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The hematologic malignancies consist of a broad group of disorders comprising approximately 10% of new cancer diagnoses each year in the United States. Patients with hematological malignancies experience widely varying clinical trajectories, prognoses, and treatment intensities. Some patients, with chronic or more indolent diseases characteristics may have no symptoms from their disease and can be managed with observation alone. Others, such as those with acute leukemias or aggressive lymphomas, may experience a rapid decline of health at the time of diagnosis and require prolonged hospitalization and intensive therapy to achieve disease control. Moreover, patients with hematological malignancies often suffer from substantial morbidity including bone marrow failure, infections, and transfusion requirements. Despite the recent introduction of novel therapies such as targeted agents and immunotherapy in the treatment of patients with hematological malignancies, mortality remains high, with approximately 56,000 individuals dying each year in the United States from these diseases. There remains a critical need for innovations that optimize the quality of life and care delivery for patients with hematological malignancies.
In recent years, there has been an increasing interest in describing palliative care needs of and overcoming barriers to palliative care integration for patients with hematological malignancies. These barriers include patients, families, and clinicians equating palliative care with end-of-life care, prognostic uncertainty and misconceptions about prognosis, and system-level factors such as lack of palliative care availability and inability to receive transfusions in hospice (see Chapter 37 for an in-depth discussion of these barriers). This chapter outlines the unique trajectory and features of hematological malignancies that lead to the specific palliative care needs of those affected by them. Specific topics include an overview of hematological malignancies, symptom burden, prognostic awareness of patients and families, and patterns of resource utilization, particularly near the end of life.
Hematological malignancies are a remarkably heterogeneous group of diseases, and are rarer in general than most solid tumors. While a thorough review of hematological malignancies is beyond the scope of this chapter, it is important to understand the fundamental differences between types of hematological malignancies, and to recognize how these diseases contrast with more common solid tumors. Unique features of hematological malignancies include at least one of the following: (1) an unpredictable illness course, or wide variability in the range of possible outcomes including the possibility of cure; (2) the need for intensive treatments to achieve a good outcome, which is associated with risk of toxicity and early death; (3) a nontraditional staging system; and (4) in some diseases, a more chronic course punctuated by the intermittent need for active therapy, or a requirement for continuous therapy indefinitely. Following is a brief review of the three major categories of hematological malignancies and their salient features.
Leukemias generally come in two types, acute or chronic. Acute leukemias are characterized by a rapid onset and generally cause significant symptoms or mortality quickly if left untreated. The most common type of acute leukemia in adults is acute myeloid leukemia (AML). Acute leukemias are generally more responsive to chemotherapy compared to many solid tumors. As a result, some acute leukemias can be cured with chemotherapy. However, this typically requires high-dose chemotherapy, which is much more intensive than typical solid tumor regimens. Patients receiving high-dose therapies are hospitalized for several weeks at a time and face the risk of death from toxicity of these treatments. Additionally, sometimes stem cell transplantation follows initial intensive therapy, to increase the chance of cure or to provide a chance at cure in more aggressive or relapsed diseases. Patients receiving high-dose therapy including stem cell transplantation have immense symptoms, quality-of-life impairments, and psychological distress.
Chronic leukemias often have an insidious onset, sometimes even asymptomatic, and their prognosis is usually measured in years, if not decades. The two most common types of chronic leukemias in adults are chronic myeloid leukemia (CML) and chronic lymphoid leukemia (CLL). Patients with CML usually take oral targeted therapy with a tyrosine kinase inhibitor such as imatinib. This leads to a survival that is similar to age-matched population controls with very good health and quality of life. It is only those with atypical cases of CML who might have shortened survival, including treatment-refractory CML with resistance mutations, or in cases where CML transforms into AML. CLL is generally a disease of older age, often identified incidentally on routine bloodwork. CLL is slow growing and may not require treatment initially, if at all. However, it can become very aggressive and life-limiting after an initial period of a more indolent course, or in some cases it may even present as a more aggressive variant. The median survival with CLL is about 10 years, but there is marked clinical heterogeneity in how it impacts each person’s life. Once CLL becomes more aggressive, it is often punctuated by a 1- to 2-year terminal phase, with a risk of transformation into more aggressive lymphoma, which portends a poor prognosis. Patients with CLL also face frequent infectious complications, which are sometimes life-threatening, but are also difficult to predict and are often readily treatable.
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