Expression of shared idiotypes in chronic lymphocytic leukemia and small lymphocytic lymphoma

The expression of shared idiotypes (Slds) has been studied on malignant CD5+B cells from patients with chronic lymphocytic leukemia (CLL) and small lymphocytic lymphoma (SLL) using a panel of 37 murine monoclonal antibodies previously demonstrated to be reactive with Slds derived from follicular B-cell lymphomas. Thirteen anti-Slds identified tumor cells from 31 of 105 (30%) CLL patients and 4 of 6 SLL patients. In comparison, the same panel of anti-Slds is reactive with 33% of follicular and diffuse B-cell lymphomas. Ten of these anti-Slds reacted with CLL cases at similar frequencies to that of the B-cell lymphomas. Two anti-Slds, which are known to react with autoantibodies, were significantly more prevalent in CLL than in B-cell lymphomas. These data confirm the presence of Slds in CLL and provide further evidence of an association between CLL and autoimmunity. The identification of a panel of antibodies reactive with a significant number of CLL and SLL patients will facilitate the application of anti-idiotype antibody therapy in these diseases.     

Chronic lymphocytic leukemia in China

Chronic lymphocytic leukemia (CLL) is a very common form of leukemia among middle-aged and elderly persons in Western countries. In the Japanese, or in those with Japanese ancestry born and living in the USA, CLL is rather rare. It is also rare in China, but convincing data to support this statement are found primarily in the Chinese literature. We have reviewed previously unpublished data from Peking Union Medical College (PUMC) concerning the frequency of CLL among adult patients with the four common types of leukemia. Of 4,174 such patients (1952-1986), 4.6% had CLL; 22.9% chronic myeloid leukemia (CML); 50.3% acute myeloid leukemia (AML); and 22.3% acute lymphoblastic leukemia (ALL). This low frequency of CLL is none the less higher than that found in a multiinstitutional collection of Chinese patients with leukemia. Various clinical and laboratory aspects of a carefully studied, previously published group of patients with CLL from PUMC were compared to similar published findings in patients from the University of Utah. No differences in the overall nature of the disease were detected between these groups. However, not unexpectedly, the duration of symptoms before the diagnosis was made longer in Chinese than in USA patients and the proportion diagnosed at a time when no CLL related symptoms had been noted was less in Chinese. Neither differences in life span in the two populations nor an excess of CML, AML, and ALL at the expense of CLL were viable hypotheses to explain the rarity of CLL in China. We suggest that the dearth of CLL in Chinese is on a genetic basis, as it is thought to be in the Japanese.     

The prognostic significance of cytopenia in chronic lymphocytic leukaemia/small lymphocytic lymphoma

The development of cytopenia in chronic lymphocytic leukaemia (CLL) patients can predict poor prognosis. All CLL patients seen in the Division of Hematology at Mayo Clinic Rochester from 1 January 1995 to 31 December 2004 (n = 1750) were evaluated for cytopenia, aetiology of cytopenia and clinical outcome. Cytopenia occurred in 423 (24.2%) patients and was attributable to CLL in 303 (17.3%) cases, with 228 (75%) of these having bone marrow (BM) failure and 75 (25%) having autoimmune disease (AID). Survival from onset of cytopenia was significantly better for patients with AID (median 9.1 years) compared to patients with BM failure (median 4.4 years, P < 0.001). Patients with AID diagnosed within 1 year of the diagnosis of CLL (n = 35) had similar survival from diagnosis compared to patients without CLL-related cytopenia (median 9.3 vs. 9.7 years, P = 0.881). Although cytopenia caused by BM failure predicted a poorer prognosis in CLL, cytopenia caused by AID was not an adverse prognostic factor. These findings suggest that patients with cytopenia due to AID cannot be meaningfully classified by the current clinical staging systems. Revisions of the National Cancer Institute Working Group 96 criteria should consider the aetiology of cytopenia in staging CLL patients.     

