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

The authors describe a rare case of Richter's syndrome (transition of chronic lymphatic leukaemia into reticulosarcoma) developing during rheumatoid arthritis in a women aged 53 years. Chronic antigenic stimulation and disturbances of interaction between lymphocytes T and B are suggested as a cause of Richter's syndrome.
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PMID:[Richter's syndrome and cervical cancer in the course of a rheumatoid arthritis]. 33 67

We report clinical, morphological and surface marker studies on seven patients with the common type of chronic lymphocytic leukaemia (CLL) whose disease underwent an insidious though progressive change in character with increasing refractoriness to treatment. This transformation was accompanied by the appearance of a population of immature-appearing cells in the peripheral blood which resembled prolymphocytes, both at light and electron microscopy. The characteristic morphological feature was the presence of two distinct populations of cells, the typical CLL lymphocytes and the 'prolymphocytoid' cells. These cells retained the surface characteristics of CLL, i.e. the information of mouse RBC rosettes and sparse surface-bound immunoglobulin. This transformation can be distinguished by morphological and surface marker criteria from acute leukaemia occurring in CCL, Richter's syndrome and prolymphocytic leukaemia. The recognition of this group of CLL patients may add a new prognostic index to CLL and may help plan subsequent trials for the treatment of the disease.
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PMID:'Prolymphocytoid' transformation of chronic lymphocytic leukaemia. 42 Jul 39

Chronic lymphocytic leukemia (CLL) is the most common leukemia in the Western world and the only leukemia for which a possible genetic component has been described. Analysis of this genetic component has been hindered by the fact that disease onset normally occurs after age 50. We report here the aged NZB mouse as an animal model for CLL. NZB mice have a genetically regulated, age-dependent onset of clonal, aneuploid cells which are IgM+ and Ly1+ (CD5+ B-cells). Peripheral blood smears from old NZB mice show an increase in circulating lymphocytes and "smudged" or ruptured cells, often seen in human CLL. Electron microscopic examination of these cells shows them to be mature lymphocytes. Light microscopy of the spleen shows infiltration of small lymphocytes and is consistent with CLL pathology. These long-lived, CLL-like cells can be serially passaged into recipient animals. This continued passage occasionally results in the development of a large cell lymphoma detectable in the spleen, lymph nodes, and liver. The histology of this lymphoma is quite distinct from that of the CLL-like cells, but the phenotype is that of an aneuploid CD5+, IgM+ cells. This apparently represents a continued transformation of the CLL-like clone similar to the development of Richter's syndrome in human CLL. Therefore, the NZB mouse can be a valuable tool for the determination of the genetic basis of CLL ontogeny and the conversion of CLL into Richter's syndrome.
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PMID:The NZB mouse as a model for chronic lymphocytic leukemia. 137 Feb 14

Ki-67 is a monoclonal antibody that recognises a nuclear antigen expressed during most phases of the cell cycle. We have analysed, by immunocytochemistry, the frequency, morphology, and clinical significance of Ki-67+ cells in 108 patients with B-cell chronic lymphocytic leukemia (CLL). Because in normal peripheral blood Ki-67+ cells are mainly T lymphocytes, we have also investigated, by double immunoenzymatic staining, the proportion of Ki-67+ T cells (Ki-67/CD3+) in CLL. Four groups of patients were identified: (i) 47 with stage A, (ii) 32 with stages B + C, (iii) 24 with greater than 10% of circulating prolymphocytes (CLL/PL) and (iv) five with Richter's syndrome. Within stage A CLL, two groups were considered: A' (Hb greater than or equal to 12 g/dl and lymphocytes less than 30 + 10(9)/l) and A" (Hb less than 12 g/dl or lymphocytes greater than or equal to 30 x 10(9)/l). The percentage and absolute number of Ki-67+ leukemic cells was found to increase with the stage of the disease and correlate with the proportion of prolymphocytes. On the other hand, the proportion of Ki-67+ T cells (CD3+) was significantly higher in patients with CLL stage A' (29.3 +/- 4.5), which includes patients with long-standing, stable disease, than in CLL stage A" (9.5 +/- 3.3), B + C (7.1 +/- 4.6), and CLL/PL (6.4 +/- 2.8). Ki-67 seems to identify patients with more aggressive forms of CLL, such as CLL/mu 2PL with more than 10% Ki-67+ cells (25% of the cases) and Richter's syndrome, in which all the large lymphoma cells are Ki-67+. Long-term follow-up will establish whether Ki-67 is a good prognostic marker and can predict disease outcome.
Leukemia 1992 Sep
PMID:Monoclonal antibody Ki-67 identifies B and T cells in cycle in chronic lymphocytic leukemia: correlation with disease activity. 138 94

