34

Management of Patients
With Hematologic
Neoplasms
LEARNING OBJECTIVES

On completion of this chapter, the learner will be able to:

1. Distinguish between hematologic clonal disorders and frank
malignancy.
2. Compare the leukemias in terms of their incidence, physiologic
alterations, clinical manifestations, management, and prognosis.
3. Use the nursing process as a framework for care of patients with
acute leukemia.
4. Compare the myeloproliferative disorders in terms of their incidence,
clinical manifestations, management, complications, and prognosis.
5. Describe nursing management of patients with lymphoma or
multiple myeloma.

GLOSSARY
absolute neutrophil count (ANC): a calculation of the number of
circulating neutrophils, derived from the total white blood cells
(WBCs) and the percentage of neutrophils counted in a microscope’s
visual field
angiogenesis: formation of new blood vessels
apoptosis: programmed cell death
blast cell: primitive leukocyte

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clone: proliferation from same cell of origin so that descendent cells
are identical to the cell of origin
cytokines: proteins produced by leukocytes that are vital to regulation
of hematopoiesis, apoptosis, and immune responses
erythrocyte sedimentation rate (ESR): laboratory test that measures
the rate of settling of red blood cells (RBCs); elevation is indicative
of inflammation; also called the sed rate
granulocyte: granulated WBC (neutrophil, eosinophil, basophil); term
sometimes used as synonymous with neutrophil
hematopoiesis: complex process of the formation and maturation of
blood cells
indolent neoplasm: a slow-growing cancer that often remains
localized or causes few symptoms
leukocyte: one of several cellular components of blood involved in
defense of the body; subtypes include neutrophils, eosinophils,
basophils, monocytes, and lymphocytes (synonym: white blood cell
[WBC])
leukemia: uncontrolled proliferation of WBCs, often immature
leukopenia: less-than-normal amount of WBCs in circulation
lymphadenopathy: enlargement of a lymph node or lymph nodes
lymphocyte: type of WBC involved in immune functions
lymphoid: pertaining to lymphocytes
lysis: destruction of cells
monocyte: large WBC that becomes a macrophage when it leaves the
circulation and moves into body tissues
myeloid: pertaining to nonlymphoid blood cells that differentiate into
RBCs, platelets, macrophages, mast cells, and various WBCs
neutropenia: lower-than-normal number of neutrophils
neutrophil: fully mature WBC capable of phagocytosis; primary
defense against bacterial infection
pancytopenia: abnormal decrease in WBCs, RBCs, and platelets
petechiae: tiny capillary hemorrhages
phagocytosis: process of cellular ingestion and digestion of foreign
bodies
red blood cell (RBC): a cellular component of blood involved in the
transport of oxygen and carbon dioxide (synonym: erythrocyte)
reticulocytes: slightly immature RBCs, usually only 1% of total
circulating RBCs

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 splenomegaly: enlargement of the spleen
stem cell: primitive cell, capable of self-replication and differentiation
into myeloid or lymphoid stem cell
thrombocytopenia: lower-than-normal platelet count
thrombocytosis: higher-than-normal platelet count
white blood cell (WBC): synonym: leukocyte

Hematopoiesis is characterized by a rapid, continuous turnover of blood

cells. Normally, the production of specific blood cells from their stem cell
precursors is carefully regulated according to the body’s needs. If the
mechanisms that control the production of these cells are disrupted, the
cells can proliferate excessively, as seen in the development of
hematologic neoplasms. As is true with nonmalignant hematologic
disorders, the pathophysiologic processes that undergird the development
of hematologic neoplasms are complex. Understanding these processes
and the rationale for treatments is important so that nurses may
appropriately assess, monitor, educate, and intervene with patients with
hematologic neoplasms.

Figure 34-1 • The development of myeloid

neoplasms. Changes within the myeloid stem
cell can cause the development of neoplasms
that are either proliferative or, in the case of
myelodysplastic syndrome, dysplastic.
Although any indolent myeloid neoplasm can
evolve into a malignant condition (e.g., acute
myeloid leukemia [AML]), in most instances

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this will not occur. For example, the incidence
of evolution from primary thrombocythemia
is very low, yet it is much higher when from
primary myelofibrosis. The actual incidence
of evolution continues to change as therapy
before more effective, as in the case of
chronic myeloid leukemia. Note that AML
can arise directly from the altered myeloid
stem cell; it need not evolve from an existing
indolent neoplasm. Similar events occur with
lymphoid malignancy development.

