Thrombocytopenia

• a reduction of platelets below 150,000/μL (150 × 109/L).

• Acute, severe, or prolonged decreases from this normal range can result in abnormal hemostasis
that presents as prolonged bleeding from minor trauma or spontaneous bleeding without
injury.
• Platelet disorders: While they can be inherited (e.g., Wiskott-Aldrich syndrome), most are acquired.
• Common cause of acquired disorders is the ingestion of certain drugs. Although some drugs are
directly myelosuppressive (e.g., chemotherapy, ganciclovir), the usual mechanism of drug-related
thrombocytopenia is accelerated platelet destruction caused by antibodies.
• Aspirin doses as low as 81 mg can alter platelet aggregation.
• Normal function is restored when new platelets are made.
◦ Immune Thrombocytopenia (ITP):
‣ most common acquired thrombocytopenia, is an abnormal destruction of circulating
platelets
‣ platelets are coated with antibodies.
‣ These platelets function normally; however, when they reach the spleen, these antibody-
coated platelets are mistaken as foreign and destroyed by macrophages.
‣ autoimmune disease (such as SLE) or infection, such as Helicobacter pylori or viral
infection (HIV), contributes to this disorder
‣ In ITP platelet survival is shortened (less than 8-10 days)
‣ the syndrome presents as an acute condition in children and a chronic condition in adults.
◦ Thrombotic Thrombocytopenic Purpura
‣ uncommon syndrome characterized by hemolytic anemia, thrombocytopenia, neurologic
abnormalities, fever (in the absence of infection), and renal abnormalities.
‣ associated with hemolytic-uremic syndrome (HUS)
‣ caused by the deficiency of a plasma enzyme (ADAMTS13) that breaks down the von
Willebrand clotting factor (vWF) into normal size. Without the enzyme, unusually large
amounts of vWF attach to activated platelets, promoting platelet aggregation.
‣ Occurs primarily in healthy adults with a slight female predominance
‣ thought to be due to an autoimmune disorder against ADAMTS13) or due to certain
drug toxicities (e.g., chemotherapy, cyclosporine, quinine, oral contraceptives,
valacyclovir, clopidogrel [Plavix]), pregnancy or preeclampsia, infection, or a known
autoimmune disorder, such as SLE or scleroderma.3
‣ TTP is a medical emergency because bleeding and clotting occur at the same time.
◦ Heparin-Induced Thrombocytopenia
‣ patients develop thrombocytopenia 5 to 10 days after the onset of heparin therapy.
‣ HIT should be suspected if the platelet count falls by more than 50% or falls to below
150,000/μL.
‣ Major Problem is venous thrombosis
‣ Complications: thromboses (DVT, pulmonary emboli), arterial vascular infarcts,
resulting in skin necrosis, stroke, and end organ damage
‣ Bleeding is unusual
• Clinical Manifestations
◦ Many patients are asymptomatic
◦ Most common symptom is bleeding usually mucosal (nosebleeds, gingival bleeding) or
cutaneous
◦ Large bullous hemorrhages may appear on the buccal mucosa
◦ Bleeding into the skin is seen as petechiae, purpura, or superficial ecchymoses.
◦ Petechiae are small, flat, red or reddish brown microhemorrhages (caused when platelet count
 is low, RBCs may leak out of the blood
vessels and into the skin)
◦ Many petechiae can cause reddish skin
purpura
◦ Purplish lesions caused by hemorrhage are
termed ecchymoses.
◦ Pain and tenderness are sometimes
present.
◦ Major complication of thrombocytopenia
is hemorrhage.
◦ The hemorrhage may be insidious or acute
and internal or external. It may occur in
any area of the body, including the joints,
retina, and brain.
◦ Cerebral hemorrhage may be fatal.
◦ Insidious hemorrhage may be first
detected by discovering the anemia that
accompanies blood loss.
◦ Manifestations of internal blood loss:
weakness, fainting, dizziness, tachycardia,
abdominal pain, and hypotension.
