Composite Prolymphocytoid and Hodgkin Transformation of Chronic Lymphocytic Leukemia

2000 ◽  
Vol 124 (6) ◽  
pp. 907-909 ◽  
Author(s):  
Maureen J. O'Sullivan ◽  
Zahid Kaleem ◽  
Michael J. Bolger ◽  
Paul E. Swanson ◽  
Mary M. Zutter
Keyword(s):  
Chronic Lymphocytic Leukemia ◽  
Hodgkin Lymphoma ◽  
Progressive Disease ◽  
Plasma Cells ◽  
Single Agent ◽  
Malignant Neoplasm ◽  
Lymphocytic Leukemia ◽  
Richter Syndrome ◽  
Classic Hodgkin Lymphoma ◽  
Single Agent Chemotherapy

Abstract The indolent course of chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) is occasionally altered by transformation to a histologically distinct, rapidly progressive, and clinically unresponsive hematologic malignant neoplasm. We report a case of CLL that, after 3 years of slowly progressive disease and treatment with single-agent chemotherapy (fludarabine phosphate), underwent a composite prolymphocytoid and classic Hodgkin lymphoma transformation. The diagnosis of classic Hodgkin lymphoma was based on the presence of Reed-Sternberg cells with typical morphologic structure and immunophenotype (CD15+, CD30+, CD45−, CD20−) associated with the characteristic polymorphous inflammatory background consisting of numerous eosinophils, plasma cells, and reactive T lymphocytes. The remainder of the lymph node and the peripheral blood showed increased numbers of prolymphocytes admixed with typical small CLL cells. Recognition of such a transformation is of the utmost importance, since histologically similar Reed-Sternberg–like cells may be seen in Richter transformation. In contrast to prolymphocytoid transformation of CLL, Richter syndrome is rapidly fatal, with a median survival of 4 to 5 months. The patient pursued a clinical course similar to pure prolymphocytoid transformation and died with disease after 30 months following treatment with combination chemotherapy.

Outcome of Patients with Hodgkin Lymphoma transformed from Chronic Lymphocytic Leukemia

Blood ◽  
2015 ◽  
Vol 126 (23) ◽  
pp. 5298-5298
Author(s):  
Heidi Mocikova ◽  
Jana Markova ◽  
Lubica Gaherova ◽  
Lukas Smolej ◽  
Martin Simkovic ◽  
...  
Keyword(s):  
Chronic Lymphocytic Leukemia ◽  
Hodgkin Lymphoma ◽  
Conflicts Of Interest ◽  
Rare Event ◽  
Lymphocytic Leukemia ◽  
Richter Syndrome ◽  
Insufficient Response ◽  
Trisomy 12 ◽  
New Treatment ◽  
Involved Field

Abstract Background. Hodgkin lymphoma (HL) that develops in the course of chronic lymphocytic leukemia (CLL) is a rare event. Optimal management of these patients (pts) is not defined. We analyzed outcome of 10 pts treated for Hodgkin´s variant (HV) of Richter syndrome. Patients and methods. Overall 10 pts (8 males) with CLL and subsequent HV were treated between 1996 and 2015. Median age at diagnosis of HV was 68 (range 54 - 83) years. Prognostic markers of CLL at diagnosis: unmutated IGHV (5 pts), del(17p) or TP53 mutations (0 pt), del(11q) (2 pts), deletion of 13q14 region (4 pts), trisomy 12 (2 pts), ZAP 70 pos. (6 pts), CD38 pos.(4pts). The diagnosis of HV included various subtypes of HL: nodular sclerosis (3pts), mixed cellularity (3pts), lymphocyte rich (1pt), not othervise specified (3pts). EBV status of pts at diagnosis of HV of Richter syndrome: 6 pos., 1 negat. nad 3 not done. Initial and second-line treatments of CLL consisted of fludarabine- based regimens combined with rituximab or alemtuzumab and 3 pts received chlorambucil. Treatment of HV of Richter syndrome included: ABVD (7 pts) in combination with rituximab (2 pts), COPP (3 pts) and involved field radiotherapy of 30 Gy after chemotherapy (2 pts). Further treatment was required in 6 pts due to insufficient response with persistent CLL: rituximab alone (2 pts), R-bendamustine (1pt), R-DHAP (1 pt), cyclophosphamide and dexamethasone (1 pt) and gemcitabine (1 pt). None of these patients underwent autologous or allogeneic stem cell transplantation. Results. The median time from CLL diagnosis to development of HV was 8.4 (range 2.6 - 16.5) years. Median overall survival from diagosis of CLL was 17.3 years and median survival after diagnosis of HV was 3.5 years. Out of 10 pts 5 are alive :3 in complete remission (CR), 1in partial remission of CLL (HL in CR), 2 in progression of CLL (HL in CR). Five pts died (3 lymphoma progressions, 1 second cancer, 1 unknown causality). Conclusion. Our results indicate a long period for developing of HV of Richter syndrome and its subsequent poor outcome. Current HL based chemotherapy is not sufficient in HV of Richter syndrome and new treatment approaches should be considered. Disclosures No relevant conflicts of interest to declare.

scholarly journals Factors predicting survival in chronic lymphocytic leukemia patients developing Richter syndrome transformation into Hodgkin lymphoma

