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Query: UMLS:C0019829 (Hodgkin's disease)
 30,247 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

In a patient who developed Richter's syndrome, complex cytogenetic abnormalities of the centroblastic non-Hodgkin lymphoma (NHL) was associated with chemotherapy resistance. The clonal origin of the preexisting chronic lymphocytic leukemia (CLL) and the subsequent NHL cells was investigated. Both cell populations were present in the peripheral blood and could be separated efficiently by counterflow centrifugal elutriation. In the lymph node biopsy mainly NHL centroblasts were found and only a minor population of small lymphocytes. The separated CLL and NHL cells from blood as well as the lymph node cells were found to express mu and kappa Ig chains. Since expression of identical light chains is not synonymous with common clonal origin, Southern blot analysis of the Ig heavy chain genes was also performed, which showed that the two cell populations had identical Ig heavy chain gene rearrangements. Therefore, it was concluded that the CLL cells and the NHL cells in the present case originate from the same precursor cell and that the NHL has to be regarded as a malignant progression of the CLL. These findings are different from our previous report on another patient with Richter's syndrome, in whom the CLL and the NHL represented two unrelated malignancies. Therefore, the occurrence of NHL in Richter's syndrome apparently may represent either a clonal progression of the CLL or a second lymphoid malignancy.
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PMID:Richter's syndrome with identical immunoglobulin gene rearrangements in the chronic lymphocytic leukemia and the supervening non-Hodgkin lymphoma. 281 81

The authors report three cases of diffuse aggressive non-Hodgkin's lymphomas (large cell, two; mixed small and large cell, one) arising in association with clinically silent small lymphocytic proliferations involving bone marrow, and lymph nodes. Immunologic marker studies suggested origin from a common B cell clone in two cases; in one case the large cell lymphoma lacked detectable surface markers. Some cases of diffuse aggressive lymphomas of adults may arise by large cell transformation of clinically silent small lymphocytic proliferations, a process akin to Richter's syndrome.
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PMID:Large cell transformation of subclinical small lymphocytic leukemia/lymphoma: a variant of Richter's syndrome. 349 75

Clinical and histological findings of six patients with low grade non-Hodgkin lymphoma (NHL) which transformed to high grade tumours are reported: 5 out of the 6 cases showed transformation from the centroblastic-centrocytic type to the centroblastic-polymorphic type (Kiel classification). Clinical aggressivity, large tumour mass, high frequency of extranodal sites of disease, systemic symptoms and resistance to treatment were observed. This type of transformation is not rare, estimated to be about 20 p. 100 of cases in the literature, and is more common than Richter's syndrome in chronic lymphocytic leukaemia.
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PMID:[Transformation of malignant non-Hodgkin's lymphomas from low- to high-grade malignancy]. 377 43

Two cases of CLL terminating in Richter's syndrome and in blastic crisis raise the problem of the physiopathological signification of these unusual complications. Richter's syndrome is described as a "histiocytic" lymphoma or a Hodgkin's disease supervening on a CLL. It does not seem that these two pictures have actually to be distinguished since clinical and haematological aspects are not different, and histopathologic studies show always atypical aspects with difficulty of an accurate diagnosis. Cases of blastic crisis of CLL are very uncommon. They correspond to the transformation of a CLL in acute lymphoblastic leukaemia. These two terminal complications of CLL suggest they may be interpreted as two pictures of the transformation of the same initial B cellular clone. This hypothesis, highly probable for the Richter's syndrome, has been actually demonstrated for the acute transformation of CLL.
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PMID:[Richter's syndrome and acute transformation: two terminal haematological complications of chronic lymphoid leukaemia (author's transl)]. 624 58

