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The decision of when to start treatment for patients with chronic lymphocytic leukemia (CLL) has not markedly changed; it is triggered by parameters of active disease, such as progressive symptoms or progressive cytopenias related to bone marrow compromise from disease. How to treat patients with CLL has become less complicated, given the growing list of options for CLL, and generally depends on the patient's age, presence of comorbidities, and risk factors (such as chromosome 17 or 11 deletion). During his presentation at the NCCN 20th Annual Conference, Dr. William Wierda focused attention on many of the new kids on the therapeutic block for CLL—the CD20 monoclonal antibodies obinutuzumab and ofatumumab, the BTK inhibitor ibrutinib, the PI3K inhibitor idelalisib, and the BCL-2 inhibitor venetoclax—and reviewed some of the clinical data supporting the use of these agents in different patient populations with CLL.

Progress in the treatment of chronic lymphocytic leukemia/small lymphocytic lymphoma has led to a renewed focus on minimal residual disease as a treatment endpoint, particularly in assessing survival outcomes with combination therapies. B-cell receptor signaling pathway inhibitor monotherapy is associated with low rates of undetectable minimal residual disease, and continued treatment is still required; however, BCL-2 inhibitor therapy with venetoclax is associated with high rates of undetectable minimal residual disease, as are new combinations with BCL-2 inhibitors. Early research indicates that BCL-2 inhibitors may be an effective treatment option for disease that is refractory to Bruton’s tyrosine kinase (BTK) inhibitor therapy.

With the enormous progress made in treatment and management, many oncologists have called this the golden age of chronic lymphocytic leukemia (CLL). The past few years alone have seen the approval of multiple agents, including small molecule inhibitors that have led to longer, more durable periods of disease control. However, the introduction of these new drugs into the armamentarium has raised an important question regarding standard of care: is there still a role for chemoimmunotherapy in the first-line setting? At the NCCN 2019 Annual Congress: Hematologic Malignancies, Drs. William G. Wierda and Jennifer R. Brown presented opposing sides of the debate.

Chronic lymphocytic leukemia (CLL) is generally characterized by an indolent disease course. Histologic transformation (also known as Richter's transformation) to more aggressive lymphomas, such as diffuse large B-cell lymphoma or Hodgkin lymphoma, occurs in approximately 2% to 10% of patients and is associated with a poor prognosis. These NCCN Guidelines Insights discuss the recommendations for the diagnosis and management of patients with histologic transformation.

Hairy cell leukemia (HCL) is a rare type of indolent B-cell leukemia, characterized by symptoms of fatigue and weakness, organomegaly, pancytopenia, and recurrent opportunistic infections. Classic HCL should be considered a distinct clinical entity separate from HCLvariant (HCLv), which is associated with a more aggressive disease course and may not respond to standard HCL therapies. Somatic hypermutation in the IGHV gene is present in most patients with HCL. The BRAF V600E mutation has been reported in most patients with classic HCL but not in those with other B-cell leukemias or lymphomas. Therefore, it is necessary to distinguish HCLv from classic HCL. This manuscript discusses the recommendations outlined in the NCCN Guidelines for the diagnosis and management of classic HCL.

Non-Hodgkin’s lymphomas (NHL) are a heterogeneous group of lymphoproliferative disorders originating in B lymphocytes, T lymphocytes, or natural killer cells. Mantle cell lymphoma (MCL) accounts for approximately 6% of all newly diagnosed NHL cases. Radiation therapy with or without systemic therapy is a reasonable approach for the few patients who present with early-stage disease. Rituximab-based chemoimmunotherapy followed by high-dose therapy and autologous stem cell rescue (HDT/ASCR) is recommended for patients presenting with advanced-stage disease. Induction therapy followed by rituximab maintenance may provide extended disease control for those who are not candidates for HDT/ASCR. Ibrutinib, a Bruton tyrosine kinase inhibitor, was recently approved for the treatment of relapsed or refractory disease. This manuscript discusses the recommendations outlined in the NCCN Guidelines for NHL regarding the diagnosis and management of patients with MCL.

Chronic lymphocytic leukemia (CLL) and small lymphocytic lymphoma (SLL) are different manifestations of the same disease, which are managed in the same way. The advent of novel monoclonal antibodies (ofatumumab and obinutuzumab) led to the development of effective chemoimmunotherapy regimens. The recently approved small molecule kinase inhibitors (ibrutinib and idelalisib) are effective treatment options for CLL in elderly patients with decreased tolerance for aggressive regimens and in patients with poor prognostic features who do not benefit from conventional chemoimmunotherapy regimens. This portion of the NCCN Guidelines for Non-Hodgkin’s Lymphomas describes the recent specific to the incorporation of recently approved targeted therapies for the management of patients with newly diagnosed and relapsed or refractory CLL/SLL.

The treatment landscape of chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) has significantly evolved in recent years. Targeted therapy with Bruton’s tyrosine kinase (BTK) inhibitors and BCL-2 inhibitors has emerged as an effective chemotherapy-free option for patients with previously untreated or relapsed/refractory CLL/SLL. Undetectable minimal residual disease after the end of treatment is emerging as an important predictor of progression-free and overall survival for patients treated with fixed-duration BCL-2 inhibitor-based treatment. These NCCN Guidelines Insights discuss the updates to the NCCN Guidelines for CLL/SLL specific to the use of chemotherapy-free treatment options for patients with treatment-naïve and relapsed/refractory disease.