Richter's syndrome: Diffuse histiocytic lymphoma in patients with chronic lymphocytic leukemia

Trump, Donald L.; Mann, Risa B.; Phelps, Roger; Roberts, Huw; Conley, C. Lockard

doi:10.1016/0002-9343(80)90300-9

Cited by 138 publications

(60 citation statements)

References 29 publications

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“…Histological progression to large-cell lymphoma also corresponds to a general clinical alteration, as observed in our patients (1,2,12). Transformation occurs in lymph nodes, bone marrow, spleen, or other tissues (1, 2, 12).…”

Section: Discussionsupporting

confidence: 75%

“…This process is commonly referred to as Richter's syndrome (RS; 9, 10). RS is characterized by rapid lymph node enlargement, splenomegaly, hepatomegaly, fever, and weight loss and is associated with a rapid fatal outcome (1,2,9,11,12). Because patients with CLL have a greater tendency to develop secondary tumors, there has been a debate about the origin of the DLCL.…”

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confidence: 99%

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Primary Digestive Richter's Syndrome

et al. 2001

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The clinical and morphologic transformation of 3 to 5% of chronic lymphocytic leukemia (CLL) to diffuse large-cell lymphoma (DLCL) is commonly referred to as Richter's syndrome. Richter's syndrome occurs mostly in lymph nodes and may represent a second neoplasm or a transformation from the same clonal population. Clinical features in six patients with digestive Richter's syndrome were recorded. Paired samples of CLL and DLCL were investigated by immunohistological analysis (n ‫؍‬ 6) and by polymerase chain reaction (PCR) for immunoglobulin heavy-chain gene rearrangement (n ‫؍‬ 4). Histological examination revealed the involvement of the gastrointestinal tract by DLCL of B-cell phenotype (n ‫؍‬ 6). The same monoclonal rearrangement between CLL and DLCL was demonstrated by PCR and sequencing analyses in two patients. The monoclonal rearrangement was different between CLL and DLCL in only one case. Median survival was 22 months for five patients receiving chemotherapy, suggesting that digestive Richter's syndrome has a better prognosis than nodal Richter's syndrome. Indeed, appropriate surgical resection combined with chemotherapy led to partial or complete remission in four patients.

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Section: Discussionsupporting

confidence: 75%

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confidence: 99%

Primary Digestive Richter's Syndrome

et al. 2001

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“…Some unusual variants of TLCL have been reported: transformation to lymphoma with Hodgkin-like cells, large cells with histiocytic differentiation, and even high-grade T-cell lymphoma. 15,17,18 In SLL-AP, the aspirate is usually more heterogeneous, comprised of small lymphoid cells admixed with scattered large cells. Recognition of SLL-AP, therefore, can be challenging in some patients based on the findings from routine stains alone.…”

Section: Discussionmentioning

confidence: 99%

Cytomorphologic spectrum of small lymphocytic lymphoma in patients with an accelerated clinical course

Shin

Caraway

Katz

2003

Cancer

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“…Skeletal radiography proven in three cases. 5,7,8 Radiologic skeletal abnorrevealed multifocal osteolysis in the left ribs, with inmalities were also observed three times: diffuse ostense isotope uptake on bone scintigraphic scans. teopenia twice and multifocal osteolysis once.…”

Section: 17mentioning

confidence: 98%

“…[5][6][7][8] The authors report four cases of such an association, including complementary examinations undertaken to study hyper-Lymphoproliferative disorders were diagnosed which led to the diagnosis of RS. Skeletal radiography showed diffuse osteopenia without any local lytic bone based on bone marrow or lymph node biopsy, according to the International Working Formulation.…”

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confidence: 99%

Hypercalcemia may indicate Richter's syndrome

Beaudreuil

Lortholary

Martin

et al. 1997

Cancer

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lymphocytic leukemia in a woman 73 years of age. Radiologic and histologic feaPhilippe Casassus, M.D. 1 tures were reported. Circulating levels of parathormone-related peptide (PTHrP), tumor necrosis factor-alpha (TNF-a), and interleukin-6 (IL-6) were measured. 1 Service de Médecine Interne, Hôpital Avi- RESULTS.Radiologic skeletal abnormalities were found in three of three cases:cenne, Université Paris-Nord, Cedex, France.osteopenia twice and multifocal osteolysis once. All four patients had large cell 2 Service d'Anatomie et Cytologie Pathologlymphoma (LCL). Bone resorption assessed histomorphometrically was elevated iques, Hôpital Avicenne, Université Paris-Nord, in two of two cases. Serum TNF-a and IL-6 levels were high in three of three and Cedex, France.one of three cases, respectively. Elevated values were also found in four patients with LCL but without hypercalcemia. The serum PTHrP level was increased in the 3 Service d'Hématologie Biologique, Hôpital Avicenne, Université Paris-Nord, Cedex, France.only hypercalcemic patient tested and values were normal in the three control patients. CONCLUSIONS.Hypercalcemia arising in a patient with a low grade lymphoproliferative disorder may indicate RS. Hypercalcemia is due to increased bone resorption, which may be caused by the secretion of osteoclast-stimulating factors by the LCL invading the bone marrow. Independently, TNF-a and/or IL-6 were unlikely causative factors of hypercalcemia in the patients in this study; however, synergism with PTHrP was suspected in one case.

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Richter's syndrome: Diffuse histiocytic lymphoma in patients with chronic lymphocytic leukemia

Cited by 138 publications

References 29 publications

Primary Digestive Richter's Syndrome

Primary Digestive Richter's Syndrome

Cytomorphologic spectrum of small lymphocytic lymphoma in patients with an accelerated clinical course

Hypercalcemia may indicate Richter's syndrome

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