◦ Prolonged bleeding after routine procedures, such as venipuncture or IM injection indicate
thrombocytopenia
◦ symptoms of vascular ischemic problems may occur (subtle confusion, headache, or seizures
and coma)
• Diagnostic Studies
◦ prolonged bleeding from trauma or injury does not usually occur until platelet counts are less
than 50,000/μL
◦ Below 20,000 life threatening hemorrhages can occur
◦ Transfusions generally not recommended until the count is below 10,000 unless actively
bleeding
◦ Laboratory tests that assess secondary hemostasis or coagulation, such as the prothrombin time
(PT) and aPTT, can be normal even in severe thrombocytopenia. If they are increased, this may
point toward DIC.
◦ ITP antigen-specific assay, platelet activation/function assay, or PF4-heparin complex for HIT,
can be done to assist with the diagnosis.
◦ Bone marrow examination may be done to rule out production problems as the cause of
thrombocytopenia (e.g., leukemia, aplastic anemia, other myeloproliferative disorders) or when
other tests are inconclusive. megakaryocytes on bone marrow biopsy is consistent with
thrombocytopenia
• Interprofessional Care
◦ should avoid aspirin and other drugs that affect platelet function or production
◦ Immune Thrombocytopenic Purpura
‣ Corticosteroids (e.g., prednisone, methylprednisolone) are used initially to treat ITP
because of their ability to suppress the phagocytic response of splenic macrophages. This
increases the life span of the platelets. Corticosteroids depress antibody formation and
reduce capillary leakage.
‣ High doses of IV immunoglobulin and a component of IVIG, anti-Rh0(D) may be used in
patients who are unresponsive to corticosteroids or splenectomy
‣ Rituximab (Rituxan) may be used for its ability to lyse activated B cells, thus reducing the
 immune recognition of platelets.
‣ Romiplostim (Nplate) and eltrombopag (Promacta) are used for patients with chronic ITP
who had an insufficient response to the other treatments.
‣ Splenectomy may be needed if the patient does not respond to treatment. (Effectiveness:
spleen normally sequesters around one third of the platelets, so its removal increases the
number of platelets in circulation).
‣ Immunosuppressive therapy (cyclosporine) may be used in refractory cases
‣ Platelet transfusions in life threatening hemorrhage
‣ Platelets should be given prophylactically because of risk for antibody formation
‣ Epsilon-aminocaproic acid (EACA, Amicar), an antifibrinolytic agent, may treat severe
bleeding
◦ Thrombotic Thrombocytopenic Purpura
‣ First should treat underlying disorder or remove the causative agent
‣ If untreated can result in irreversible renal failure and death
‣ Plasma exchange (plasmapheresis) can aggressively reverse platelet consumption by
supplying the appropriate vWF and enzyme (ADAMTS13) and removing the large vWF
molecules that bind with platelets. Should be continued daily until PLT normalize.
Corticosteroids can be accompanied.
‣ Rituximab is used for patients who are refractory to plasma exchange.
‣ Immunosuppressants: cyclosporine or cyclophosphamide
‣ Splenectomy
‣ Platelet administration is contraindicated because of risk of clotting
◦ Heparin-Induced Thrombocytopenia
‣ Heparin & low molecular heparin must be stopped/ should never receive again
‣ Fondaparinux (Arixtra), a factor Xa inhibitor (indirect thrombin inhibitor), or bivalirudin
(a synthetic thrombin inhibitor) may be used
‣ Warfarin should be started only when the platelet count has reached 150,000/μL.
‣ If clotting is severe: plasmapheresis to clear the platelet-aggregating IgG from the blood,
protamine sulfate to interrupt the circulating heparin, thrombolytic agents to treat the
thromboembolic events, and surgery to remove clots.
‣ Platelet transfusion not effective
◦ Thrombocytopenia from decreased Platelet Production
‣ Corticosteroids
‣ Platelet transfusions
• Nursing Management
◦ Nursing Assessment: subjective and
objective data should be obtained
◦ Diagnoses: risk for bleeding
◦ Planning: 1) have no bleeding, (2) maintain
vascular integrity, and (3) manage home care
to prevent any complications
◦ Nursing Implementation:
‣ Health Promotion: Discourage the use of
over the counter medications. Aspirin
contributes to bleeding. Observe for
early signs of thrombocytopenia in the
patient receiving cancer chemotherapy
drugs.
