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Query: UMLS:C0023418 (leukemia)
 93,477 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Patients with B-cell chronic lymphocytic leukemia (CLL) infrequently may develop high-grade B-cell lymphoma, or Richter's syndrome lymphoma (RS lymphoma). Such lymphomas differ from the original leukemia in both histology and clinical behavior. Studies seeking to define the clonal relationship between the cells of the two malignancies in any one patient have yielded conflicting reports. We examined the clonal relationship between the early and late neoplastic cells of a patient who underwent Richter's transformation. In contrast to the original leukemia cells, the secondary high-grade lymphoma was CD5-. However, both the leukemia cells and the evolved RS lymphoma expressed surface IgM lambda reactive with Lc1, a murine monoclonal antibody specific for a supratypic cross-reactive idiotype encoded by a subset of human Ig variable region genes of the VH4 subgroup. Nucleic acid sequence analyses of the heavy and light chain variable region genes expressed by both leukemia and lymphoma cells show that the CD5- B-cell lymphoma constitutes a clonal expansion of mutant cells derived from the original CD5+ B-cell leukemia. Moreover, certain sets of somatic mutations distinguish the Ig variable region genes used by RS lymphoma from those expressed by the CLL B cells. This is the first study to establish the clonal relationship between CLL and RS lymphoma through primary structural analyses of the expressed Ig genes.
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PMID:Common clonal origin of chronic lymphocytic leukemia and high-grade lymphoma of Richter's syndrome. 769 38

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.
Leukemia 1995 Apr
PMID:Localized gastric non-Hodgkin's lymphoma of high-grade malignancy in patients with pre-existing chronic lymphocytic leukemia or immunocytoma. 772 93

p53 protein expression has been investigated by immunohistochemistry in 58 patients with leukemia. Seven of 24 cases with acute myeloid leukemia (AML), 3 of 15 cases with chronic lymphocytic leukemia (CLL), one of 11 cases with chronic myeloid leukemia (CML) and 4 of 8 cases with acute lymphoid leukemia (ALL) had p53 protein expression. Of patients having p53 expression, one case with AML had refractory anemia with excess blasts-transformation (RAEB/t), one case with CLL had Richter's syndrome and another one with CML was in accelerated phase. Finally, 26% of leukemia cases had p53 protein expression. It may be concluded that p53 protein abnormalities may have an important role in leukomogenesis and in the development of more malignant clones in chronic leukemias.
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PMID:p53 protein expression in leukemias. 786 30

We present the clinical and immunological features of a rare case of chronic lymphoid leukaemia with lymphoplasmacytoid morphology. The patient was first admitted suffering from weakness, pallor, dyspnoea, marked splenomegaly, hepatomegaly and systemic lymphadenopathy and panhypogammaglobulinaemia. White blood cell count revealed important leukocytosis (220 x 10(9) WBC/l) with 2% neutrophils and 98% lymphoid cells showing lymphoplasmacytoid features, while lymphoid cells of identical morphology severely infiltrated the bone marrow and lymph nodes. The disease, initially controlled by non aggressive chemotherapy over a period of 30 months, later evolved to a clinical and haematological picture suggestive of Richter's syndrome. Immunophenotyping of the leukaemic cells demonstrated a monoclonal expansion of B-cells bearing surface markers of typical CLL (CD5, CD19, CD20, CD21, CD22, CD23, CD24, CD40 and low density IgM+IgD/kappa) and also the CD11c and CD38 antigens. A proportion of these cells expressed activation markers (CD25, CD69 and CD71). Following in vitro activation with TPA or PWM, the cells responded by weak incorporation of 3H-TdR but failed to secrete immunoglobulins. These findings confirm the broad morphological, phenotypical and clinical spectrum of chronic lymphoid leukaemias.
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PMID:Monoclonal expansion of immunoglobulin not-secreting CD5+ CD11c+ CD38+ B-cells in a rare case of chronic lymphoplasmacytoid leukaemia. 797 Dec 44

A 69-year-old woman presented with Rai stage 0 chronic lymphocytic leukemia. Ten years later she developed a diffuse centroblastic lymphoma involving the stomach. The surface membrane phenotype of the CLL cells was MD lambda while that of the large cell lymphoma (LCL) cells was MD kappa. The two populations had different heavy and kappa light chain rearrangements. Cytogenetic analysis of the CLL cells showed a deletion involving chromosome 13, band q14, but was unsuccessful in the LCL cells. However, use of a probe (p68 RS2.0) which recognizes a variable number tandem repeat sequence in the retinoblastoma gene, localized to chromosome 13q14, showed two alleles in the LCL cells but only one in the CLL cells. These data suggest that in this case of Richter's syndrome the CLL cells and the LCL cells are clonally distinct.
Leukemia 1993 Nov
PMID:Use of a retinoblastoma gene probe to investigate clonality in Richter's syndrome. 823 Dec 58

