Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019829 (Hodgkin's disease)
 30,247 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 56-year-old man with chronic lymphocytic leukemia who developed Richter's syndrome is described. The criteria for diagnosis are given and the histology is discussed. We agree that Richter's syndrome represents a peculiar complication of chronic lymphocytic leukemia and not a separate disease entity. As such, Richter's syndrome must be known and recognized by those evaluating patients with lymphadenopathy whose basic diagnosis may be confused with Hodgkin's disease or "histiocytic" lymphoma.
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PMID:Richter's syndrome. 33 29

Long, John C., AND Aisenberg, Alan C.: Richter's syndrome. A terminal complication of chronic lymphocytic leukemia with distinct clinicopathologic features. Am J Clin Pathol 63: 786-795, 1975. Two patients with chronic lymphocytic leukemia of 8 and 9 years duration had a terminal illness characterized by weight loss, persistent fever, lymphadenopathy, and dysglobulinemia. In both cases autopsy revealed a pleomorphic histiocytic lymphoma which contained multinucleate tumor cells and was associated with persistent chronic lymphocytic leukemia. These two cases are examples of Richter's syndrome, a clinically distinct complication of chronic lymphocytic leukemia that may be confused pathologically with Hodgkin's disease. Review of the medical literature suggests that this syndrome has frequently gone unrecognized. (Key words: Richter's syndrome; Chronic lymphocytic leukemia.)
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PMID:Richter's syndrome. A terminal complication of chronic lymphocytic leukemia with distinct clinicopathologic features. 109 89

The association of chronic lymphocytic leukemia (CLL) and Hodgkin's disease has been controversial. Pleomorphic lymphoreticular cells resembling Reed-Sternberg cells have been observed in Richter's syndrome. Although most observers have favored the view that these cells are a component of a pleomorphic non-Hodgkin's lymphoma, some cases of histologically typical Hodgkin's disease have been described. We have studied two cases that appear to represent composite CLL and Hodgkin's disease, providing evidence for an interrelationship of these two disorders. Classic Reed-Sternberg cells and variants (RS-H) were seen in a background that was otherwise typical of CLL. Both patients initially presented with characteristic findings of CLL in the peripheral blood and bone marrow. The first patient was found to have RS-H cells within lymph nodes at initial presentation, and ultimately progressed to develop a disseminated lymphoma characteristic of Hodgkin's disease. In the second patient, RS-H cells were not discovered until 5 years later. Immunophenotypic studies confirmed these morphologic impressions. The predominant lymphocyte population had a phenotype consistent with B-cell CLL. By contrast, the RS-H cells were strongly positive for CD15 (Leu M1) with staining of the Golgi region and cell membrane. Additionally, the RS-H cells were surrounded by rosettes of lymphocytes that marked as T cells. In both of the patients, a small percentage of RS-H cells expressed positivity for the B-cell marker L-26, which may indicate an origin from the underlying CLL. These findings support a B-cell origin for the malignant cell in some cases of Hodgkin's disease and suggest that Hodgkin's disease in some patients may be related to or derived from a coexisting lymphoid malignancy.
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PMID:Chronic lymphocytic leukemia with coexistent Hodgkin's disease. Implications for the origin of the Reed-Sternberg cell. 167 Jul 53

Large cell lymphoma associated with chronic lymphocytic leukemia (CLL)--Richter's syndrome--is a well-recognized entity. Rarely, Hodgkin's or "Hodgkin's-like" lymphoma associated with CLL has been reported. The authors present the clinicopathologic findings in eight cases of well-documented CLL in which solid tumors with histologic and immunostaining features of Hodgkin's disease subsequently developed. The histologic patterns observed in these tumors included nodular sclerosing (four cases) and mixed cellularity (four cases). In all eight cases, diagnostic Reed-Sternberg cells stained strongly with antibody to Leu-M1/CD15 but showed no reactivity with antibody to leukocyte common antigen (LCA/CD45). Survival ranged from 2 months to more than 8 years, with a 50% cumulative survival of 12 months; this is in contrast to the 2- to 4-month survival that typifies non-Hodgkin's Richter's syndrome. Therefore, it is important to recognize the Hodgkin's subgroup of lymphomatous CLL transformation.
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PMID:Hodgkin's disease variant of Richter's syndrome. Report of eight cases. 168 38

