急性淋巴细胞白血病化疗后可逆性后部白质脑病综合征一例并文献复习

目的 探讨急性淋巴细胞白血病巩固化疗后可逆性后部白质脑病综合征(RPLS)的临床表现、诊治及其预后情况.方法 回顾分析1例急性淋巴细胞白血病患者的临床及影像学资料并结合文献进行复习.结果 该患者主要临床表现为腹胀、反复发热,伴乏力症状,经血常规及骨髓相关检查,诊断为早前B细胞性高危组急性淋巴细胞白血病,经诱导缓解化疗及巩固化疗后患者出现血压增高及神经系统症状,结合影像学检查诊断为RPLS,经积极治疗后完全恢复,影像学表现迅速改善.结论 RPLS可由多种病因产生,临床表现及影像学检查缺乏特异性,一般预后较好,早期作出正确诊治是关键.     

Gemcitabine-induced pulmonary toxicity: case report and review of the literature

Gemcitabine is a pyrimidine analog with a similar chemical structure and mechanism of action, as cytarabine. It has been shown to be a highly active agent for non-small cell lung cancer, pancreatic cancer, urothelial cancer, breast cancer and ovarian cancer. Gemcitabine is relatively well tolerated and myelosuppression is the dose-limiting toxicity. Pulmonary toxicity with gemcitabine is relatively uncommon, but a well recognized entity, associated with significant morbidity and mortality. A high index of suspicion, early diagnosis and timely intervention with oxygen supplementation, steroids, and diuretics is necessary to manage patients with this complication.     

Chemotherapy induced reversible posterior leukoencephalopathy syndrome

The reversible posterior leukoencephalopathy syndrome (RPLES) is a condition characterised by reversible neurological and radiological findings that has been associated with use of immunosuppressive, chemotherapeutic and more recently novel targeted therapies. We describe the case of a 50-year-old woman with advanced non-small cell lung cancer who developed status epilepticus shortly after receiving cisplatin and gemcitabine chemotherapy. The clinical, radiological and EEG findings during and post event are presented and are in keeping with a diagnosis of RPLES. Early recognition of this rare syndrome, supportive management and withdrawal of the offending agent appear to result in a reversal of the manifestations described.     

Reversible posterior leukoencephalopathy syndrome and anti-neoplastic agents: a review

Reversible Posterior Leukoencephalopathy Syndrome (RPLS) is a well recognized entity with a variety of benign and malignant conditions. Recently it has been found to be associated with the use of anti-neoplastic agents including targeted therapies. RPLS occurs rapidly with the use of some drugs and more slowly with others. Combined therapies are associated with a more frequent and more rapid presentation. This review was based on a literature search for English Language articles concerning RPLS and chemotherapeutic agents published from June 1996 to March 2007. We used the PubMed database with keywords: “RPLS”, “Posterior reversible encephalopathy syndrome”, “(PRES)”, “Chemotherapy” and “MRI”. This syndrome has classical Clinical-Radiologic features that are easy to recognize. Early recognition and withdrawal of the offending agent is all that is needed in most cases. This review highlights the features of the syndrome. It draws our attention to an entity which is being more frequently recognized and whose exact pathologic mechanisms need to be further studied. This syndrome is associated with the use of neurotoxic as well as non-neurotoxic agents and usually runs a benign course if there is an early diagnosis and management.     

