Leukemia

Peripheral T-cell lymphomas (PTCLs) are uncommonly encountered malignancies in the United States, and hepatosplenic T-cell lymphoma (HSTCL), subcutaneous panniculitis-like T-cell lymphoma (SPTCL), and enteropathy-type T-cell lymphoma (ETTCL) are rare subtypes of PTCLs that often present with primarily extranodal disease. Despite the fact that these tumors have distinct clinical and pathologic features, they are often diagnosed after significant delay. The combination of delay in diagnosis with ineffective therapies has resulted in a poor prognosis in most cases. Techniques that identify T-cell receptor gene rearrangements and flow cytometry that can identify characteristic immunophenotypes have guided our understanding of the underlying cell of origin of these rare PTCLs. As knowledge regarding the biology of these lymphomas increases alongside the development of newer therapeutics with novel mechanisms, clinicians must accordingly improve their familiarity with the clinical settings in which these rare malignancies arise as well as the pathologic features that make them unique

NEW ORLEANS-Genentech and Biogen announced at ASH 2009 that the three-year follow-up of the CLL8 trial demonstrated that rituximab (Rituxan) plus fludarabine and cyclophosphamide (FC) chemotherapy improved overall survival in patients with previously untreated chronic lymphocytic leukemia (CLL) vs FC therapy alone.

In this issue of ONCOLOGY, Dr. Tobinai presents a thorough and thoughtful review of the current state of the art of HTLV-related adult T-cell leukemia/lymphoma (ATLL). As described, ATLL is most prevalent in Asia, where it has also been most studied, but is also seen in patients from other HTLV-endemic areas including the Caribbean, South America, and parts of Africa. ATLL is rare in North America and Europe, representing 1% to 2% of T-cell lymphomas compared to 25% in Asia.[1]

In this issue of ONCOLOGY, Tobinai reviews the management of human T-cell lymphotropic virus type 1 (HTLV-1)–associated adult T-cell leukemia/lymphoma (ATL). Although rare in the United States, an estimated 10 to 20 million people are infected with HTLV-1 worldwide and 2% to 5% will develop ATL.[1]

Sunesis Pharmaceuticals, Inc, announced that the US Food and Drug Administration has granted voreloxin orphan drug designation for the treatment of acute myeloid leukemia (AML). Sunesis is currently conducting two phase II clinical trials of voreloxin in AML: a single-agent study (REVEAL-1) in newly diagnosed elderly AML patients unlikely to benefit from standard induction chemotherapy and a study evaluating the drug in combination with cytarabine in relapsed/refractory AML.

Adult T-cell leukemia/lymphoma (ATL) is defined as a histologically or cytologically proven peripheral T-cell malignancy associated with a retrovirus, human T-cell lymphotropic virus type I (HTLV-1).[1] Southwestern Japan is the district with the highest prevalence of HTLV-1 infection and the highest incidence of ATL in the world. A high prevalence of HTLV-1 infection is also found in the Caribbean islands, tropical Africa, South America, and northern Oceania.

The purpose of this review is to familiarize oncologists with the clinical and pathologic features of this relatively rare disease spectrum. This should enable appropriate clinical management and reassurance of patients concerned about their prognosis.

Our ability to treat patients with B-cell lymphomas has improved dramatically over the past few decades. Today the majority of patients with diffuse large B-cell lymphoma are cured, the survival of patients with low-grade follicular lymphoma is improving (ie, some estimates have the average survival more than doubling), most patients with Hodgkin lymphoma (also a B-cell lymphoma) are cured, most patients with Burkitt lymphoma are cured, and our ability to diagnose and treat patients with the various marginal zone lymphomas has improved considerably.

Allos Therapeutics has received accelerated FDA approval for Folotyn (pralatrexate) as a single-agent treatment in patients with relapsed or refractory peripheral T-cell lymphoma.

One of the greatest challenges facing the physician caring for patients with chronic lymphocytic leukemia (CLL) is the heterogeneity of this disease. Over the past decade, there have been major advances in understanding the pathophysiology of CLL, and in the identification of biomarkers that are helpful to predict the clinical course for individual patients. Over the same period, the available therapeutic options have developed dramatically, exemplified by the introduction of combination therapy with purine analogs and monoclonal antibodies, resulting in significant opportunities to induce complete remission (CR) in CLL patients.

