Selection of initial therapy for symptomatic or advanced chronic lymphocytic leukemia
- Kanti R Rai, MD
Kanti R Rai, MD
- Professor of Medicine and Molecular Medicine
- Hofstra North Shore-LIJ School of Medicine at Hofstra University
- Stephan Stilgenbauer, MD
Stephan Stilgenbauer, MD
- Professor of Medicine
- Ulm University
Chronic lymphocytic leukemia (CLL) is one of the chronic lymphoproliferative disorders (lymphoid neoplasms). It is characterized by a progressive accumulation of functionally incompetent B lymphocytes, which are usually monoclonal in origin.
CLL is considered to be identical (ie, one disease with different manifestations) to the mature (peripheral) B cell neoplasm small lymphocytic lymphoma (SLL), one of the indolent non-Hodgkin lymphomas. The term CLL is used when the disease manifests primarily in the blood, whereas the term SLL is used when involvement is primarily nodal. While there is some difference to the treatment of early stage CLL and SLL, the treatment of advanced stage disease is the same. (See "Clinical presentation, pathologic features, diagnosis, and differential diagnosis of chronic lymphocytic leukemia".)
The selection of initial therapy for advanced stage or symptomatic disease will be reviewed here. General issues regarding the treatment of CLL/SLL, the treatment of relapsed/refractory CLL, the use of hematopoietic cell transplantation in CLL, and the management of complications of CLL and its treatment are discussed separately. (See "Overview of the treatment of chronic lymphocytic leukemia" and "Overview of the complications of chronic lymphocytic leukemia" and "Treatment of relapsed or refractory chronic lymphocytic leukemia" and "Hematopoietic cell transplantation in chronic lymphocytic leukemia".)
The pathophysiology, clinical manifestations, diagnosis, staging, and prognosis of CLL and SLL are also discussed separately. (See "Clinical presentation, pathologic features, diagnosis, and differential diagnosis of chronic lymphocytic leukemia" and "Staging and prognosis of chronic lymphocytic leukemia" and "Pathophysiology and genetic features of chronic lymphocytic leukemia".)
Our general approach — There is no single agreed upon standard treatment regimen for symptomatic chronic lymphocytic leukemia (CLL) or advanced small lymphocytic lymphoma (SLL). Experts in the field use different treatment approaches. As such, there are several initial treatment options for patients with symptomatic CLL or advanced SLL. These include the following options, administered as single agents or as combinations:
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- INITIAL THERAPY
- Our general approach
- Infectious complications and prophylactic antimicrobials
- Fludarabine-based therapy
- - Choice of fludarabine regimen
- - Fludarabine, cyclophosphamide, and rituximab
- - Fludarabine plus rituximab
- Bendamustine-based therapy
- Pentostatin-based therapy
- SPECIAL PATIENT POPULATIONS
- Older patients
- - Defining "older adult"
- - Choice of therapy
- - Ibrutinib
- - Chlorambucil-based therapy
- Chlorambucil plus anti-CD20 monoclonal antibodies
- Single agent chlorambucil
- - Fludarabine-based regimens
- High-risk disease: del(17p) or TP53 mutations
- SUMMARY AND RECOMMENDATIONS