Posterior reversible encephalopathy syndrome following chemotherapy with oxaliplatin and a fluoropyrimidine: a case report and literature review

Posterior reversible encephalopathy syndrome (PRES) is a neuro-radiological syndrome characterized by seizures, altered level of consciousness and visual disturbance. PRES is associated with hyperintense lesions on magnetic resonance imaging (MRI) most commonly seen in the posterior regions. In most cases symptoms and radiological lesions are reversible. The aims of this article are: (i) to review the literature for all cases involving oxaliplatin, fluoropyrimidine and bevacizumab and (ii) highlight the increasing number of cases attributed to anti-neoplastic agents. An in-depth literature review was conducted by utilizing Pubmed's MEDLINE and Google Scholar databases. We found that there have been nine cases of PRES associated with oxaliplatin or fluoropyrimidine therapy; five cases also involved therapy with bevacizumab. Eight of the nine patients made a full recovery with a complete resolution of MRI changes. This is the first Australian case of PRES following treatment with oxaliplatin and a fluoropyrimidine and only the second case reported in which the patient did not recover despite appropriate medical management. It appears that PRES maybe more commonly associated with multi-agent therapies and although reversible in most cases, PRES may result in adverse outcomes despite rapid intervention.     

急性白血病大剂量阿糖胞苷化疗后继发不典型可逆性后部脑病综合征一例并文献复习

目的:探讨急性白血病行大剂量阿糖胞苷化疗后继发不典型可逆性后部脑病综合征（PRES）的临床表现、影像学特点、治疗及预后,提高对不典型PRES的认识。方法:回顾性分析山东省滨州市人民医院收治的1例白血病化疗后继发不典型PRES 15岁男性患者的诊疗情况,并复习相关文献。结果:患者急性白血病复发,行大剂量阿糖胞苷化疗后出现头痛、视觉障碍及意识障碍等,行颅脑磁共振成像（MRI）检查,诊断为不典型PRES。经积极改善循环、成分输血、镇静、营养神经等治疗,临床症状完全缓解,复查颅脑MRI提示颅内病灶明显吸收好转。随诊观察1个月,临床症状无反复。结论:早期识别急性白血病行大剂量阿糖胞苷化疗后继发不典型PRES时,临床表现、颅脑MRI影像学特点及基础疾病是诊断的关键,积极治疗可明显改善预后。     

Glucagonoma syndrome: case report and literature review

The glucagonoma syndrome is characterized by dermatitis, glucose intolerance, hypoaminoacidemia, and hyperglucagonemia secondary to an alpha-cell tumor of the pancreas. Other clinical features include anemia, glossitis, and weight loss. A 62-year-old woman with the syndrome sought medical attention for a chronic dermatitis. A skin biopsy was suggestive of necrolytic migratory erythema. A glucagonoma was surgically removed from the tail of the pancreas. Review of the literature indicates that 56 proven and 33 probable cases of glucagonoma syndrome have been reported.     

Gemcitabine-induced peripheral edema: report on 15 cases and review of the literature

Some degree of peripheral edema occurs in up to 20% of patients treated with gemcitabine (Gemzar). The edema is typically mild, requiring discontinuation of the drug in less than 1% of patients. Most patients require no therapy. However, in cases of peripheral edema, grade II or higher, suspension of gemcitabine and treatment with corticosteroids are often necessary, and permanent discontinuation of gemcitabine may be required on occasion. We have identified 15 cases of peripheral edema, grade II or greater, developing in patients receiving gemcitabine chemotherapy at Memorial Sloan-Kettering Cancer Center. The diagnosis was made based on temporal association with drug administration, and exclusion of other potential acute causes of edema including progression of disease and deep vein thrombosis. The 15 patients in this series represent less than 1% of all patients treated with gemcitabine at this institution over the same time period. Gemcitabine was immediately discontinued at the time of the onset of edema in 7 of the 15 patients due to severity of the symptoms. Thirteen of the 15 patients in this case series had experienced peripheral edema previously, and 6 had active low-grade edema at the time the gemcitabine was first administered. Patients receiving gemcitabine should be advised of this potential complication and urged to promptly report its development so that comorbid conditions can be excluded and proper supportive measures initiated. Patients predisposed to peripheral edema from some other cause may be at increased risk for developing severe peripheral edema with gemcitabine.     