Graft-versus-leukemia in chronic lymphocytic leukemia

Immune-mediated anti-leukemia effects, often termed graft-versus-leukemia (GvL), operate after bone marrow or blood cell transplants for acute lymphoblastic leukemia, acute myelogenous leukemia and chronic myelogenous leukemia. Sometimes the magnitude of this anti-leukemia effect exceeds that of high-dose anti-leukemia drugs and radiation and can result in leukemia cure. We analyzed leukemia relapse data after transplants for chronic lymphocytic leukemia (CLL) in this context. These data support the notion of a strong GvL effect in CLL. However, as most of these data are from studies of allotransplants, it is uncertain whether GvL operates in settings where the anti-leukemia effector cells and target CLL cells are genetically identical except for leukemia-related mutations. It is also uncertain whether GvL is distinct from GvHD. These potential limitations have important implications on whether immune therapy of CLL will work in non-allotransplant settings.     

Chronic lymphocytic leukemia in Japan

Forty-one patients diagnosed as having B-cell chronic lymphocytic leukemia in Japan were evaluated. Patients were classified into five groups (CLL, CLL-L, CLL/PL, PLL, LSCL) according to differential counts of small lymphocytes, large lymphocytes, prolymphocytes, immunoblasts and/or lymphosarcoma cells among the lymphoid cells in the first available peripheral smears based on Melo's morphological criteria. Patients with typical CLL, defined as having more than 90% small lymphocytes, were seven (17%) of 41 patients. Phenotypical characteristic of CLL and its subgroups in Japan was considered to be the high expression of activated antigens, such as CD22, PCA1 and CD25. This finding may support the relatively higher proportion of atypical CLL with high percentage of large lymphocytes or prolymphocytes in Japan than that in Western countries. The molecular biological analysis revealed the deletion of C mu gene in one allele in three sIg-negative cases. The incidence of CLL, CLL-L and CLL/PL in Japan was deduced to be 1/10 of that in Western countries.     

Endobronchial lymphocytic infiltration: Unusual manifestation of chronic lymphocytic leukemia

This report describes a patient with chronic lymphocytic leukemia followed over a 15-year period. The distinctive feature of her course was the infiltration of the bronchial wall by malignant lymphocytes, which produced endobronchial obstruction, atelectasis, and infection. Symptomatic improvement occurred following local irradiation and steroid therapy. A review of the literature establishes that the development of endobronchlal infiltration during the course of chronic lymphocytic leukemia and other lymphoproliferatlve disorders is distinctly uncommon.     

Histologic transformation of chronic lymphocytic leukemia/small lymphocytic lymphoma

Although generally considered a clinically indolent neoplasm, CLL/SLL may undergo transformation to a clinically aggressive lymphoma. The most common form of transformation, to DLBCL, is also known as Richter syndrome. Transformation determines the course of the disease and is associated with unfavorable patient outcome. Precise detection of transformation and identification of predictive biomarkers and specific molecular pathways implicated in the pathobiology of transformation in CLL/SLL will enable personalized therapeutic approach and provide potential avenues for improving the clinical outcome of patients. In this review, we present an overview of the pathologic features, risk factors, and pathogenic mechanisms of CLL/SLL transformation. Am. J. Hematol. 91:1036–1043, 2016. © 2016 Wiley Periodicals, Inc.     

慢性淋巴细胞性甲状腺炎

慢件淋巴细胞件甲状腺炎于1912年由日本学者首先报道,属于最常见的自身免疫性甲状腺疾病,也是甲状腺功能减退(甲减)最主要的病因,好发于中老年女性.患者起病隐匿,常无特征性的临床表现,容易漏诊.本病诊断尚缺乏国际统一标准,主要依靠一些非特异性的症状与体征,尤其是甲状腺肿大、甲状腺功能减退,并结合相应的辅助检查.如甲状腺自身抗体阳性,且滴度较高,对诊断意义更大.甲状腺穿刺细胞学检查不作为常规项目,但对诊断困难者具有确诊价值.伴有甲减者,需要常规采用替代治疗,尤其是甲状腺肿大明显者、孕妇以及儿童可以给予左旋甲状腺素替代治疗,但仅有亚临床甲减者,或甲状腺功能正常而甲状腺自身抗体阳性的患者是否需要治疗尚存在争议.     