2017 ◽  
Vol 92 (6) ◽  
pp. 529-535 ◽  
Author(s):  
Francesca Romana Mauro ◽  
Piero Galieni ◽  
Alessandra Tedeschi ◽  
Luca Laurenti ◽  
Giovanni Del Poeta ◽  
...  
Keyword(s):  
Chronic Lymphocytic Leukemia ◽  
Hodgkin Lymphoma ◽  
Lymphocytic Leukemia ◽  
Richter Syndrome

scholarly journals Analysis with antiidiotype antibody of a patient with chronic lymphocytic leukemia and a large cell lymphoma (Richter's syndrome)

Blood ◽  
1987 ◽  
Vol 70 (1) ◽  
pp. 45-50 ◽  
Author(s):  
LF Bertoli ◽  
H Kubagawa ◽  
GV Borzillo ◽  
M Mayumi ◽  
JT Prchal ◽  
...  
Keyword(s):  
B Cells ◽  
Chronic Lymphocytic Leukemia ◽  
Plasma Cells ◽  
Cell Lymphoma ◽  
Large Cell ◽  
Lymphocytic Leukemia ◽  
Immunoglobulin Gene ◽  
Anaplastic Lymphoma ◽  
Large Cell Lymphoma ◽  
Richter’S Syndrome

Abstract A murine monoclonal antibody made against an idiotypic determinant (Id) of surface IgM/IgD lambda molecules on chronic lymphocytic leukemia (CLL) cells of a 71-year-old woman was used for clonal analysis by two- color immunofluorescence. The anti-Id antibody identified IgM+/IgD+/lambda+ B cells as the predominant cell type of her CLL clone. In addition, substantial proportions of the IgG and IgA B cells and most of the IgM plasma cells in her bone marrow and blood were Id+. Six years after diagnosis, the patient died of respiratory failure due to infiltration of lungs by malignant cells. Autopsy revealed a dramatic change in the tumor cell morphology. The lungs, hilar nodes, and liver were infiltrated by a diffuse large cell lymphoma admixed with the leukemic cells. By immunohistologic staining these anaplastic lymphoma cells were IgM+/IgD-/lambda+ B cells expressing the same Id noted earlier on the CLL cells. The immunoglobulin gene rearrangement pattern on Southern blot analysis was also the same in leukemic blood cells and in the tissues involved by the lymphoma. Thus, the combination of antiidiotype and immunoglobulin gene analyses in this patient with Richter's syndrome revealed that a CLL clone, seemingly “frozen” in differentiation, was actually undergoing isotype switching, differentiation into plasma cells, and evolution into a rapidly growing and fetal lymphoma.

Phase I to II Multicenter Study of Oblimersen Sodium, a Bcl-2 Antisense Oligonucleotide, in Patients With Advanced Chronic Lymphocytic Leukemia

2005 ◽  
Vol 23 (30) ◽  
pp. 7697-7702 ◽  
Author(s):  
Susan M. O'Brien ◽  
Charles C. Cunningham ◽  
Anatoliy K. Golenkov ◽  
Anna G. Turkina ◽  
Steven C. Novick ◽  
...  
Keyword(s):  
Chronic Lymphocytic Leukemia ◽  
Phase I ◽  
Phase Ii ◽  
Intravenous Infusion ◽  
Single Agent ◽  
Plasma Concentrations ◽  
Parent Drug ◽  
Lymphocytic Leukemia ◽  
Complete Disappearance ◽  
Continuous Intravenous Infusion

Purpose To determine the maximum-tolerated dose (MTD), efficacy, safety, and pharmacokinetics of oblimersen sodium in patients with advanced chronic lymphocytic leukemia (CLL). Patients and Methods Eligible patients had relapsed or refractory CLL after treatment with fludarabine. Oblimersen was administered at doses ranging from 3 to 7 mg/kg/d as a 5-day continuous intravenous infusion in cycle 1 and as a 7-day continuous intravenous infusion in subsequent cycles every 3 weeks in stable or responding patients. Results Forty patients were enrolled and treated (14 patients in phase I and 26 patients in phase II). Dose-limiting reactions in phase I included hypotension and fever, and the MTD for phase II dosing was established at 3 mg/kg/d. Two (8%) of 26 assessable patients achieved a partial response. Other evidence of antitumor activity included ≥ 50% reduction in splenomegaly (seven of 17 patients; 41%), complete disappearance of hepatomegaly (two of seven patients; 29%), ≥ 50% reduction of lymphadenopathy (seven of 22 patients; 32%), and ≥ 50% reduction in circulating lymphocyte counts (11 of 22 patients; 50%). Adverse events included transient hypotension, fever, fatigue, night sweats, diarrhea, nausea, vomiting, hypokalemia, and cough. Plasma concentrations of oblimersen (parent drug) and its major metabolites were variable. Renal clearance represented only a small portion of total parent drug clearance. Conclusion Dosing with oblimersen sodium in patients with CLL is limited by development of a cytokine release syndrome that is characterized by fever, hypotension, and back pain. Oblimersen sodium has modest single-agent activity in heavily pretreated patients with advanced CLL, and further evaluation of its activity in combination with cytotoxic drugs is warranted.

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