In a patient with Richter's syndrome, the chronic lymphocytic leukemia (CLL) expressed lambda, mu, and delta immunoglobulin (lg) chains and the non-Hodgkin lymphoma (NHL) kappa, mu, and delta lg chains. The difference in lg light chain expression suggests that the CLL and NHL are independent malignancies, or that the oncogenic event occurred in a B cell differentiation stage after the heavy chain gene rearrangements but before the selection of the light chain. Analysis of DNA by Southern blotting revealed that the lg heavy chain genes of the two malignancies were rearranged in a different way. We therefore conclude that in this patient the NHL cannot be regarded as a progression of the CLL but should most likely be considered as an independent B cell malignancy, which arose in a susceptible host.
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PMID:Richter's syndrome with different immunoglobulin light chains and different heavy chain gene rearrangements. 633 51

The incidence of a lymphoreticular system malignancy in patients with chronic lymphocytic leukemia (CLL), Richter's syndrome (RS), is 3.3 to 10.6%. In 89 cases in the literature, the second neoplasm was either reticulum cell sarcoma or large cell diffuse histiocytic lymphoma (DHL), and there were 61 cases of Hodgkin's disease (HD). In a few cases the lymphoma was simultaneously diagnosed, for other cases an association with preexisting CLL was reported. Appearance of lymphoma was associated with leukemia regression for only 4 patients with DHL and 3 with HD. We report one case of B-lymphocyte CLL with macroglobulinemia, treated with melphalan and prednisone, in which DHL developed and the hematologic and histologic signs of CLL and of paraproteinemia remissed. Such patients might constitute a subgroup or a variant of RS. Since the non-Hodgkin malignant lymphomas (NHML) are considered to be monoclonal neoplastic expansion of the B-cell or T-cell lymphocytes, and since in some cases it has been proved that the proliferative cell clone was the same as that of the initial lymphoproliferative disease, RS could be a dedifferentiation or a transformation of CLL, resulting in an aggressive clinical course. The inclusion in this syndrome of CLL cases associated with HD is still controversial. Many of these cases could be giant cell pleomorphic lymphomas, while, on the contrary, in typical cases this association might be merely fortuitous.
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PMID:[Richter's syndrome. Presentation of a rare variant with regression of chronic lymphatic leukemia and review of the literature]. 652 45

A patient suffering from typical chronic lymphocytic leukaemia (CLL) developed a rapidly fatal high-grade lymphoma (Richter's syndrome). In cases of CLL Richter's syndrome must be distinguished from coexistent Hodgkin's disease and prolymphocytic transformation. The prognosis is dismal but not hopeless if the condition is treated early. Lymph node biopsy is essential for diagnosis and treatment.
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PMID:Richter's syndrome--an indication for lymph node biopsy in chronic lymphocytic leukaemia. A case report. 662 32

Richter's syndrome (RS) has been defined as "histiocytic" lymphoma (HL) or Hodgkin's disease (HD) supervening in the course of chronic lymphocytic leukemia (CLL) and related disorders. The clinical, histologic, and immunologic findings in 25 cases (11 women, 14 men) of RS are presented. The initial diagnosis was CLL in 19 cases, diffuse well-differentiated lymphocytic lymphoma in 2 cases, and Waldenstrom's macroglobulinemia in 4 cases. The interval between the initial diagnosis and that of RS ranged from 0 (two cases) to 120 months (median 49 months). At the time of diagnosis of RS, the initial lymphoproliferative disorder was in apparent complete remission in only two cases. The lymphoma was disseminated in at least 18 cases. The overall median survival was four months, but complete remission was achieved in six cases and has been maintained for 15 to 77 months. In four of these six cases, the RS was localized. The histologic diagnosis of HD was made in only two cases. In the other 23 cases, the diagnosis was HL, but in five of these cases, the proliferation was heterogeneous and was considered as an early aspect of HL. Immunologic studies of lymph node cell suspensions were performed in seven cases. In all cases, the B-lymphocytic origin of the lymphoma cells could be ascertained. Detailed studies in four cases showed that lymphoma cells carried SIg of the same isotype and light chain type as that of SIg detected on CLL cells or of monoclonal serum Ig. In these cases, the lymphoma was actually related to the initial B-cell chronic lymphoid disease.
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PMID:Malignant lymphoma supervening in chronic lymphocytic leukemia and related disorders. Richter's syndrome: a study of 25 cases. 702 53