Chronic lymphocytic leukaemia

Chronic lymphocytic leukaemia is the commonest form of leukaemia in Europe and North America, and mainly, though not exclusively, affects older individuals. It has a very variable course, with survival ranging from months to decades. Major progress has been made in identification of molecular and cellular markers that could predict disease progression in patients with chronic lymphocytic leukaemia. In particular, the mutational profile of immunoglobulin genes and some cytogenetic abnormalities are important predictors of prognosis. However, these advances have raised new questions about the biology, prognosis, and management of chronic lymphocytic leukaemia, some of which are addressed here. In particular, we discuss how better understanding of the function of the B-cell receptor, the nature of genetic lesions, and the balance between proliferation and apoptosis have affected our ability to assess prognosis and to manage chronic lymphocytic leukaemia. Available treatments generally induce remission, although nearly all patients relapse, and chronic lymphocytic leukaemia remains an incurable disease. Advances in molecular biology have enhanced our understanding of the pathophysiology of the disease and, together with development of new therapeutic agents, have made management of chronic lymphocytic leukaemia more rational and more effective than previously. Unfortunately, we know of no way that chronic lymphocytic leukaemia can be prevented. Early detection is practised widely, but seemingly makes no difference to the patient's eventual outcome.     

Infectious complications in chronic lymphocytic leukaemia

Infections are the major cause of morbidity and mortality in patients with chronic lymphocytic leukemia (CLL). Predisposition to infection in CLL is mediated through various abnormalities including both the immune defects inherent in the primary disease (impairment in humoral and cellular immunity) and in the further immunosuppression related to the management of CLL. Hypogammaglobulinemia is probably the most important immune defect in terms of risk of severe bacterial infections, its frequency and severity progressing with the duration of the disease. Newer antineoplastic agents such as purine analogues, especially when used in previously treated patients, may be associated with a new spectrum of pathogens (Listeria monocytogenes, Pneumocystis carinii, cytomegalovirus, herpes simplex virus, and mycobacteria) involving T-cell dysfunction. In this review we focus on the clinical characteristics of infections in CLL and on the risk factors involved in the pathogenesis of this complication. Furthermore, we describe the evolving patterns of infections associated with purine analogues and discuss the currently accepted approaches to prophylaxis and treatment.Les complications infectieuses de la leucémie lymphoïde chronique     

Chronic lymphocytic leukemia

Chronic lymphocytic leukemia (CLL) is the most common adult leukemia in the western world and is due to the accumulation of mature B lymphocytes in the peripheral blood, bone marrow and secondary lymphoid organs. The leukemic cells show a distinct phenotype, which is essential to reach the correct diagnosis. Despite the phenotypic homogeneity, the clinical outcome may be significantly different. Some patients have an indolent leukemia, with long survival while others experience an aggressive disease, with early and frequent need of treatment. At present, no chemotherapeutic regimens can be considered curative and all patients will die with (or because of) their disease. In recent years, research on CLL has led to important discoveries that help defining patients' prognosis at the moment of diagnosis. These prognostic factors, which are derived from the biological features of the leukemic lymphocytes, are now rapidly moved into the clinical arena. They are used to stratify patients in selected clinical trials to assess the value of early and more modern treatments, which are becoming available to hematologists.     

Collagenous and lymphocytic colitis in Iceland

The aim of this study was to determine the nationwide incidence of collagenous and lymphocytic colitis in Iceland and the location of histopathological changes in the large bowel. All pathology reports of patients diagnosed with or suspected of having collagenous colitis or lymphocytic colitis in the period 1995–1999 were identified. All pathology samples were reevaluated using strict diagnostic criteria. After reevaluation 125 patients fulfilled our diagnostic criteria, 71 as collagenous colitis and 54 as lymphocytic colitis. The mean annual incidence for collagenous colitis was 5.2/100,000 inhabitants, and the mean age at diagnosis was 66.1 years. The mean annual incidence for lymphocytic colitis was 4.0/100,000 inhabitants, the mean age at diagnosis was 68.7 years. Both diseases more commonly involved the colon than the rectum. The incidence of collagenous colitis and lymphocytic colitis is high in Iceland. The mean annual incidence of collagenous colitis is much higher in Iceland than hitherto reported elsewhere.