‣ Acute Care: bleeding is usually from
superficial sites. Deep bleeding (into
 muscles, joints, and abdomen) usually occurs only when clotting factors are decreased.
Stress that a
minor nosebleed
or new petechiae
may indicate
potential
hemorrhage and
to notify the HCP.
Avoid IM injections.
Help the patient
understand the
importance of
adhering to self-
care measures that
reduce the risk for
bleeding. Menstrual
blood loss may
exceed the usual
amount and
duration. Counting
sanitary napkins
used during menses
is another
important intervention to detect excess blood loss. Monitor the platelet count, coagulation
studies, hemoglobin, and hematocrit.
‣ Ambulatory Care: If the patient cannot avoid causative agents (e.g., chemotherapy), teach
them to avoid injury or trauma during these periods and to be aware of the signs and
symptoms of bleeding
◦ Evaluation: Have no evidence of bleeding or bruising, State needed knowledge and skills to
manage disease process

Hemophilia and Von Willebrand Disease

◦ Hemophilia is an X-linked recessive genetic disorder caused by a defective or deficient
coagulation factor.
◦ Hemophilia A (classic hemophilia, factor VIII deficiency, more common)
‣ acquired hemophilia A, which is due to the development of antibodies against the body’s
own factor VIII.
◦ Hemophilia B (Christmas disease, factor IX deficiency, common congenital bleeding disroder)
◦ von Willebrand disease is a related disorder involving a deficiency of the von Willebrand
coagulation protein
• Clinical Manifestations and Complications
◦ All manifestations relate to bleeding which may lead to life threatining hemorrhage
◦ 1) slow, persistent, prolonged bleeding from minor trauma and small cuts; (2) delayed bleeding
after minor injuries (the delay may be several hours or days)
◦ (3) uncontrollable hemorrhage after dental extractions or irritation of the gingiva with a hard-
bristle toothbrush;
◦ (4) nosebleeds, especially after a blow to the face;
◦ (5) GI bleeding from ulcers and gastritis;
◦ (6) hematuria and potential renal failure from GU trauma and splenic rupture resulting from
falls or abdominal trauma;
 ◦ (7) bruising and subcutaneous hematomas and possible compartment syndrome
◦ (8) neurologic signs, such as pain, anesthesia, and paralysis, which may develop from nerve
compression caused by hematoma formation
◦ (9) hemarthrosis (bleeding into the joints)
• Diagnostic Studies
◦

• Interprofessional Care:
◦ For mild hemophilia A and certain subtypes of von Willebrand disease, desmopressin
acetate (DDAVP), a synthetic
analog of vasopressin, may be
used to stimulate an increase in
factor VIII and vWF (can be
given IV, subcutaneously, or by
intranasal spray). Beneficial
effects (e.g., decreased
bleeding time) of DDAVP when
given IV are seen within 30
minutes and can last for more
than 12 hours. Patient must be
monitored closely and repeated
doses may be needed.
Appropriate for minor bleeding
episodes and dental procedures
◦ Antifibrinolytic therapy (tranexamic acid [Cyklokapron] and EACA) inhibits fibrinolysis by
inhibiting plasminogen activation in the fibrin clot, thus enhancing clot stability. (may be used
in oral cavity, nosebleeds and menorrhagia)
◦ Complications of the treatment of hemophilia include development of inhibitors to factors VIII
or IX, transfusion-transmitted infectious disorders (hepatitis, HIV), allergic reactions, and
thrombotic complications with the use of factor IX because it contains activated coagulation
factors
◦ Most common problems with acute management are starting factor replacement therapy too
late and stopping it too soon. Minor bleeding episodes should be treated for at least 72 hours

• Nursing Implementation
◦ Health promotion: hereditary nature of hemophilia, referral of affected persons for genetic
counseling before reproduction is an important measure
◦ Acute Care: Stop the topical bleeding as quickly as possible. Apply direct pressure or ice, pack
the area with Gelfoam or fibrin foam, and apply topical hemostatic agents. Give the specific
 coagulation factor and monitor for hypersensitivity. When joint bleeding occurs, in addition to
giving replacement factors, it is important to use the “RICE” protocol. Do not give aspirin. No
weight bearing. Manage any life-threatening complications that may develop.