The authors investigated the structural organization of the bcl-1 locus, a putative oncogene associated with reciprocal chromosomal translocation t(11;14), by Southern blot hybridization analysis and its frequency, distribution, and prognostic significance in a panel of 156 clinically and pathologically well-defined B-cell chronic lymphocytic leukemias (CLLs) and non-Hodgkin's lymphomas (NHLs). The authors detected bcl-1 rearrangements in only 2 of 42 CLLs and 4 of 114 NHLs, specifically 3 of 29 diffuse small lymphocytic and 1 of 10 diffuse small cleaved cell and none of 5 diffuse intermediate lymphocytic, 13 follicular predominantly small cleaved, 17 follicular mixed small cleaved and large cell, 4 diffuse mixed small and large cell, 26 diffuse large cell, and 10 diffuse small noncleaved cell lymphomas. None of seven cases of Rai stage III or IV CLL or seven diffuse large cell lymphomas occurring as Richter's syndrome exhibited bcl-1 rearrangements. In conclusion, the bcl-1 locus rearranges in only about 4% of B-cell CLLs and NHLs, is predominantly rearranged in low-grade B-cell neoplasms, and does not appear to be preferentially associated with those occasional CLLs and low-grade NHLs displaying clinical aggressiveness, advanced clinical stage, or large cell transformation (Richter's syndrome). Therefore the demonstration of bcl-1 rearrangement does not appear to have clinically useful prognostic significance.
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PMID:bcl-1 rearrangement. Frequency and clinical significance among B-cell chronic lymphocytic leukemias and non-Hodgkin's lymphomas. 190 Mar 88

A 75-year-old man was admitted to our hospital because of hepatosplenomegaly, generalized lymphadenopathy and lymphocytosis in February, 1989. The leukocyte counts were 93,200/microliters with 95% small lymphocytes which expressed surface membrane immunoglobulin (SmIg) M, D and kappa. Histological finding of the cervical lymph node was diffuse small cell lymphoma. A diagnosis of chronic lymphocytic leukemia (CLL) was made. He was followed up without chemotherapy. In January, 1990, he was re-admitted because of progressively enlarged lymph nodes and increased white blood cell counts, up to 183,200/microliters with 98% lymphocytes. He was treated with vincristine, cyclophosphamide, prednisolone. The leukocyte counts decreased to 5,000/microliters and lymph node swelling decreased in size. In April, 1990, generalized lymphadenopathy re-appeared. The biopsied lymph node specimen showed diffuse large cell non-Hodgkin lymphoma (NHL-DL). The lymph node cells were found to express SmIgM and kappa. The diagnosis of Richter's syndrome was made. DNA analysis using Southern blot method revealed identical immunoglobulin heavy and kappa chain gene rearrangements in the two neoplasms. These findings suggest that the CLL cells and the NHL-DL cells originate from the same clone in this case.
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PMID:[Richter's syndrome with identical immunoglobulin gene rearrangements]. 190 17

