Leukemia

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.

Two of the largest trials to date on the treatment of chronic lymphocytic leukemia indicate that the combination treatment of fludarabine, cyclophosphamide, and rituximab (Rituxan) should become the new standard of care for both untreated and previously treated patients.

Cephalon, Inc, announced that the US Food and Drug Administration (FDA) has approved injectable bendamustine hydrochloride (Treanda) for the treatment of patients with indolent B-cell non-Hodgkin lymphoma (NHL) that has progressed during or within 6 months of treatment with rituximab (Rituxan) or a rituximab-containing regimen. The data supporting the FDA approval show that bendamustine is effective, has a tolerable side effect profile in patients with indolent NHL, and that treatment results in a high durable response rate. In March of this year, bendamustine received approval for the treatment of patients with chronic lymphocytic leukemia, the most common form of leukemia in the United States.

Results from the multinational randomized phase III REACH trial demonstrate that the combination known as FCR (fludarabine, cyclophosphamide, rituximab [Rituxan]) provides a 10-month improvement in progression-free survival, compared with fludarabine/cyclophosphamide (FC) alone, and a near doubling of complete response rates in patients with relapsed or refractory chronic lymphocytic leukemia (CLL). The study results were presented by lead investigator Tadeusz Robak, md, of the Medical University of Lodz in Poland, at the 50th Annual Meeting of the American Society of Hematology (ASH), held Dec 6–9 in San Francisco (abstract LBA-1).

Results of the first clinical study examining the use of fostamatinib disodium, an investigational treatment that targets a protein called SYK (spleen tyrosine kinase), showed that the new agent represents a safe and novel therapeutic approach that should be further developed for the treatment of B-cell non-Hodgkin lymphoma.

The results of an international randomized trial found that the use of dexamethasone in the induction phase of combination chemotherapy led to a one-third reduction in the risk of relapse as compared with the standard corticosteroid, prednisone, translating into a significant benefit in terms of event-free survival in children with acute lymphoblastic leukemia.

PROPEL, a multicenter phase II open-label study-the largest prospective clinical trial in patients with relapsed or refractory peripheral T-cell lymphoma-found that the investigational chemotherapy agent pralatrexate produces complete responses (disappearance of all signs of cancer) in patients who had previously failed an average of three treatment regimens, including an autologous stem cell transplant for some patients. Designed to be selectively transported into tumor cells, the novel antifolate pralatrexate accumulates to high concentrations in the tumor cell, inhibiting DNA synthesis, said Owen A. O’Connor, md, phd, Columbia University Medical Center, New York, at the ASH meeting (abstract 261).

Results from a multinational phase III clinical trial suggest that fludarabine, cyclophosphamide, and rituximab (FCR) chemoimmunotherapy may become the new standard first-line therapy for the treatment of advanced chronic lymphocytic leukemia.

Results from three studies presented at the ASH meeting showed that treatment with alemtuzumab (Campath) had activity in high-risk chronic lymphocytic leukemia (CLL) patients who have poor prognostic indicators.

Burkitt lymphoma (BL) is a unique B-cell lymphoma characterized by a high proliferation rate and cytogenetic changes related to c-myc proto-oncogene overexpression. Burkitt lymphoma is a highly aggressive B-cell lymphoma that is most frequently seen in children and young adults in endemic areas.

Hodgkin lymphoma (HL) is one of the most curable malignancies in adults. However, survival rates for elderly patients with HL (often defined as ≥ 60 years of age) are inferior to those achieved by younger populations.