◦ Ambulatory Care: Immediate medical attention is needed for severe pain or swelling of a
muscle or joint that restricts movement or inhibits sleep and for a head injury, swelling in the
neck or mouth, abdominal pain, hematuria, melena, and skin wounds in need of suturing. Daily
oral hygiene. Take part only in noncontact sports (e.g., golf) and to wear gloves when doing
household chores to prevent cuts or abrasions from knives.

Disseminated Intravascular Coagulation

• a serious bleeding and thrombotic disorder that results
from abnormally initiated and accelerated clotting. May
lead to uncontrollable hemorrhage.
◦ Etiology and Pathophysiology
‣ abnormal response of the normal clotting
cascade stimulated by a disease process or
disorder
‣ can occur as an acute, catastrophic condition,
or it may exist at a subacute or chronic level.
‣ Each condition may have 1 or multiple
triggering mechanisms to start the clotting
cascade.
‣ Tissue factor released at the site of tissue
injury by some cancer and enhances normal
coagulation mechanisms--->abundant
intravascular thrombin is made---> catalyzes
conversion of fibrinogen to fibrin & enhances
platelet aggregation----> results in
thrombosis--> can result in multiple organ
failure. Antithrombotic III and protein C are
depressed.
‣ Chronic and subacute DIC is most often seen in
patients with long-standing illnesses, such as
cancer or autoimmune disease.
◦ Clinical Manifestations
‣ Bleeding and Thrombotic
‣ Factors that cause bleeding: result from
consumption and depletion of platelets and coagulation factors, as well as clot lysis and
formation of FSPs that have anticoagulant properties.
‣ Bleeding manifestations include: petechiae, purpura, oozing blood, venipuncture site
bleeding, hematomas, and occult hemorrhage; respiratory system (e.g., tachypnea,
hemoptysis, and orthopnea); cardiovascular system, such as tachycardia and hypotension;
GI bleeding, abdominal distention, and bloody stools; vision changes, dizziness, headache,
mental status changes, and irritability;
‣ Thrombotic manifestations are a result of fibrin or platelet in the microvasculature.
Manifestations affect: affect the (1) skin (e.g., cyanosis, ischemic tissue necrosis,
hemorrhagic necrosis); (2) respiratory system (e.g., tachypnea, dyspnea, pulmonary
emboli, and acute respiratory distress syndrome [ARDS]); (3) cardiovascular system (e.g.,
ECG changes, venous distention); (4) GI tract (e.g., abdominal pain, paralytic ileus); and
(5) kidney damage and oliguria, leading to failure.
◦ Diagnostic Studies:
◦ Interprofessional Care:
‣ If chronic DIC is diagnosed in a patient who is not bleeding, no therapy for DIC is needed.
‣ Treatment of the underlying disease may be enough to reverse the DIC (e.g., chemotherapy
when DIC is caused by cancer).
‣ Blood products are given cautiously based on specific component deficiencies to patients
who have serious bleeding, are at high risk for bleeding (e.g., surgery), or require invasive
procedures.
‣ Blood product support with platelets, cryoprecipitate, and fresh frozen plasma (FFP) is
usually reserved for a patient with life-threatening hemorrhage
‣ Therapy stabilizes a patient, prevents exsanguination or massive thrombosis
‣ Cryoprecipitate replaces factor VIII and fibrinogen and is given if the fibrinogen level is
below 100 mg/dL
‣ patient with thrombosis is often treated by anticoagulation with heparin or LMSH. (only
when the benefit (reduce clotting) outweighs the risk (further bleeding)
◦ Nursing Diagnoses
‣ Risk for bleeding
‣ Ineffective tissue perfusion
‣ Acute pain
‣ Impaired cardiac output
◦ Nursing Implementation
‣ Early detection of bleeding and clotting, both occult and overt, must be a primary goal.
Assess for signs of external bleeding (e.g., petechiae, oozing at IV or injection sites), signs
of internal bleeding (e.g., increased heart rate, changes in mental status, increasing
abdominal girth, pain), and any signs that microthrombi may be causing clinically
significant organ damage (e.g., decreased renal output).
Neutropenia
• A reduction in neutrophils is termed neutropenia.
