Lymphomatoid granulomatosis-like lesions in a child with leukemia in remission

This case report describes an eight year old leukemic child who was in remission for four and one-half years and showed hypogammaglobulinemia and deficient leukocyte chemotaxis and migration. She developed pulmonary lesions with laryngeal and tracheobronchial disease and died from pulmonary hemorrhage. The lesions found at autopsy were lymphomatoid granulomatosis-like, but were atypical in their anatomic distribution, behavior, and histologic features.     

Lymphomatoid granulomatosis and malignant lymphoma of the central nervous system in the acquired immunodeficiency syndrome

While primary and secondary malignant lymphomas have been well-documented in the CNS of patients with the acquired immunodeficiency syndrome (AIDS), only one case of lymphomatoid granulomatosis (LG) involving the CNS has been reported. We present three AIDS patients with multiple grossly evident foci of necrosis in the cerebral hemispheres which, on histologic evaluation, were seen to contain angiocentric mixed chronic inflammatory infiltrates with atypical mononuclear cells, luminal thrombosis, and infarction, which is typical of LG. LG was also identified in sections of the lung in one case. Lymphoma was found in other regions of the brain in two cases, suggesting the evolution of LG into cerebral lymphoma. In addition, widespread perivascular multinucleate syncytial giant cells, associated with human immunodeficiency virus (HIV) infection of the CNS, were identified in all patients. The features of LG, its relationship to lymphoma, and the possible etiologic role of an immunodeficiency state or the HIV virus in the pathogenesis of LG are discussed.     

Lymphomatoid granulomatosis: evidence that some cases represent Epstein-Barr virus-associated B-cell lymphoma

Lymphomatoid granulomatosis is currently classified as part of a spectrum of angiocentric immunoproliferative lesions. These were initially thought to be of T-cell phenotype, but recent papers have shown that some cases are B-cell proliferations, sometimes associated with Epstein-Barr virus infection. We reviewed the clinicopathological features of 16 patients with pulmonary lymphomatoid granulomatosis, using immunohistochemistry to assess the phenotype of the infiltrate, the polymerase chain reaction to look for immunoglobulin heavy chain and T-cell receptor gene rearrangements, and in-situ-hybridization to look for Epstein-Barr virus infection. In seven of seven cases the atypical lymphoid population was of B-cell phenotype, with four cases showing evidence of either monoclonality or oligoclonality. All seven cases, including those that lacked unequivocal proof of malignancy, behaved aggressively. Epstein-Barr virus RNA was detected in four cases. We conclude that some cases of lymphomatoid granulomatosis are B-cell lymphomas, sometimes associated with Epstein-Barr virus infection.     

Cytogenetics on malignant lymphoma

Translocations involving band q32 on chromosome 14 are commonly identified as a 14q + marker-chromosome and the involvement is the single most frequent abnormality in various types of lymphoid cancer. Based on these findings, we have proposed that the 14q + marker-positive lymphoid cancer should be divided into subclasses according to the precise translocations, each of which is a primary chromosome change, and the subclass initiates serial processing of the karyotype evolution associated with the development of more aggressive lymphoid cancer. The proposal is strongly supported by recent advances in molecular genetics. Genes or unknown DNA sequences, which had evolutionally been conserved, were isolated from the breakpoint on the partner chromosome of the 14q+ translocation, and the DNA sequences identified to juxtapose to the IgH gene locus on chromosome 14 band q32 accompanied by individual translocation. Thus, we can subclassify the lymphoid cancers marked with chromosome changes involving the loci of other functional genes.     

Cytology of malignant lymphoma

Malignant lymphoma can occur in various organs, including the mediastinum, spleen, Peyer's patch, skin, gastrointestinal tract and lungs, in addition to the lymph nodes; therefore, with a sample such organs, differential diagnosis with malignant lymphoma should be kept in mind. Furthermore, the treatment of choice for malignant lymphoma depends on the histological subtype, because of the broad spectrum of tumors, i.e., low-grade to high-grade malignancy. Accordingly, appropriate methods to obtain samples and their adequate preparation are quite important to make an accurate diagnosis. Cytology has an advantage over histological diagnosis in terms of a less invasive procedure, low cost and short turnaround time. In this review, the important points to make an accurate cytologic diagnosis of malignant lymphoma will be discussed.     

Rosette formation in malignant lymphoma.

Lymph node biopsies in a patient with follicular lymphoma showed rosette structures as seen in neuroepithelial neoplasms. These specimens were studied by histologic, immunoperoxidase (for immunoglobulins, intermediate filaments (IF), actin and neuron-specific enolase), immunofluorescence (for immunoglobulins, and with a panel of monoclonal antibodies), and electron-microscopic examination. The rosettes were formed by neoplastic lymphocytes arranged around eosinophilic fibrillary material. Ultrastructurally, this was composed of cytoplasmic processes, projecting from the lymphocytes and containing thin and intermediate filaments. Immunohistochemically, it stained for monoclonal IgM lambda, all other antigens present on the neoplastic cells, and weakly for vimentin and actin. Based on recent information about lymphocyte surface changes, it is speculated that the rosettes might represent an aggregation of neoplastic lymphocytes activated by a microenvironmental stimulus, perhaps antigen-antibody binding at the cell membrane. The practical implication of this hitherto unreported finding is that the presence of rosettes cannot be used to rule out a lymphoma.     

Lymphomatoid granulomatosis

Lympliomatoid granulomatosis, defined on the basis of study of the first 40 patients, is an angiocentric and angiodestructive lymphoreticular proliferative and granulomatous disease involving predominantly the lungs. The infiltrate is polymorphous Nrith varying numbers of cells in mitosis and may contAm atypical, especially plasmacytoid, cells of reticuloendothelial origin, but, remarkably, usually tends to spare lymph nodes, spleen, and bone marrow. Rarely, however, generalized atypical lymphoid hyperplasia may precede the onset of pulmonary lesions, sometimes by many years. The question usually raised is whether the process represents lymphoma or Wegener's granulomatosis.     