We describe an unusual case of small lymphocytic lymphoma with multiple prominent pseudofollicular proliferation centers in which immunohistochemistry reveals that the small cells are surface IgM kappa positive and the large pseudofollicular cells are surface IgA kappa positive. We further show by genomic DNA analysis that the tumor tissue contains two JH rearrangements, one Cmu rearrangement, one C alpha rearrangement, and a single C kappa rearrangement, strongly suggesting that the large cells within the proliferation centers have arisen from the small cells via a clonal heavy chain immunoglobulin isotype switch. Isotype switching in human lymphoma and leukemia appears to be an uncommon event. However, there are reports that strongly support isotype switching in pre-B-cell leukemia, Richter's syndrome, lymphoid blast crisis of chronic myelogenous leukemia, and multiple myeloma. To our knowledge, there have been no previous reports demonstrating isotype switching within the proliferation centers of small lymphocytic lymphoma. We present evidence of in vivo intratumoral isotype switching within the proliferation centers of a small lymphocytic lymphoma documented at the level of immunohistochemistry and DNA rearrangement.
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PMID:Clonal heavy chain isotype switching within the proliferation centers of a small lymphocytic lymphoma: implications regarding the origin of proliferation centers. 831 58

The development of a large-cell non-Hodgkin's lymphoma in a patient suffering from chronic lymphocytic leukaemia is known as Richter's syndrome, representing one of the possible anaplastic transformations of the leukaemia. Cutaneous involvement is an extremely rare event. The case of a 45-year-old man with B-cell chronic lymphocytic leukaemia is reported. Five years later multiple cutaneous nodules developed on his extremities. Biopsies of the skin showed a diffuse large-cell lymphoma, bearing the same cell surface immunoglobulin light chain isotype as the leukaemia, suggesting the original relationship between both neoplasms.
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PMID:Centroblastic transformation of chronic lymphocytic leukaemia with primary skin involvement--cutaneous presentation of Richter's syndrome. 834 23

We report the case of a 71-year old male patient with a chronic lymphoid leukaemia of 10 years' duration who abruptly suffered deterioration of this general condition and haematological disturbances suggesting worsening of this blood disease (Richter's syndrome). Blood cultures demonstrated a Campylobacter coli septicaemia. Treatment with antibiotics resulted in a return to the previous clinical and biological situation. The various visceral manifestation of the infection are discussed.
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PMID:[Hematologic manifestations in Campylobacter coli septicemia. Apropos of a case]. 836 7

The term Richter's syndrome is used to describe the transformation of chronic lymphatic leukaemia (CLL) into a high-grade systemic lymphoma and is associated with a poor prognosis. We have undertaken detailed molecular studies in two patients with cutaneous B-cell lymphoma (CBCL) and CLL. Patient 1 exhibited a low-grade CBCL with different immunoglobulin gene rearrangements in blood and skin. By contrast, patient 2 showed identical gene rearrangements, confirmed by gene sequencing, and died within 4 months of presentation. The latter patient fulfilled the criteria for a diagnosis of cutaneous Richter's syndrome, whereas the former patient demonstrated the coincidence of CLL with a primary CBCL. Our results highlight the importance of gene rearrangement studies with sequencing for the accurate diagnosis of cutaneous Richter's syndrome.
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PMID:B-cell lymphoma associated with chronic lymphatic leukaemia: two cases with contrasting aggressive and indolent behaviour. 1023 27

A retrospective analysis on chronic lymphocytic leukemia (CLL) patients </=55 years observed at a single institution was performed with the purpose of characterizing the clinical features and outcome of young CLL and of identifying patients with different prognostic features. Over the period from 1984 to 1994, 1,011 CLL patients (204 [20%] </=55 years of age and 807 [80%] >55 years of age) were observed. At diagnosis, younger and older patients displayed a similar distribution of clinical features, except for a significantly higher male/female ratio in younger patients (2.85 v 1. 29; P <.0001). Both groups showed an elevated rate of second primary cancers (8.3% v 10.7%), whereas the occurrence of Richter's syndrome was significantly higher in younger patients (5.9% v 1.2%; P <. 00001). Younger and older patients showed a similar overall median survival probability (10 years) but were characterized by a different distribution of causes of deaths: CLL unrelated deaths and second primary malignancies predominated in the older age group, whereas the direct effects of leukemia were prevalent in the younger age group. Although younger and older patients displayed a similar survival, the evaluation of the relative survival rates showed that the disease had a greater adverse effect on the expected survival probability of the younger population. Multivariate analysis showed that for young CLL patients only dynamic parameters, such as lymphocyte doubling time and other signs of active disease, were the independent factors that significantly influenced survival probability (P =.00001). A prolonged clinico-hematologic follow-up allowed us to identify two subsets of young CLL patients with a different prognostic outcome: a group of patients (40%) with long-lasting stable disease without treatment and an actuarial survival probability of 94% at 12 years from diagnosis and another group (60%) with progressive disease and a median survival probability of 5 years after therapy. For the latter patients, the therapeutic effect of innovative therapies with curative intents needs to be investigated in prospective, comparative clinical trials.
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PMID:Clinical characteristics and outcome of young chronic lymphocytic leukemia patients: a single institution study of 204 cases. 1039 12


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