Richter综合征一例报告并文献复习

为了探讨Richter综合征的临床病理特点、诊断、治疗与预后,报道1例经典型Richter综合征,通过临床表现、骨髓细胞学、淋巴结病理及免疫组化研究该病的特点,并复习有关文献.结果患者外周血及骨髓见大量成熟小淋巴细胞,细胞形态学符合慢性淋巴细胞白血病骨髓像,骨髓细胞免疫表型为CD5(+)、CD10(-)、CD19(+)...     

A case of reversible posterior leukoencephalopathy syndrome(RPLS)induced by modified FOLFOX6

We report here a case of reversible posterior leukoencephalopathy syndrome(RPLS)induced by modified FOLFOX6(mFOLFOX6). The patient was a 43-year-old woman who had sigmoid colon cancer with multiple liver metastases. Treatment with mFOLFOX6 was started. Early in the morning of day 11, the patient was transported by ambulance to the hospital due to nausea with headache, disturbed consciousness, and visual disturbance. The patient experienced sudden, severe nausea and subsequently presented generalized tonic-chronic seizures. The seizures subsided after treatment. On the evening of day 11, another episode of generalized tonic-chronic seizures occurred. Status epilepticus developed and tracheal intubation was performed for airway protection. Cranial MRI showed increased signal intensity in both occipital lobes, centered on the boundary between the gray and white matter on FLAIR images. Her condition stabilized with no seizure recurrence following intubation. Although hypertension was present on admission to the emergency room, blood pressure gradually fell to within the normal range without antihypertensive treatment. She was extubated on day 18. There were no neurologic sequelae. Cranial MRI on day 40 showed that the increased intensity in both occipital lobes had almost disappeared. Because the patient's condition was characterized by a reversible central nervous system disorder, RPLS was diagnosed.     

Crouzon syndrome: A case report and review of literature

Crouzon syndrome, also called craniofacial dysostosis is an autosomal dominant disorder characterized by premature closure of cranial sutures, midfacial hypoplasia and orbital defects. Herein we report a case of this rare entity who presented with brachycephaly, maxillary hypoplasia, wide parrot beaked nose, repaired bilateral cleft lip and cleft palate along with dental and orbital abnormalities.     

Mitomycin-C induced hemolytic uremic syndrome: a case report and literature review

Hemolytic uremic syndrome spontaneously arises in a few patients with advanced cancer, but it is more commonly related to the use of certain chemotherapeutic agents. Mitomycin-C is, etiologically, the most common causative agent inducing hemolytic uremic syndrome, in a dose dependent manner. We report this syndrome, attributable to mitomycin-C at a cumulative dose of 40 mg/m2, in a gastric cancer patient. A 42-year-old female with stage III gastric cancer underwent radical gastrectomy and was given mitomycin-C at 10 mg/m2 intravenously every four weeks as adjuvant therapy. Hemolytic uremic syndrome was diagnosed three months after the last dose of mitomycin-C administration. The most prominent symptoms included pallor, hypertension and anasarca, with laboratory evidence of microangiopathic hemolytic anemia, azotemia and hyperkalemia. Her disease was progressive, but fortunately stabilized after staphylococcus column A dialysis. Her disease remained in remission for 24 months from the time of diagnosis, and then relapsed in the form of peritoneal carcinomatosis with partial intestinal obstruction.      

Non-Hodgkin's lymphoma in Job's syndrome: a case report and literature review

Job's or hyper immunoglobulin E recurrent infection syndrome (Hyper-IgE syndrome) is a rare, often inherited multisystem disorder, characterized by cutaneous abscesses, pneumonia, elevated IgE levels and skeletal defects. We report a case of a 22-year-old man with Job's syndrome who presented with back pain. He was found to have diffuse large B-cell lymphoma involving his second lumbar vertebrae and spleen. Treatment with dose-adjusted EPOCH-rituximab (DA-EPOCH-R) chemotherapy achieved a complete remission after 4 cycles. A review of reported cases of lymphoma in Job's syndrome indicates an increase in relative risk of 259 (95% confidence interval 102, 416). The cause of the increased risk has yet to be defined but has similarities to a pathogenetic model of AIDS related lymphoma. In previous reports of lymphoma in Job's syndrome, patients presented with extranodal disease and had poor outcomes. With appropriate chemotherapy and hematological support, lymphoma associated with Job's syndrome can achieve complete remission.     