Terminal transformation of chronic lymphocytic leukaemia (CLL) comparable to the blast-cell transformation which is responsible for almost all deaths in the chronic myeloid leukaemias is a rare event because the majority of CLL patients die without a major or easily recognised morphological transformation of the leukaemia cells having occurred. However, a substantial proportion of these patients die with progressive treatment-resistant disease or from intractable infections resulting from CLL-related immunodeficiency. In many of these patients biological transformation of the leukaemia cells can be associated with the appearance of complex chromosomal changes not present earlier or additional to the commonly present trisomy 12(1). In a broad sense, therefore, all of these patients may be said to undergo lethal transformation of their disease in contrast to the, admittedly substantial numbers, especially of elderly males who die from causes totally unrelated to their CLL, especially cardiovascular and cerebrovascular diseases, and other malignant diseases. In the narrow sense the term 'terminal transformation' is usually restricted to those conditions in which the terminal event is the proliferation of a new population of lymphoid cells of enhanced malignancy. There are three well-known types of transformation. One, 'prolymphrocytoid' transformation is relatively low-grade, while the other two, 'Richter's syndrome' and 'immunoblastic transformation' are rapidly progressive conditions. The last two account for about 5% of all deaths in CLL, but the slowly progressive prolymphocytoid transformation has been described too recently to permit reliable estimates of its frequency in unselected series to CLL. It may be of the order of 10%.
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PMID:Terminal transformation in B-cell chronic lymphocytic leukaemia. 265 95

Primary parenchymal central nervous system (CNS) non-Hodgkin's lymphoma subsequently developed in two patients with chronic lymphocytic leukemia (CLL). These two patients represent what we believe to be the first examples of Richter's syndrome due to primary brain lymphoma. Neither evidence for systemic lymphoma nor of progression of the leukemia was found. We believe that the description of these two cases expands the clinical spectrum in which Richter's syndrome may occur. In patients with CLL, careful attention must be given to neurologic symptoms, particularly those that develop abruptly. Primary CNS lymphoma must enter into the differential diagnosis when a cerebral mass lesion is found in such patients by the appropriate neuro-imaging.
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PMID:Primary central nervous system lymphoma as a variant of Richter's syndrome in two patients with chronic lymphocytic leukemia. 276 25

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.
Leukemia 1989 Nov
PMID:Richter's syndrome with identical immunoglobulin gene rearrangements in the chronic lymphocytic leukemia and the supervening non-Hodgkin lymphoma. 281 81

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

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

Apoptosis, or programmed cell death, was studied in B-1 (CD5+ B) cells from NZB mice and their hybrids. NZB mice, as they age, develop clones of B-1 cells with the majority of the clones possessing extra chromosomes (hyperdiploid). These clones differ in growth characteristics, varying from a slow-growing non-invasive clonal expansion of B-1 cells, similar to chronic lymphocytic leukemias (CLL), to an aggressive fast-growing invasive malignancy, similar to Richter's syndrome. Apoptosis was induced in cultures of B-1 cells purified from peritoneal wash-out cells with either anti-immunoglobulin (anti-IgM) or lipopolysaccharide (LPS). The malignant hyperdiploid B-1 cells had increased apoptosis in response to these stimuli as determined by the presence of fragmented DNA. The amount of apoptosis was directly related to the aggressive nature of the B-1 cells. The increased apoptosis observed in malignant B-1 cells was also correlated with the state of activation of the cells. Malignant B-1 cells undergoing high levels of apoptosis had high spontaneous activation and IgM production. The supernatant levels of IgM in unstimulated cultures of aggressive malignant B-1 cells were the same as that stimulated with LPS, indicating that the malignant B-1 clones were maximally activated in vivo. In conclusion, hyperdiploid B-1 cells appear to have altered responses to stimuli that normally activate mature B cells. A signal for apoptosis rather than stimulation may result when malignant B-1 clones have their antigen receptors engaged. The increased apoptosis capability of malignant B-1 cells may be exploited as a therapeutic tool.
Leukemia 1993 Jun
PMID:Apoptosis induction in CD5+(Ly1+) malignant B cells. 768 96


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