Hodgkin’s disease type Richter’s syndrome in chronic lymphocytic leukemia

Juneja, Surender; Carney, Dennis; Ellis, David W.; Januszewicz, E.; Wolf, Max; Prince, H. Miles

doi:10.1038/sj.leu.2401389

Cited by 13 publications

(8 citation statements)

References 6 publications

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“…Brecher et al found that in the group of 8 patients with H-RS treated in their center the survival ranged between 2 months to 8 years and was 12 months on average [10], while in RS manifesting as NHL it was 2-4 months [10]. Similar results were obtained by others [3,12,16]. In the majority of cases these patients respond well to the chemotherapy regimens usually applied in classical HD, such as MOPP (mustard, vincristine, procarbazine, prednisone), BCVP (carmustine, cyclophosphamide, vincristine, procarbazine, prednisone) or ABVD.…”

Section: Discussionsupporting

confidence: 64%

“…We cannot exactly define, what type of H-RS developed in our case. However, in FNB RS-cells were surrounded by small monomorphic lymphocytes with expression of CD5 and CD20, what strongly suggests type 1 H-RS [5,16].…”

Section: Discussionmentioning

confidence: 99%

“…It has been demonstrated by single cells PCR analysis and DNA sequencing and by immunohistochemical investigation that in some cases of H-RS, Hodgkin and CLL cells belong to the same clonal population [5,14,15]. However, in other cases the immunohistological and IgH PCR analysis have not showed the presence of CLL cells in the background, which is mainly composed of CD3/CD4/CD5 þ lymphocytes as is usually observed in the classical HD [14,16].…”

Section: Introductionmentioning

confidence: 99%

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Hodgkin's Type of Richter's Syndrome in Familial Chronic Lymphocytic Leukemia Treated with Cladribine and Cyclophosphamide

Robak

Szmigielska-Kapłon

Smolewski

et al. 2003

Leukemia & Lymphoma

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Second malignancies are frequent complications in patients with chronic lymphocytic leukemia (CLL). Hodgkin's disease (HD) has been observed in approximately 0.5% of the patients with CLL and is known as Hodgkin's type Richter's syndrome (H-RS). We present a 64-year-old male patient with a familial history of CLL who developed H-RS in abdominal lymph nodes 6 years after CLL diagnosis and 18 months after treatment with cladribine (2-CdA) and cyclophosphamide. HD was diagnosed by fine needle aspiration. The disease was refractory to treatment with two courses of CHOP and three courses of ABVD chemotherapy. In the current literature we found case reports of only 6 patients with H-RS who were treated with fludarabine (FA) before transformation, and, to our knowledge the presented patient is the first to develop H-RS after treatment with 2-CdA combined with cyclophosphamide. He is also the first published patient with familial CLL in whom this complication developed.

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Section: Discussionsupporting

confidence: 64%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Hodgkin's Type of Richter's Syndrome in Familial Chronic Lymphocytic Leukemia Treated with Cladribine and Cyclophosphamide

Robak

Szmigielska-Kapłon

Smolewski

et al. 2003

Leukemia & Lymphoma

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“…Only a handful of reports on Hodgkin's lymphoma (HL) manifesting as a variant of RS, originally described by Richter (1928) and called ‘reticular cell sarcoma’, have been published, particularly in patients treated with purine analogues (Choi & Keller, 1981; Brecher & Banks, 1990; Shin et al , 1993; Rubin et al , 1994; Butts et al , 1995; Weisenberg et al , 1995; Fayad et al , 1996; Juneja et al , 1999; Kanzler et al , 2000; Adiga et al , 2003; Nemets et al , 2003). The frequency of CLL transformation into HL varies between 0.5% and 2.3% (Fayad et al , 1996; Keating et al , 1998).…”

mentioning

confidence: 99%

Hodgkin's disease variant of Richter's syndrome in chronic lymphocytic leukaemia patients previously treated with fludarabine

Fong¹,

Kaiser

Spizzo³

et al. 2005

Br J Haematol

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Summary The transformation of chronic lymphocytic leukaemia (CLL) into large‐cell lymphoma (Richter's syndrome, RS) is a well‐documented phenomenon. Only rarely does CLL transform into Hodgkin's lymphoma (HL). To further analyse the clinico‐pathological and genetic findings in the HL variant of RS, we performed a single‐institution study in four patients, who developed HL within a mean of 107 months after diagnosis of CLL. All were treated with fludarabine. Three cases were Epstein–Barr virus (EBV)‐associated mixed cellularity (MC) HL, the fourth was nodular sclerosis (NS) HL without EBV association. The sites involved by HL included supra‐ and infradiaphragmal lymph nodes and the tonsils; stage IV disease was also documented. All patients presented with CLL treatment‐resistant lymphadenopathies and B‐symptoms. In two of the MC cases, molecular analysis performed on CLL samples and microdissected Hodgkin and Reed–Sternberg cells (HRSC) suggested a clonal relationship, while in NS no indication of a clonal relationship was detected. In summary, HL can occur in CLL patients at any site, up to 17 years after initial diagnosis, especially after treatment with fludarabine. The majority present with B‐symptoms and CLL treatment‐resistant lymphadenopathy, are of the MC type, clonally related to CLL and might be triggered by an EBV infection.

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“…The diagnosis of large‐cell type or especially Hodgkin's‐type Richter's transformation of CLL can be confirmed on PSI of trephine biopsies using a panel of antibodies specific for B and T lineages and for Hodgkin's cells; in many cases, such diagnosis cannot be made by flow cytometry of the bone marrow aspirate specimen ( Juneja et al , 1999 ).…”

Section: Chronic Leukaemiasmentioning

confidence: 99%