• Some use the terms granulocytopenia and neutropenia interchangeably because the largest
constituency of granulocytes is the neutrophils.
• Absolute neutrophil count (ANC) is determined by multiplying the total WBC count by the
percent of neutrophils.
• Neutropenia is defined as ANC less than 1000 cells/μL
• Normal range of neutrophils is 2200 to 7700
• Severe neutropenia is defined as an ANC less than 500 cells/μL
• The faster the drop and the longer the duration, the greater the chance of life-threatening
infection, sepsis, and death.
• Risk factors for infection: immune disorders (HIV), being older than 60, existing infection,
hospital setting, COPD
• most common cause of neutropenia is the use of chemotherapy and immunosuppressive
therapy in the treatment of cancer and autoimmune disease.
◦ Clinical Manifestations
‣ manifestations of inflammation—redness, heat, and swelling—may not occur.
(decrease in WBC)
‣ Fever (requires immediate attention, indication of infection, can lead to sepsis and
death, patient does not have ability to fight infection)
‣ Manifestations related to infection of mucous membranes include: sore throat and
dysphagia, ulcerative lesions of the pharyngeal and buccal mucosa, diarrhea, rectal
tenderness, vaginal itching or discharge, shortness of breath, and nonproductive
cough
‣ Pain should be reported
◦ Diagnostic Studies
‣ Peripheral WBC count and bone marrow aspiration and biopsy
‣ WBC count neutropenia of 500 to 1000- patient is at moderate risk for for a
 bacterial infection
‣ Neutropenia of less than 500 patient at severe risk
‣ Patients with acute leukemia who present with a high WBC may in fact have
neutropenia
‣ Peripheral blood smear assesses for immature forms of WBCs
◦ Interprofessional and Nursing Management
‣ Sometimes the cause of the neutropenia can be easily treated
‣ Monitor the neutropenic patient for signs and symptoms of infection
‣ Early identification of a potentially infective organism depends on obtaining
cultures from various sites.
‣ Giving broad-spectrum antibiotics is usually by the IV route because of the rapidly
lethal effects of infection.
‣ The use of a third- or fourth-generation cephalosporin with broad microorganism
coverage (e.g., cefepime, ceftazidime) or a carbapenem (e.g., imipenem/cilastatin
[Primaxin]) will be started and augmented depending on the patient’s response
and/or results of the diagnostic tests.
‣ Antifungal therapy is started whenever a culture is positive, or in patients who do
not become afebrile with broad-spectrum antibiotic coverage.
‣ Cultures of sputum, throat, lesions, wounds, urine, and feces may part of patient
surveillance.
‣ CT scans, bronchoscopy with bronchial brushings, or lung biopsy to diagnose
pneumonic infiltrates
‣ Invasive diagnostic studies are often contraindicated
‣ Hand washing is the single most important preventive measure to minimize the risk
for infection in the neutropenic patient.
‣ Separate immunocompromised patients from those who are infected or have
conditions that increase the probability of transmitting infections (e.g., poor hygiene
caused by lack of understanding or cognitive dysfunction).
‣ If the patient is hospitalized, a private room should be used.
‣ High-efficiency particulate air (HEPA) filtration is an air-handling method with a
high-flow filtering system that can reduce or eliminate the number of aerosolized
pathogens in the environment
‣ Teach the patient and caregiver to avoid potentially hazardous foods, such as raw
and undercooked meats and eggs and soft cheeses with molds
Myelodysplastic Syndrome
◦ a group of related hematologic disorders characterized by peripheral blood cytopenias (from
ineffective blood cell production) and changes in the cellularity of the bone marrow with
dysplastic changes
◦ Etiology and Pathophysiology
‣ People who have received radiation therapy, had chemotherapy with alkylating agents
(e.g., chlorambucil, cyclophosphamide, melphalan), or were exposed to industrial solvents
(e.g., benzene) have a higher risk for developing MDS
‣ Referred to as Clonal disorder: some bone marrow stem cells continue to function
normally, while others do not.
‣ MDS may progress to acute myelogenous leukemia (AML).
◦ Clinical Manifestations
‣ infection and bleeding caused by inadequate numbers of ineffectively functioning
circulating granulocytes or platelets. (may be found in patients for symptoms of anemia,
 thrombocytopenia or neutropenia: however, these conditions can develop in advanced
MDS).