淋巴瘤样肉芽肿型大B细胞淋巴瘤

目的 探讨淋巴瘤样肉芽肿型大B细胞淋巴瘤(原名淋巴瘤样肉芽肿病,lymphomatoid granulomatosis,LYG)的病理形态特征、病变性质及鉴别诊断要点。方法 对l例LYG的组织形态学、免疫组织化学、EBV原位分子杂交(EBER)结合临床特征进行了分析。结果 l例63岁男性患者,临床上表现为多系统多器官性病变,主要累及肺,表现为双肺内境界清楚的圆形结节,呈孤立性或弥漫性分布,并出现多发性皮下结节,发热、体重减轻、全身无力等症状。皮下结节活检示多个肉芽肿样结构,细胞形态多样,见组织样细胞、非典型淋巴样细胞、小淋巴细胞、浆细胞及散在多核巨细胞,可见一血管壁有淋巴样细胞浸润,未见明显中心粒细胞,可见核分裂象。肺部穿刺组织示弥漫淋巴样细胞浸润,并见灶性坏死,免疫表型示瘤细胞呈CD20 ,CD79α ,CD43 ,CD3-,GraB-,EBV散在 ,CK-,Syn-,原位杂交示EBER 。结论 本例LYG是一种罕见的淋巴瘤样肉芽肿型大B细胞性淋巴瘤,与EBV相关。临床与影像学上与Wegener肉芽肿相似。肺部出现结节状病灶时,临床上易与结核、肉芽肿病、肺癌及炎性假瘤等相混淆,病理上须与结核、非特异性肉芽肿病、结外的外周T细胞淋巴瘤等相鉴别,形态学、免疫表型结合临床特征可明确诊断。     

恶性淋巴瘤的组织形态分析

众所周知,恶性淋巴瘤(以下称淋巴瘤)由一组不同类型的独特疾病构成,而这些疾病的定义和识别,又建立在对其形态、免疫表型、遗传学以及临床特点综合认识的基础之上[1-2].近年来,随着科技进步,淋巴瘤的诊断和研究工作也已逐渐深入到蛋白、基因层面的分子水平.但在临床实践中,绝大部分淋巴瘤病例仍然必须通过组织病理学检查而明确诊断.这当中,通过常规HE染色切片对肿瘤的组织形态进行分析尤为重要.     

Klinefelter's syndrome and malignant lymphoma

The high risk for malignancy for carriers of several congenital chromosomal abnormalities is discussed. We report herein a patient with Klinefelter's syndrome associated with a high grade malignant non-Hodgkin lymphoma. Chromosome analysis of PHA-stimulated lymphocytes showed a mosaic of 46,XXY (90%) and normal 46,XY (10%) metaphases. To our knowledge, this is the third reported case of a malignant non-Hodgkin lymphoma in a patient with Klinefelter's syndrome in the literature.     

Germinal center derived malignant lymphoma in cystadenolymphoma

Summary Two cases of malignant non-Hodgkin's lymphoma arising in an yAlbrecht-Arzt-tumour are reported. In the first case a centroblastic-centrocytic lymphoma in a palatinal cystadenolymphoma of a 64-year-old female is described. In the other case a centroblastic lymphoma developed in an Albrecht-Arzt-tumor of the submandibular region in an 82-year-old man. The occurence of a high-grade malignant lymphoma in cystadenolymphoma has not been reported in the literature so far.     

Antipyretic effect of indomethacin in malignant lymphoma

Fifteen patients with lymphoid malignancies and tumour-related fever (greater than 38.0 degrees C) were given 50 mg indomethacin (IM) orally. This resulted in a reduction of body temperature in all cases (mean +/- SD 3.4 +/- 2.0 degrees C). In one patient with untreated Hodgkin's disease, temperature fell from 40.6 degrees C to 30.6 degrees C within 12 hours without any cardiovascular or respiratory distress. The lytic effect of IM on fever was more pronounced and more rapid in the 15 patients with lymphoma than in a group of 10 patients with acute myocardial infarction. IM therapy has a clear value in relieving tumour-associated fever in patients with malignant lymphoma. There may be a qualitative difference between the IM response of tumour-related fever and fever related to non-malignant diseases.     

Kaposi's sarcoma and malignant lymphoma in AIDS

Summary A 48-year-old homosexual with contacts in different countries, including Haiti, presented with multiple pigmented or bluish nodules on both lower legs and upper arms. He had a history of secondary syphilis, hepatitis B and herpes zoster ophthalmicus. Biopsies of the skin tumors revealed a typical Kaposi's sarcoma of low grade malignancy. The endothelial origin of the tumor was indicated by the presence of specific endothelial organelles (Weibel-Palade bodies) in the cytoplasma of the tumor cells. Erythrocytophagocytosis was found in tumor cells within and without the vascular channels. Laboratory tests were compatible with the clinical diagnosis of an acquired immune deficiency syndrome (AIDS) with a helper: suppressor T-lymphocyte ratio of 0.28 and a cutaneous anergy. In the course of the illness tumors of the stomach and duodenum were detected. Histology showed a malignant non-Hodgkin lymphoma of high grade malignancy. Within weeks the patient died in a cachectic state. Autopsy revealed a Kaposi's sarcoma of the skin with metastases in the stomach and a wide-spread malignant lymphoma in the gastrointestinal tract, in several visceral organs and in many lymph nodes.