Hematopoietic malignancies are often classified by the cells involved.

Leukemia is a neoplastic proliferation of a particular cell type
(granulocytes, lymphocytes, or infrequently erythrocytes or
megakaryocytes). The defect originates in the hematopoietic stem cell, the
myeloid, or the lymphoid stem cell. Lymphomas are neoplasms of
lymphoid tissue, usually derived from B lymphocytes. Multiple myeloma
is a malignancy of the most mature form of B lymphocyte—the plasma
cell.

CLONAL STEM CELL DISORDERS

When some hematologic neoplasms develop, hematopoietic control
mechanisms may be in place to continue to produce adequate numbers of
normal blood cells. These are commonly referred to as indolent
neoplasms, where the increased numbers of cells produced from a culprit
clone all have the same genotype (see Chapter 8 for further discussion of
genotypes). However, at some time, the control mechanisms may fail and
the “indolent” clone may then evolve into a more aggressive clone.
Not all malignancies arise from an indolent neoplasm, however. Rather,
they may evolve directly from changes in the stem cell. Similarly, not all
indolent neoplasms will eventually evolve into a malignancy. Nonetheless,
such evolution is possible for virtually any clonal disorder. Figure 34-1
illustrates this concept for myeloid stem cell disorders. Similar neoplastic
conditions are derived from disorders within the lymphoid stem cell
disorder (Shlush & Minden, 2015). Refer to later discussions about
specific diseases noted in Figure 34-1.

LEUKEMIA
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The term leukocytosis refers to an increased level of leukocytes (white
blood cells [WBCs]) in the circulation. Typically, only one specific cell
type is increased. Because the proportions of several types of leukocytes
(e.g., eosinophils, basophils, monocytes) are small, an increase in other
types can be great enough to elevate the total leukocyte count, particularly
the neutrophil or lymphocyte. Although leukocytosis can be a normal
response to increased need (e.g., in acute infection), the elevation in
leukocytes should decrease as the physiologic need decreases. A prolonged
or progressively increasing elevation in leukocytes is abnormal and should
be evaluated. A significant cause of persistent leukocytosis is hematologic
malignancy (i.e., leukemia).
The common feature of the leukemias is an unregulated proliferation of
leukocytes in the bone marrow. In acute forms (or late stages of chronic
forms), the proliferation of leukemic cells leaves little room for normal cell
production. There can also be a proliferation of cells in the liver and spleen
(extramedullary hematopoiesis). With acute forms, there can be infiltration
of leukemic cells in other organs, such as the meninges, lymph nodes,
gums, and skin. The cause of leukemia is not fully known, but exposure to
radiation or chemicals, certain genetic disorders, and viral infections are
known to be risk factors for certain types of leukemia. Bone marrow
damage from pelvic radiation or certain types of chemotherapy drugs can
cause acute leukemia, typically occurring years after treatment for another
malignancy (Leukemia & Lymphoma Society, 2015a).
The leukemias are commonly classified according to the stem cell line
involved, either lymphoid (referring to stem cells that produce
lymphocytes) or myeloid (referring to stem cells that produce
nonlymphoid blood cells). They are also classified as either acute or
chronic, based on the time it takes for symptoms to evolve and the phase
of cell development that is halted (i.e., with few leukocytes differentiating
beyond that phase).
In acute leukemia, the onset of symptoms is abrupt, often occurring
within a few weeks. Leukocyte development is halted at the blast phase,
and thus most leukocytes are undifferentiated cells or blasts. Acute
leukemia can progress rapidly, with death occurring within weeks to
months without aggressive treatment.
In chronic leukemia, symptoms evolve over a period of months to years,
and the majority of leukocytes produced are mature. Chronic leukemia
progresses more slowly; the disease trajectory can extend for years.