◦ Diagnostic Studies
‣ Bone marrow biopsy with aspirate analysis is essential for both the diagnosis and
classification of the specific type of MDS.
◦ Interprofessional and Nursing Management
‣ hematologic monitoring (serial bone marrow and peripheral blood examinations),
antibiotic therapy, or transfusions with blood products along with iron chelators to prevent
iron overload.
‣ Low risk patients: treated with EPO, myeloid growth factors, and thrombopoietin
‣ Intensive chemotherapy and/or HSCT: high-risk features to treat bone marrow dysfunction
‣ Azacitidine (Vidaza) and decitabine (Dacogen) are drugs that help restore normal growth
control and differentiation of hematopoietic cells and reduce the frequency of
transformation of MDS to acute leukemia.
‣ lenalidomide (Revlimid), cytarabine with or without antitumor antibiotics (anthracyclines),
antithymocyte globulin, and cyclosporine

Leukemia
◦ Describe a group of cancers affecting
the blood and blood-forming tissues of
the bone marrow, lymph system, and
spleen
◦ results in an accumulation of
dysfunctional cells because of a loss of
regulation in cell division.
Etiology and Pathophysiology
◦ Begins as a mutation in the DNA of
certain cells
◦ Causes of leukemia: abnormal genes
(oncogenes), chemical agents, chemotherapeutic agents, viruses, radiation, immunologic
deficiencies, farmworkers, smoking, and obesity
◦ Occurs more often in those with certain congenital or inherited abnormalities (down syndrome)
Classification
◦ 4 major types of leukemia:
acute lymphocytic leukemia
(ALL), acute myelogenous
leukemia (AML), chronic
myelogenous (granulocytic)
leukemia (CML), and chronic
lymphocytic leukemia (CLL).
◦ Acute leukemia is characterized
by the clonal proliferation of
immature hematopoietic cells.
◦ Chronic leukemias involve
more mature forms of the
WBC.
‣ Acute Myelogenous
Leukemia: Its onset is
often abrupt and dramatic.
AML is characterized by uncontrolled proliferation of myeloblasts.
 ‣ Acute Lymphocytic Leukemia: immature small lymphocytes proliferate in the bone marrow.
Most patients have fever at the time of diagnosis. Signs and symptoms may appear abruptly
with bleeding or fever, or they may be insidious with progressive weakness, fatigue, bone
and/or joint pain, and bleeding tendencies. Philadelphia chromosome interferes with
normal cell cycle events, such as the regulation of cell proliferation.
‣ Chronic Myelogenous Leukemia: caused by excessive development of neoplastic
granulocytes in the bone marrow. The chronic phase of CML can last for several years. It
usually can be well controlled with treatment, even with treatment it can end in blastic
phase
‣ Chronic Lymphocytic Leukemia: CLL is characterized by the production and accumulation
of functionally inactive but long-lived, small, mature-appearing lymphocytes. Pressure on
nerves from enlarged lymph nodes causes pain and even paralysis. Many patients in the
early stages of CLL do not need treatment.
Clinical Manifestations
◦ The abnormal WBCs continue to accumulate because they do not go through the normal cell life
cycle to death (apoptosis).
◦ The leukemic cells may infiltrate the patient’s organs, leading to problems such as
splenomegaly, hepatomegaly, lymphadenopathy, bone pain, meningeal irritation, and oral
lesions.
◦ Solid masses resulting from collections of leukemic cells, called chloromas, can occur.
◦ More than 100,000 cells/μL) can cause the blood to thicken and potentially block circulatory
pathways (leukostasis).
Diagnostic Studies
◦ Peripheral blood evaluation and bone marrow examination are the primary methods for
classifying types of leukemia
◦ Morphologic, histochemical, immunologic, and cytogenetic methods are used to identify
leukemic cell types, stage of development, and significant genetic mutations.
◦ lumbar puncture and PET/CT scans
Interprofessional Care
◦ chemotherapy is the mainstay of the treatment
◦ Radiation and biologic therapies
◦ nonsymptomatic patients with CLL, watchful waiting with active supportive care may be
appropriate.