Diffuse large B cell lymphomas (DLBLs) represent a heterogeneous collection of aggressive non-Hodgkin's lymphomas that can arise either de novo or as a result of transformation from chronic lymphocytic leukemia, small lymphocytic lymphoma, follicular lymphomas, or lymphomas of mucosa-associated lymphoid tissue. A small percentage of DLBLs express the CD5 antigen. The majority of these cases have evolved from a pre-existing low grade non-Hodgkin's lymphoma (Richter's syndrome). However, we identified and characterized nine CD5-positive DLBLs in which the patients did not have a previous history or concomitant evidence of chronic lymphocytic leukemia, small lymphocytic lymphoma, follicular lymphoma, or mucosa-associated lymphoid tissue-associated non-Hodgkin's lymphoma, suggesting that they arose de novo. All nine cases expressed CD20 and monotypic immunoglobulin, all eight cases examined expressed CD19, CD22 and CD43, eight of the nine cases expressed HLA-DR, and two of eight cases expressed CD11c. None of the cases expressed CD3, CD10, CD11b, CD21, CD23 or CD30. CD5 expression by these cells was found to be identical to that of CD5-positive B cell chronic lymphocytic leukemia by quantitative polymerase chain reaction analysis of CD5 mRNA. These nine de novo CD5-positive DLBLs exhibited clonal immunoglobulin heavy and light chain gene rearrangements but lacked integration of the Epstein-Barr virus genome and structural alterations of the bcl-1, bcl-2, c-myc, H-ras, K-ras, and N-ras proto-oncogenes and the p53 tumor suppressor gene. However, bcl-6 proto-oncogene rearrangement, which is involved in chromosome band 3q27 aberrations, was found in four cases (44.4%). This is comparable with the frequency of bcl-6 gene rearrangement in CD5-negative DLBL. In contrast, bcl-6 gene rearrangement was absent in six cases of DLBL associated with Richter's syndrome. These findings suggest that de novo CD5-positive DLBLs are genotypically similar to CD5-negative DLBLs and may be pathogenetically distinct from the DLBLs associated with Richter's syndrome.
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PMID:De novo CD5-positive and Richter's syndrome-associated diffuse large B cell lymphomas are genotypically distinct. 754 11

Analyses for clonality in cases of Richter's syndrome have provided evidence for a clonal evolution of high-grade lymphoma in most patients, while in others an independent cellular clone seems to exist in the secondary neoplasm. Richter's syndrome with an isolated high-grade lymphoma of the stomach has been rarely reported in patients with pre-existing B cell chronic lymphocytic leukemia (CLL). We investigated four cases of CLL or lymphoplasmacytoid immunocytoma (LPIC) with development of a localized high-grade B cell lymphoma in the stomach. Southern blotting showed different rearrangements of the immunoglobulin light and heavy chain genes in the tumor cells of the low-grade lymphoma and the gastric tumor in two cases. Comparison of the DNA sequences of the CDR3 region of the immunoglobulin genes revealed different clones in another case. By means of chromosomal in situ hybridization, trisomy 3 was detected in two cases of high-grade lymphoma of the stomach, but not in the cells of the associated low-grade tumor. Our findings indicate that high-grade non-Hodgkin's lymphomas arising localized in the stomach of patients with CLL or immunocytoma are not clonally related to the pre-existing low-grade lymphoma and, therefore indeed, present true secondary neoplasms.
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PMID:Localized gastric non-Hodgkin's lymphoma of high-grade malignancy in patients with pre-existing chronic lymphocytic leukemia or immunocytoma. 772 93


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