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Acute Myeloid Leukemia
Acute myeloid leukemia (AML) results from a defect in the hematopoietic
stem cell that differentiates into all myeloid cells: monocytes,
granulocytes (e.g., neutrophils, basophils, eosinophils), erythrocytes, and
platelets. Any age group can be affected, although it infrequently occurs
before age 45, and the incidence rises with age, with a peak incidence at
age 67 (American Cancer Society [ACS], 2014a). AML is the most
common non-lymphocytic leukemia.
The prognosis and survival rates are highly variable. Factors influencing
a more positive outcome are younger age at diagnosis, more favorable
cytogenetic alterations (which are strongly associated with younger age),
and few concurrent (or mild) health problems. In contrast, individuals with
significant comorbidities, of older age, with cytogenetic features deemed
to be adverse, or who are frail, are more likely to have a poor prognosis.
AML evolving from a preexisting clonal myeloid disease or from prior
cytotoxic therapy for another malignancy or immune disease is also
associated with a poorer prognosis (Liesveld & Lichtman, 2016).
Prior to the development of chemotherapy treatment for AML, the
median survival rate was only 6 weeks. While platelet transfusion support
and aggressive chemotherapy have markedly improved these statistics,
survival rates remain suboptimal. Data from the Surveillance,
Epidemiology, and End Results (SEER) Program of the National Cancer
Institute established 5-year survival rates of 56% for those under age 45,
39% for those aged 45 to 54, 27% for those aged 55 to 64, 11% for those
between 65 and 74 years old, and less than 2% for those over age 75
(Liesveld & Lichtman, 2016). Despite these poor statistics, cure is possible
for some individuals, either with stem cell transplant or with
chemotherapy. Death usually is due to infection or bleeding.

Clinical Manifestations
AML develops without warning, with symptoms typically occurring over a
period of weeks. Signs and symptoms result from insufficient production
of normal blood cells. Fever and infection result from neutropenia (low
neutrophil count); weakness and fatigue, dyspnea on exertion, and pallor
from anemia; and petechiae, ecchymoses, and bleeding tendencies from
thrombocytopenia. The proliferation of leukemic cells within organs leads
to a variety of additional symptoms: pain from an enlarged liver or spleen,

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hyperplasia of the gums, and bone pain from expansion of marrow (see
Fig. 34-2). Petechiae (pinpoint red or purple hemorrhagic spots on the
skin) or ecchymoses (bruises) are common on the skin (see Fig. 33-4 in
Chapter 33); occasionally, leukemic infiltrates are also seen (see Fig. 34-
3). Leukemic cells can also infiltrate the gingiva or synovial spaces of
joints. Lymphadenopathy (enlargement of lymph nodes) or
splenomegaly (enlargement of the spleen) is rare. Fevers may occur and
are not always due to infection.

Assessment and Diagnostic Findings

The complete blood count (CBC) shows a decrease in both erythrocytes
and platelets. Although the total leukocyte count can be low, normal, or
high, the percentage of normal cells is usually vastly decreased. A bone
marrow analysis shows an excess (more than 20%) of immature
leukocytes, called blast cells; this is the hallmark of the diagnosis. AML
can be further classified into seven different subgroups, based on
cytogenetics, histology, and morphology of the blasts. The actual
prognosis varies somewhat between subgroups and with the extent of
cytogenetic abnormalities and genetic mutations, yet the clinical course
and treatment differ substantially with only one subtype. Patients with the
specific AML subtype acute promyelocytic leukemia (APL, or AML-M3)
have more potentially fatal bleeding episodes, because they have
underlying coagulopathy and a higher incidence of disseminated
intravascular coagulation (DIC); however, the potential to cure this form
of AML is high (Coombs, Tavakkoli, & Tallman, 2015).

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Figure 34-2 • Gingival infiltration of
leukemic cells in a patient with acute myeloid
leukemia. From Greer, J. P., Foerster, J.,
Rodgers, G. M., et al. (2009). Wintrobe’s
clinical hematology (12th ed., Fig. 72.8, p.
1680). Philadelphia, PA: Lippincott Williams
& Wilkins.

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Figure 34-3 • Leukemia cutis. Infiltration
of leukemic cells in skin on extensor surface
of forearms. Reproduced with permission
from Stedman’s Medical Dictionary.
Copyright 2008 Lippincott Williams &
Wilkins.

Medical Management
Despite advances in understanding of the biology of AML, substantive
advances in treatment response rates and survival rates have not occurred
for decades, with the exception of advances made in treating APL (see
later discussion). Even for patients with AML subtypes that have not
benefited from significant advances in treatment, cure is still possible. The
overall objective of treatment is to achieve complete remission, in which
there is no evidence of residual leukemia in the bone marrow. Attempts are
made to achieve remission by the aggressive administration of
chemotherapy, called induction therapy, which usually requires