◦ complete remission, there is no evidence of overt disease on physical examination, and the bone
marrow and peripheral blood appear normal. Minimal residual disease is defined as tumor cells
that cannot be detected by morphologic examination but can be identified by molecular testing.
Partial remission is characterized by a lack of symptoms and a normal peripheral blood smear,
but there is still evidence of disease in the bone marrow. Molecular remission means that all
molecular studies are negative for residual leukemia.
◦ patients have such a high WBC count (e.g., 100,000 cells/μL or more) that initial emergent
treatment may include leukapheresis and hydroxyurea.
‣ Stages of Chemotherapy
• Induction Therapy: aggressive treatment that seeks to destroy leukemic cells in the
tissues, peripheral blood, and bone marrow to eventually restore normal
hematopoiesis on bone marrow recovery. Common chemotherapy agents for induction
of AML include cytarabine and an antitumor antibiotic (anthracycline), such as
daunorubicin, idarubicin, or mitoxantrone. patient may become critically ill because
the bone marrow is severely depressed by the chemotherapeutic agents
• Postinduction or Postremission Therapy: Intensification therapy, or high-dose therapy,
may be given immediately after induction therapy for several months. Consolidation
therapy is started after a remission is achieved. Purpose is to eliminate remaining
 leukemic cells that may not be clinically or pathologically evident.
• Maintenance Therapy:
treatment with lower
doses of the same drugs
used in induction or
other drugs given every
few weeks for a
prolonged period.
‣ Drug Therapy Regimens: all-
trans retinoic acid, which
treats acute promyelocytic
leukemia (a type of AML).
Midostaurin is a multikinase
inhibitor that inhibits this growth pathway in AML patients with the FLT3 mutation.
Imatinib (Gleevec) and other tyrosine-kinase inhibitors target the BCR-ABL protein.
Rituximab binds to the B-cell antigen (CD20) and is a treatment for CLL. corticosteroids
and radiation therapy have a role in the treatment of the patient with leukemia. Total body
radiation may be used to prepare a patient for bone marrow transplantation. In ALL,
prophylactic intrathecal methotrexate or cytarabine is given to decrease the chance of CNS
involvement. Immunotherapy and targeted therapy, such as monoclonal antibodies and
chimeric antigen receptor (CAR) T cells
‣ Hematopoietic Stem Cell Transplantation: eliminate all leukemic cells from the body
using combinations of chemotherapy. This treatment eradicates the patient’s hematopoietic
stem cells, which are then replaced with those of an HLA-matched sibling, HLA-half-
matched relative, volunteer donor (allogeneic), or identical twin (syngeneic).
Nursing Implementation
◦ Acute care: The family and patient needs help adjusting to the stress of this abrupt onset of
serious illness and the losses imposed by the sick role. 1) maximizing the patient’s physical
functioning, (2) teaching patients that acute side effects of treatment are usually temporary,
and (3) encouraging patients to discuss their quality-of-life issues.
◦ Ambulatory Care: Teach the patient and caregiver the importance of continued diligence in
disease management and the need for follow-up care. Review the drugs, self-care measures, and
when to seek medical attention. Assistance may be needed to reestablish the various
relationships that are a part of the patient’s life. Involving the patient in survivor networks and
support groups.

Lymphomas
◦ cancers originating in the
bone marrow and lymphatic
structures resulting in the
proliferation of lymphocytes.

Hodgkin's Lymphoma:
proliferation of abnormal giant,
multinucleated cells, called Reed-
Sternberg cells, or it’s variant,
Hodgkin cells (mononucleated),
which proliferate in the lymph nodes.
occurring most frequently in persons
from 15 to 30 years of age and above
55 years of age
Etiology and Pathophysiology: Causes include: infection with Epstein-Barr virus (EBV), genetic
predisposition, and exposure to occupational toxins. Incidence increases in those with HIV infection.
The disease starts in a single location and spreads. Infiltrates other organs such as lungs, spleen, and
liver.
Clinical Manifestations
◦ Symptoms usually gradual
◦ initial development is most often enlargement of cervical, axillary, or inguinal lymph nodes
(Fig. 30.14). A mediastinal node mass is the second most common location. The nodes are
movable and nontender.