Chronic lymphocytic leukemia (CLL) is a heterogeneous disease with an extremely variable course. Survival after diagnosis can range from months to decades. As the pathogenesis of the disease is increasingly understood, we begin to unfold the molecular patterns that define the different prognostic subgroups and to develop strategies to predict the clinical course.

Chronic lymphocytic leukemia (CLL) is the most common form of adult leukemia in the Western hemisphere. Both the Rai and Binet staging systems have been important clinical tools for predicting outcomes of this heterogeneous disease.

Lymphoblastic lymphoma (LBL) is a rare disease, comprising about 2% of all non-Hodgkin lymphomas (NHLs) in adults.[1] It is a highly aggressive subtype of lymphoma, most commonly of precursor T-cell origin, occurring most frequently in adolescents and young adults, with male predominance and frequent mediastinal, bone marrow, and central nervous system (CNS) involvement.

The US Food and Drug Administration approved ofatumumab (Arzerra) for patients with chronic lymphocytic leukemia (CLL) whose cancer is no longer being controlled by other forms of chemotherapy. The product was approved under the FDA’s accelerated approval process, which allows earlier approval of drugs that meet unmet medical needs.

Lymphoblastic lymphoma (LBL) is a rare disease, most commonly of T-cell origin, that shares biologic features with acute lymphoblastic leukemia (ALL). Indeed, LBL and ALL are considered a single entity (lymphoblastic leukemia/lymphoma, T and B types) in the World Health Organization (WHO) classification of precursor lymphoid neoplasms.

The US Food and Drug Administration (FDA) has granted accelerated approval for pralatrexate injection (Folotyn) for use as a single agent for the treatment of patients with relapsed or refractory peripheral T-cell lymphoma (PTCL), which comprises a biologically diverse group of aggressive blood cancers.

Data from a pivotal phase III study demonstrate that bendamustine (Treanda) improved clinical outcomes when compared to chlorambucil (Leukeran) in patients with chronic lymphocytic leukemia (CLL).

A large, population-based study of the association between venous thromboembolism and mortality in hematologic malignancies found an increased risk of death in patients with acute lymphoblastic leukemia, but not in those with acute myelogenous leukemia. The authors had no explanation for the differential association between the two types of acute leukemia.

Patients with chronic myeloid leukemia can learn more about the disease at a new educational Web portal.

Mantle cell lymphoma (MCL) is a distinct subtype of non-Hodgkin lymphoma that occurs most commonly in older patients with advanced-stage disease.

On August 22, 2008, the US Food and Drug Administration (FDA) granted marketing approval (licensure) to romiplostim (Nplate, Amgen Inc) for the treatment of thrombocytopenia in patients with chronic immune (idiopathic) thrombocytopenic purpura (ITP) who have had an insufficient response to corticosteroids, immunoglobulins, or splenectomy.

Drs. Hernandez-Ilizaliturri and Czuczman should be complimented on their comprehensive review of management options for patients with relapsed and refractory diffuse large B-cell lymphoma (DLBCL).

A triple therapy with fludarabine, cyclophosphamide, and rituximab (Rituxan) was hailed as the new standard of care for chronic lymphocytic leukemia at ASH 2008 in San Francisco. Now a new study has deemed low-dose fludarabine and cyclophosphamide combined with high-dose rituximab (FCR-Lite) as highly effective in untreated CLL patients.

SAN FRANCISCO-While genetics has opened up new possibilities for predicting treatment response in patients with acute myeloid leukemia, there are still unanswered questions about the relationship between genetic mutations and treatment outcome, according to Bob Lowenberg, MD, PhD, who delivered the Ham-Wasserman lecture at ASH 200

SAN FRANCISCO-As a single agent, pralatrexate (PDX) shows promising clinical activity in relapsed or refractory peripheral T-cell lymphoma, producing responses in 27% of the patients enrolled in a major prospective clinical trial.