Molecular genetic demonstration of the diverse evolution of Richter's syndrome (chronic lymphocytic leukemia and subsequent large cell lymphoma)

Paired samples of chronic lymphocytic leukemia (CLL) or small lymphocytic lymphoma (SLL) and the subsequent diffuse large cell lymphoma (DLL) of six cases of Richter's syndrome were investigated to establish the clonal relationship between the CLL/SLL and the DLL components and to define the oncogene and/or tumor-suppressor gene alterations involved in the morphologic transformation of CLL/SLL. Southern blot hybridization analysis showed identical clonal immunoglobulin (Ig) gene-rearrangement patterns in the CLL/SLL and DLL components in four cases and different Ig gene-rearrangement patterns in two cases. Polymerase chain reaction (PCR) amplification, cloning, and DNA sequencing of complementary determinant region 3 (CDR3) of the Ig-heavy chain gene of one of the two cases in which the Ig gene- rearrangement patterns were different showed nonidentical sequences in the CLL/SLL and DLL components. In the other case, monomorphic Epstein- Barr virus (EBV) genome integration was detected in the DLL but not in the CLL, suggesting that the CLL and DLL components in this case of Richter's syndrome also represent unrelated clones. Single-strand conformation polymorphism (SSCP) analysis and sequencing of exons 5 through 9 of the p53 tumor-suppressor gene showed a mutation in codon 176 of the DLL but not in the CLL/SLL component in one case where the CLL/SLL and DLL represented different clones. The p53 mutation probably played a role in the development of the lymphoma rather than morphologic transformation of the CLL/SLL in this case. SSCP analysis and sequencing also showed identical mutations in codon 282 in both the CLL/SLL and DLL components in a case where the CLL and DLL represented identical clones. Thus, this p53 gene mutation was present both before and after morphologic transformation, and therefore, probably did not play a primary role in this process. Southern blot hybridization analysis failed to show evidence of bcl-1, bcl-2, c-myc proto-oncogene or retinoblastoma (Rb) tumor-suppressor gene rearrangements in these six cases of Richter's syndrome. In conclusion, the original CLL/SLL and the subsequent DLL in Richter's syndrome may or may not be derived from identical clones, and the well-known proto-oncogenes and tumor- suppressor genes do not appear to play an obvious and consistent role in the morphologic transformation of CLL/SLL to DLL.     

Percutaneous fluoroscopic removal of a knotted Swan–Ganz catheter in a patient with a persistent left‐sided superior vena cava

Knotting of intravascular catheters is an uncommon but a well‐recognized occurrence. The Swan–Ganz catheter (SGC) is the one that knots most commonly. A case of a knotted SGC is described in a patient with a persistent left‐sided superior vena cava, and we propose that the presence of a left‐sided superior vena cava is a risk factor for knot formation not previously reported. We review the published work on the risk factors for knot formation and on the techniques used to remove knotted SGC. We describe a technique using a gooseneck snare and Omni Flush catheter (Angiodynamics, Queensbury, NY, USA) to loosen and untie a knotted SGC.     

膦甲酸钠治疗疱疹病毒相关性进展性卒中26例

1临床资料我院神经内科200112/200203应用膦甲酸钠(PFA,foscarnetsodium)治疗进展性卒中(progressivestroke,PS)患者26例.男12例,女14例,年龄35～70(平均57±14)岁.总病程19～72d.有高血压病史19例,有短暂性脑缺血发作病史11例,有脑梗死病史3例,有冠心病病史1例.标准符合1995年第四届全国脑血管病会议脑梗死诊断标准[1],并经脑CT或MRI证实为脑梗死;发病后1wk内病情逐步或阶梯样加重,临床常规治疗不能阻止病情进展,按1995年第四届全国脑血管病会议神经功能缺损评分标准[2]评分增加2分(包括2分)以上.采用金标免疫斑点法检测患者血清中人巨…     

Idiopathic isolated optic nerve head swelling a presentation of two cases

Optic nerve head swelling is caused by either ocular or systemic diseases of inflammatory, infective, infiltrative, compressive, or ischemic origin. Uncommonly, an isolated optic nerve head swelling without the above underlying mechanisms might also occur. Two patients presented with unilateral, isolated optic nerve head swelling. A detailed oculoneurological examination and a series of investigations failed to indicate any pathology. No probable cause for optic nerve head swelling in these patients was determined by the authors. the authors have stated that they do not have a significant financial interest or other relationship with any product manufacturer or privider of services discussed in this article. The authors also do not discuss the use of off-label products, which include unlabeled, unapproved, or investigative products or devices.     

Comment on ‘Predicting recurrence in axillary node negative breast cancer patients’, by Rosner and Lane

on behalf of the Nottingham Breast Unit