Canadian Hematology Today

Front-line Management of Follicular Lymphoma

Thu, 25 Apr 2024 00:00:00 +0000

Follicular lymphoma (FL) is the second most common type of non-Hodgkin lymphoma (NHL) in Western countries. Most patients have an indolent disease course with 10-year survival estimates of 80% among all patients in the rituximab era. However, risk stratification schema can identify subgroups of patients at higher risk of early death and/or progression following front-line therapy. In addition, histologic transformation to an aggressive NHL occurs in approximately 2% of patients per year. Many patients can initially be observed, but ultimately, most will be treated with multiple lines of therapy during their lifetimes. Current Health Canada-approved systemic treatment options include chemoimmunotherapy and lenalidomide plus rituximab. Phosphoinositide 3-kinase (PI3K) inhibitors were initially approved but were later withdrawn because of toxicity considerations. Newer therapies likely to impact care in Canada include bispecific T cell engagers (BiTEs) and chimeric antigen receptor (CAR)-T cell therapy.

Burkitt Lymphoma: The Curable Challenge

Adam J. Olszewski, MD — Thu, 25 Apr 2024 00:00:00 +0000

Among the multiple subtypes of non-Hodgkin lymphoma, Burkitt lymphoma (BL) holds a unique position as the most aggressive mature B-cell malignancy. Named after the British physician who first described rapidly growing jaw and abdominal tumours in Ugandan children in 1958, BL is now understood to be a highly proliferative lymphoma arising from B-cells in the dark-zone germinal centre. BL is driven by the hallmark genomic lesion (IG::MYC rearrangement) and occurs in specific epidemiologic and clinical contexts.

Follicular Non-Hodgkin Lymphoma: First Relapse and Beyond

Mary-Margaret Keating, MD — Thu, 25 Apr 2024 00:00:00 +0000

Follicular lymphoma (FL) is the most common indolent subtype of non-Hodgkin Lymphoma (NHL) and the second most common type of lymphoma overall. In Canada the age‑standardized incidence of FL is 38.3 cases per million individuals per year with mean age at diagnosis of 60 and similar incidence in men and women. Follicular lymphoma is treatable but not curable with systemic therapy yet it maintains a median overall survival (OS) of approximately 20 years. Historically, this long median survival has been maintained through periods of watchful waiting and subsequent treatment with chemoimmunotherapy when the disease burden becomes symptomatic. Serial relapses with progressively shorter remissions and more resistant disease is the usual natural history for FL. The management of relapsed FL remains controversial and the decision on next line of therapy is a rapidly evolving area, with the old standard repetition of chemoimmunotherapy being contested by new targeted therapies. There remains a challenge for Canadian patients to access these novel therapies outside of clinical trials and access programs. This review will present a treatment approach for relapsed FL taking into consideration Canadian funding patterns, in addition to reviewing the novel drugs with the highest level and most mature evidence to date.

Systemic Mastocytosis: Diagnosis and Management in 2024

Stephanie Lee, MD, MSc, FRCPC — Thu, 25 Apr 2024 00:00:00 +0000

Mastocytosis is a group of clonal disorders characterized by an accumulation of neoplastic mast cells (MCs) in one or more organ systems. The clinical presentation of mastocytosis is heterogenous as are the clinical outcomes. For example, some variants are associated with near normal life expectancy, while others are amongst the most aggressive known malignancies. Mastocytosis can occur in both pediatric and adult populations and can be classified into three major groups: systemic mastocytosis (SM), cutaneous mastocytosis (CM), and localized mast cell sarcoma. This review will focus on SM in adults with the aim of providing a general overview of the (1) pathophysiology, (2) diagnostic approach, and (3) current treatment landscape in Canada.

Maintenance Therapy for CD20+ Indolent Lymphoma: Who Should Receive Maintenance?

Edward Koo, MD, David A. Macdonald, MD, FRCPC — Thu, 25 Apr 2024 00:00:00 +0000

Maintenance rituximab (MR) has been a mainstay of treatment in Canada for CD20‑positive indolent lymphoma for two decades. The adoption of MR into clinical practice occurred after the publication of the EORTC 20981 trial. This trial showed a significant improvement in progression free survival (PFS) with two years of MR versus observation after induction therapy with cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP) or rituximab with cyclophosphamide, doxorubicin, vincristine, and prednisone (R-CHOP) in patients with relapsed follicular lymphoma (FL). The use of MR was broadly extended to include its use in the front‑line setting, following any R-containing inductions and including all CD20-positive indolent lymphoma histologies.

Automatic recommendations for MR became the standard practice for most patients. Given the recent changes to standard induction regimens in some indications, and with heightened concerns about infectious complications during B-cell depleting therapy, the recommendation for the use of MR should no longer be considered automatic. This review offers a balanced perspective of the evidence for MR.