Gemcitabine and cisplatin chemotherapy induced reversible posterior leukoencephalopathy syndrome in a bladder cancer patient

Reversible posterior leukoencephalopathy syndrome (RPLS) is a rare clinical entity, the common clinical symptoms of which are headache, disturbance of consciousness, altered mental status, seizures, and visual disturbance. Recently, some cases have been reported in association with the increased use of cytotoxic and immunosuppressive agents in cancer patients, and relevant reports have increased with advances in radiological examinations. We describe here the case of a 50-year-old man with advanced bladder cancer who suddenly experienced diminished spontaneity and speech, and finally became semicomatose. Two months previously, he had received gemcitabine and cisplatin chemotherapy. Computed tomography and magnetic resonance imaging revealed symmetrical edema of the posterior occipital lobe and thalamus. Based on these findings, we made a diagnosis of RPLS and treated him with supportive measures. His mental status gradually improved in 2 weeks, although slight neurological symptoms persisted. When the level of consciousness of a cancer patient worsens rapidly, this syndrome should be included in the differential diagnosis and recognized at an early stage. Early supportive management and discontinuation of the causative medication may reverse the clinical and radiological manifestations of the syndrome.     

Albrights syndrome—report of a case and review of literature

Albright’s Syndrome is a rare disease which occurs sporadically. Its aetiology is not known. It is extremely rare in males. To the best of our knowledge this Syndrome is not associated with any congenital visceral anomaly. We present a case of Albright’s Syndrome in a boy who also showed a congenital ectopic kidney. The literature of this syndrome is reviewed with a view to update the current knowledge.     

Maffucci综合征1 例报道并文献复习

目的:探讨Maffucci综合征的生物学行为、临床病理特点、诊断治疗方法及其预后。方法:对1例Maffucci综合征的临床资料、病理学形态、影像学资料进行回顾性分析,并结合国内外文献进行分析。结果:患者为25岁男性,因“发现右足及双手多发肿物17年”就诊。5岁时曾因右侧股骨远端内生软骨瘤行矫形手术治疗。专科检查示:右下肢短缩畸形,跛行,右膝、踝关节周围局部隆起,无压痛,关节活动功能受限,双手部及足部均散在分布数十个蓝紫色肿物,大小不等,压之不褪色,X线检查提示:双手部、右足部可见多发软组织肿块影,右股骨、胫腓骨骨质结构紊乱,髓腔内骨小梁正常结构消失,骨干弯曲变形,局部可见膨大,右膝关节面欠光整,关节间隙不等宽。右踝关节前方可见一团块状高密度影,边缘钙化。根据患者要求行双手部肿物切除术,术后病理报告为:（双手部血管瘤）符合血管瘤伴血栓形成,纤维化及钙化。随访半年未见复发及新发肿物。结论:Maffucci综合征是一种以多发内生软骨瘤并发软组织血管瘤为特点的非遗传性疾病。该病是否由于基因缺陷引起尚未明确,常常在早期发生恶变,以软骨肉瘤恶变为主。Maffucci综合征也常伴发其它恶性肿瘤。影像学及病理组织学检查在诊断Maffucci综合征及其恶变中起关键作用。该病治疗目的为缓解疼痛和早期发现恶变,手术切除为主要治疗手段。     

Gemcitabine-induced radiation recall dermatitis: case report

A 65-year-old male with lung adenocarcinoma received radiotherapy to the mediastinum and right upper lobe, followed by chemotherapy with gemcitabine. Radiation recall dermatitis developed in the area corresponding to the radiotherapy portal. This is one of just a few cases reported recently concerning radiation recall dermatitis stemming from gemcitabine.