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hospitalization for several weeks. Induction therapy typically involves
high doses of cytarabine (Cytosar, Ara-C) and either daunorubicin
(Cerubidine), idarubicin (Idamycin), or mitoxantrone (Novantrone);
etoposide (VP-16, VePesid) is occasionally added to the regimen. The
choice of agents is based on the patient’s physical status and history of
prior antineoplastic treatment. Older patients (especially those older than
70 years) tend to not tolerate standard therapy. Lower-intensity therapy
(using hypomethylating agents, low doses of cytarabine, or hydroxyurea)
may extend survival somewhat without a significant increase in toxicity
beyond that of the underlying disease (Erba, 2015).
Treatment of APL revolves around induction therapy using the
differentiating agent all-trans retinoic acid (ATRA), which induces the
promyelocytic blast cells to differentiate, thereby deterring the blasts from
proliferating at an immature stage. ATRA is typically combined with a
formulation of arsenic (arsenic trioxide) and, in those deemed to be at high
risk for relapse, a conventional anthracycline drug. This regimen yields a
very high response rate, and cure is possible (Coombs et al., 2015).
In AML, the aim of induction therapy is to eradicate the leukemic cells;
however, this is also accompanied by the eradication of normal types of
myeloid cells. Thus, the patient becomes severely neutropenic; an
absolute neutrophil count (ANC; a precise calculation of the number of
circulating neutrophils) of 0 is not uncommon. Anemia, and severe
thrombocytopenia (a platelet count of less than 5,000/mm3), is also
common. During this time, the patient is typically very ill, with bacterial,
fungal, and occasionally viral infections; bleeding; and severe mucositis,
which causes pain, diarrhea, and an inability to maintain adequate
nutrition. Management consists of administering blood products (packed
red blood cells and platelets) and promptly treating infections. The use of
granulocytic growth factors, either granulocyte colony-stimulating factor
(G-CSF; filgrastim [Neupogen]) or granulocyte-macrophage colony-
stimulating factor (GM-CSF; sargramostim [Leukine]), can shorten the
period of significant neutropenia by stimulating the bone marrow to
produce leukocytes more quickly; these agents do not appear to increase
the risk of producing more leukemic cells (Kam, Yiu, Loh, et al., 2015).
When the patient has recovered from the induction therapy (i.e., the
neutrophil and platelet counts have returned to normal and any infection
has resolved), consolidation therapy is given to eliminate any residual
leukemia cells that are not clinically detectable and reduce the chance for

2623
recurrence. Multiple treatment cycles of various agents are used, usually
containing some form of cytarabine. Frequently, the patient receives one
cycle of treatment that is almost the same as, if not identical to, the
induction treatment but at somewhat lower dosages. Because the amount
of leukemia cells is dramatically reduced at this point, toxicity associated
with therapy is less (Schiffer, 2014).
Another aggressive treatment option is hematopoietic stem cell
transplantation (HSCT). When a suitable tissue match can be obtained, the
patient embarks on an even more aggressive regimen of chemotherapy
(sometimes in combination with radiation therapy), with the treatment goal
of destroying the hematopoietic function of the patient’s bone marrow.
The patient is then “rescued” with the infusion of the donor stem cells to
reinitiate blood cell production. Patients who undergo HSCT have a
significant risk of infection and graft-versus-host disease (where the
donor’s lymphocytes [graft] recognize the patient’s body as “foreign” and
set up reactions to attack the “foreign” host, i.e., the patient). The most
appropriate use and timing of HSCT remain unclear. Patients with a poorer
prognosis may benefit from early HSCT; those with a good prognosis may
not need transplant at all. See Chapter 15 for a discussion of nursing
management in HSCT.
Another important option for the patient to consider is supportive care
alone. In fact, supportive care may be the only option if the patient has
significant comorbidity, such as extremely poor cardiac, pulmonary, renal,
or hepatic function; is older and frail; or both. In such cases, aggressive
antileukemia therapy is not used; occasionally, hydroxyurea (Hydrea) or
hypomethylating agents such as azacitidine (Vidaza) may be used briefly
to control the increase of blast cells. Patients are more commonly
supported with antimicrobial therapy and transfusions as needed. This
treatment approach provides the patient with some additional time outside
the hospital; however, death frequently occurs within months, typically
from infection or bleeding. Refer to Chapter 16 for a discussion of end-of-
life care.

Complications
Complications of AML include bleeding and infection, which are the
major causes of death. The risk of bleeding correlates with the level and
duration of platelet deficiency (thrombocytopenia). The low platelet
count can cause ecchymoses and petechiae. Major hemorrhages also may

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