We have investigated the frequency of p53 mutations in B- and T-cell human lymphoid malignancies, including acute lymphoblastic leukemia, the major subtypes of non-Hodgkin lymphoma, and chronic lymphocytic leukemia. p53 exons 5-9 were studied by using genomic DNA from 197 primary tumors and 27 cell lines by single-strand conformation polymorphism analysis and by direct sequencing of PCR-amplified fragments. Mutations were found associated with (i) Burkitt lymphoma (9/27 biopsies; 17/27 cell lines) and its leukemic counterpart L3-type B-cell acute lymphoblastic leukemia (5/9), both of which also carry activated c-myc oncogenes, and (ii) B-cell chronic lymphocytic leukemia (6/40) and, in particular, its stage of progression known as Richter's transformation (3/7). Mutations were not found at any significant frequency in other types of non-Hodgkin lymphoma or acute lymphoblastic leukemia. In many cases, only the mutated allele was detectable, implying loss of the normal allele. These results suggest that (i) significant differences in the frequency of p53 mutations are present among subtypes of neoplasms derived from the same tissue; (ii) p53 may play a role in tumor progression in B-cell chronic lymphocytic leukemia; (iii) the presence of both p53 loss/inactivation and c-myc oncogene activation may be important in the pathogenesis of Burkitt lymphoma and its leukemic form L3-type B-cell acute lymphoblastic leukemia.
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PMID:p53 mutations in human lymphoid malignancies: association with Burkitt lymphoma and chronic lymphocytic leukemia. 205 20

Oestrogen receptors (ER) are present in neoplastic lymphoid cells and have been considered a physiological marker of growth rate or differentiation. Tamoxifen, an oestrogen antagonist, has been given in some patients with CLL and Hodgkin's disease, with dramatic response in single cases. Until now, ER status has been assessed using a steroid binding assay (SBA) which has many inherent problems. Recently, the development of monoclonal antibodies directed against ER has been applied to the study of breast carcinomas and results obtained show good correlation with the quantitative SBA. We studied 49 cases of B-cell chronic lymphocytic leukemia (CLL) using immunostaining of cytospin preparations. In 30 of these cases ER enzyme immunoassay (ER-EIA) was also performed. Cultured MCF-7 cells, derived from a pleural effusion of a breast cancer patient, known to contain high levels of ER were used as a positive control (40-48% ER positive cells by immunocytochemistry; 200 fmol/mg protein by EIA). All of the CLL cases except two (96%) were negative for ER (less than 1% staining; less than 4 fmol/mg protein). The two positive cases expressed granular ER staining over the nucleus (9.2 and 12.1% positive cells) and were positive by EIA and SBA. It is concluded that (i) patients with CLL rarely express ER and (ii) immunocytochemical staining of cytospin preparations is a valid technique for the measurement of ER. It is of interest that one of the positive cases was diagnosed as CLL with Richter's transformation confirming earlier findings.
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PMID:Oestrogen receptor (ER) analysis in B-cell chronic lymphocytic leukemia: correlation of biochemical and immunocytochemical methods. 228 Jun 9

Richter's syndrome represents an acute transformation of chronic lymphocytic leukaemia or other type of low-grade malignant non-Hodgkin lymphoma to highly malignant anaplastic large-cell lymphoma. The prognosis of this complication is highly unfavourable. The authors report on eight cases of Richter's syndrome observed in a series of 198 patients with the clinical diagnosis of chronic lymphocytic leukaemia and evaluate some clinicopathological and immunological peculiarities of this disease.
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PMID:Clinicopathological aspects of Richter's syndrome. 244 10

Over a few year period chronic lymphocytic leukemia (CLL) is mainly characterized by stable course and only in the small per cent of patients it evolves into the aggressive form. Richter's syndrome has been diagnosed at our clinic in a patient who was for 6 years controlled and treated for CLL. In that period the patient was doing well and the out-patient treatment of CLL was being performed together with regular check-ups according to hematologic findings and clinical status of the patient. The patient was capable of work. Clinical investigation was undertaken because of the sudden aggravation of the general state and x-ray of the chest which pointed to the existence of tumorous shadow in the right lower pole of hilus. Although a complete CLL remission was established cytologic diagnosis of the newly formed tumour pointed to non-Hodgkin lymphoma of high malignity (centroblast lymphoma). Course of disease was terminated lethally very soon, because lymphoma of high malignity did not respond to polychemotherapy.
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PMID:[Clinical evolution of chronic lymphocytic leukemia in Richter's syndrome]. 270 88


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