and heart transplants. The treatment approach is described and the literature on the subject is reviewed. CONCLUSION: There is currently no consensus regarding the optimal therapeutic approach to Merkel cell carcinoma. In transplant recipients, such tumors are more common and more aggressive but their treatment does not differ from the treatment of Merkel cell carcinomas in the general population.     

侵袭性NK细胞白血病1例并文献复习

目的:提高对侵袭性NK细胞白血病(ANKL)的认识.方法:报告1例ANKL患者并结合文献进行复习.结果:患者高热、肝脾肿大、进行性血细胞减少、外周血大颗粒淋巴细胞异常增多、骨髓中异常细胞增多并伴吞噬血细胞现象等,白血病细胞免疫表型为CD2、CD56阳性,CD3、CD4、CD8和CD57阴性,临床进展迅速.结论:ANKL为一种少见疾病,除临床表现外,诊断时需结合白血病细胞的免疫表型并需注意与其他相关疾病的鉴别,该病常呈侵袭性进展,治疗效果欠佳,预后极差.     

Merkel cell carcinoma: experience of 14 cases and literature review

Merkel cell carcinoma is an aggressive skin cancer, with a significant incidence of locoregional lymphnode involvement, which requires timely diagnosis, adequate staging and aggressive therapy based essentially on surgical procedures. The aim of this study is to report our experience and to compare our results with literature findings, in order to discuss the role of the procedures adopted and their influence on prognosis. From July 1995 to April 2005, 14 patients were treated and followed-up for MCC in the National Cancer Institute of Naples. Tumor location was: buttocks (43%), extremities (36%) head (7%), unknown (14%). There were 7 Stage I, 5 Stage II and 2 Stage III patients. Surgical treatment consisted in wide excision (WE) in Stage I cases, WE and regional lymphadenectomy followed by radio- or chemo-therapy in Stage II and combined surgical and pre- and post-operative medical treatments in Stage III. Overall disease specific survival rate was 64% (median follow up 44 months). Recurrence occurred in 86% of Stage I and 20% of Stage II patients and involved, in 83.3% of Stage I patients, the lymph nodal draining basin. The treatment of recurrence implied surgery and radio or radiochemotherapy. Overall survival rate of recurrent patients was 57% (median follow-up 37.2 months). Due to the particular lymphotrophism of MCC, major care should be set on investigation and treatment of tumor lymph nodal draining basin. As long as the disease remains surgically manageable the prognosis for patients with MCC is favourable. The role of radio and chemotherapy is not yet assessed.     

Merkel cell polyomavirus (MCPyV) in chronic lymphocytic leukemia/small lymphocytic lymphoma

Merkel cell polyomavirus (MCPyV) is a novel polyomavirus that shows a strong association with Merkel cell carcinoma (MCC). Recent studies have demonstrated MCPyV in some cases of chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL), a malignancy with a similar demographic as MCC. We tested for the presence of MCPyV by PCR and immunohistochemistry in 18 cases of CLL/SLL. Very low-level MCPyV DNA was detected in 33% of CLL/SLL cases by real-time PCR, but only one case demonstrated immunohistochemical positivity for MCPyV. MCPyV was not identified in 17 cases of follicular lymphoma, suggesting either that MCPyV is involved in CLL/SLL pathogenesis or that the immunodeficiency state of CLL/SLL induces low-level MCPyV reactivation.     

乳头状肾细胞癌4例报道及文献复习

目的:探讨乳头状肾细胞癌(PRCC)的临床和病理特征。方法:对4例乳头状肾细胞癌临床表现、病理特征及免疫组化表型进行观察分析并文献复习。结果:肿瘤组织呈乳头状或管状结构排列,乳头中心及间质内有泡沫细胞浸润,有明显出血坏死。结论:乳头状肾细胞癌诊断依赖病理特征及免疫表型,并需与其他有乳头状结构的肾恶性肿瘤进行鉴别。     

Aggressive squamous cell carcinoma of the skin after chronic lymphocytic leukemia

We report two cases of squamous cell carcinoma (SCC) of the skin subsequent to chronic lymphocytic leukemia (CLL). Both cases had an unusually aggressive course for a nonmelanoma skin malignancy with extensive metastases in both, resulting in death in one patient. A literature review supports the likelihood of an increased incidence of SCC in patients with CLL. Though the mechanism is unknown, immunosuppression may play a central role. We urge patients with CLL to avoid exposure to direct sun. Any questionable skin lesion should be biopsied early, and completely excised if it is a tumor. The patient should also be examined thoroughly for metastatic disease via subsequent follow-up visits.     

A case repot of Merkel cell carcinoma on chronic lymphocytic leukemia: differential diagnosis of coexisting lymphadenopathy and indications for early aggressive treatment

Background  

Chronic lymphocytic leukemia (CLL) is a monoclonal disorder, characterized by a progressive proliferation of functionally incompetent B lymphocytes. There is increased evidence of association between CLL and skin cancers, including the uncommon Merkel cell carcinoma (MCC).     

Merkel cell carcinoma in a patient with B-cell chronic lymphocytic leukemia treated with cladribine and rituximab.

Merkel cell carcinoma (MCC) is an uncommon, neuroendocrine skin tumor with an aggressive clinical course. The etiology of the disease is unknown, although sun exposure and immunosuppression may play a role in its development. Coexistence of MCC with chronic lymphocytic leukemia (CLL) is extremely rare and to our knowledge it has been previously described in only 8 patients. We report a 51-year-old woman who presented with a red lump on the right cheek diagnosed as MCC. She had been diagnosed as having CLL 3 years earlier and was treated with 4 courses of cladribine (2-CdA) and subsequently with 4 courses of 2-CdA combined with rituximab. MCC was diagnosed on the basis of histological and immunohistochemical evaluation 2 months after the last course of 2-CdA and rituximab. Surgical excision with tumor-free margins was performed and local adjuvant radiotherapy was applied. Histopathological and immunohistochemical evaluation of the cervical lymph node specimens showed monotonous and diffuse infiltrate of small CD5+, CD20+, CD23+ lymphocytes and no MCC cells were present. To our knowledge, this is the first reported case of MCC occurring in CLL patients soon after treatment with 2-CdA and/or rituximab. The development of MCC in our patient may suggest that this complication rarely observed in CLL patients may have a link with strongly immunosuppressive therapy with 2-CdA and rituximab.     

Merkel细胞癌术后复发1例及相关文献复习

目的：探讨Merkel细胞癌的诊断、治疗及预后。方法：一位老年Merkel细胞癌患者，于2006年8月初发时先行化疗及放射治疗，1年后局部再发肿瘤，再次接受扩大性切除术及放疗和化疗。结果：术后1月Merkel细胞癌再次复发。结论：Merkel细胞癌是一种罕见且进展快的皮肤肿瘤。诊断主要依靠病理（包括免疫组化方法），即使综合治疗后仍有局部复发和远处转移的倾向，预后差。     

Gastric metastasis of Merkel cell carcinoma,a rare cause of gastrointestinal bleeding: case report and review of the literature

Merkel cell carcinoma (MCC) is a highly aggressive cutaneous tumor of neuroendocrine origin. It is usually seen in elderly Caucasian males and occurs in sun exposed areas of the body. Diagnosis of MCC can be challenging and requires confirmation by immunohistochemical studies. It has an aggressive biological behavior with early local and distant metastasis and carries a dismal prognosis. However, metastasis of MCC to the stomach is very uncommon and rarely reported in the literature. We hereby describe a patient with gastric metastasis of MCC, who presented with upper gastrointestinal (GI) bleeding.     

Merkel cell carcinoma,chronic lymphocytic leukemia and other lymphoproliferative disorders: an old bond with possible new viral ties

Merkel cell carcinoma (MCC) is a rare and aggressive skin tumor. The link between tumorigenesis and immunosuppression is well known and the increased prevalence of MCC in human immunodeficiency virus carriers and organ transplant recipients and in patients with hemato-oncological neoplasias is now well recognized over the past decade. In this respect, chronic lymphocytic leukemia (CLL) seems to be the most frequent neoplasia associated with the development of MCC. Very recently, a newly described virus, the Merkel cell polyomavirus, was found in ∼80% of MCC tumor samples and is in fact the first member of the polyomavirus family to be associated with human tumors. The virus appears to play a role in the pathogenesis of MCC and may constitute the missing link between immunosuppression and the development of MCC. This review summarizes the current knowledge relating to MCC and its pathogenesis, stressing the link with hematologic neoplasias in general and to CLL in particular. We describe the permissive immunologic environment, which enables the virus-containing tumor cells to survive and proliferate in disorders like CLL. More studies are still needed to confirm this appealing theory in a more convincing manner.