◦ weight loss, fatigue, weakness, fever, chills, tachycardia, or night sweats.
◦ Initial symptoms: fever (over 100.4°F [38°C]), drenching night sweats, and weight loss
(exceeding 10% in 6 months), are termed B symptoms.
◦ Alcohol can have a rapid onset of pain
◦ Generalized pruritus without skin lesions
◦ Cough, dyspnea, stridor, and dysphagia may all reflect mediastinal node involvement.
◦ Advanced symptoms: hepatomegaly and splenomegaly
◦ Anemia
◦ Jaundice may occur from liver involvement.
◦ Spinal cord compression leading to paraplegia may occur
◦ Bone pain
Diagnostic and Staging Studies
◦ Peripheral blood analysis, excisional lymph node biopsy, bone marrow examination, and
radiologic studies
◦ Other blood studies may show increased erythrocyte sedimentation rate, high leukocyte alkaline
phosphatase from liver and bone involvement, hypercalcemia from bone involvement, and
hypoalbuminemia from liver involvement.
◦ Radiologic evaluation can help define all sites and determine the clinical stage of the disease.
PET and CT scans
Interprofessional and Nursing Management
◦ The standard for chemotherapy is the ABVD regimen: doxorubicin (Adriamycin), bleomycin,
vinblastine, and dacarbazine. Patients with favorable early-stage disease receive 2 to 4 cycles of
chemotherapy.3 Patients with early-stage disease but unfavorable prognostic features (e.g., B
symptoms) or intermediate-stage disease receive 4 to 6 cycles of chemotherapy. Advanced-stage
Hodgkin’s lymphoma is treated more aggressively using 6 to 8 cycles of chemotherapy. A
common regimen is BEACOPP (bleomycin, etoposide, doxorubicin [Adriamycin],
cyclophosphamide, vincristine [Oncovin], procarbazine, and prednisone)
◦ chemotherapy regimens and newer agents, such as brentuximab vedotin, nivolumab,
bendamustine, and pembrolizumab are used to treat patients who have relapsed or refractory
disease
◦ Occasionally, single drugs may be given palliatively to those who cannot tolerate intensive
combination therapy.
◦ Secondary solid tumor cancers may occur 10 years after treatment for Hodgkin’s lymphoma
◦ Patients should have close follow-up and screening for early detection of these problems.
◦ supporting the patient through the consequences of treatment is extremely important.
◦ Fertility issues may be of concern because this disease is often seen in adolescents and young
adults.

Multiple Myeloma
◦ condition in which cancerous plasma cells proliferate in the bone marrow and destroy bone.
◦ more common in men than women
Etiology and Pathophysiology
◦ possible that exposure to organic chemicals (such as benzene), herbicides, and insecticides may
play a role
◦ Viral infections, such as HIV, may influence the risk for developing multiple myeloma
◦ The disease process involves excess production of plasma cells.
◦ myeloma tumors make monoclonal antibodies
◦ Monoclonal means they are all of one kind, making them ineffective and even harmful.
◦ Not only do they not fight infections, but they infiltrate the bone marrow.
◦ As myeloma protein increases, normal plasma cells are reduced. This further compromises the
body’s normal immune response.
Clinical Manifestations
◦ Develops slowly and insidiously
◦ patient often does not have symptoms until the disease is advanced.
◦ Skeletal pain is the major manifestation.
◦ Diffuse osteoporosis develops as the myeloma protein destroys bone.
◦ Vertebral destruction
◦ Bony degeneration causes calcium loss from bones, eventually causing hypercalcemia.
Hypercalcemia may cause renal, GI, or neurologic problems, such as polyuria, anorexia,
confusion, and heart problems.
◦ anemia, thrombocytopenia, neutropenia, and immune dysfunction from the replacement of
normal bone marrow with plasma cells.
Diagnostic Studies
◦ laboratory, radiologic, and bone marrow examination. M protein is often found in the blood and
urine. Possible findings include pancytopenia, hypercalcemia, Bence Jones protein in the urine,
and a high serum creatinine.
◦ MRI, PET, &CT scans
◦ The simplest measure of staging and prognosis in multiple myeloma is based on blood levels of
2 markers: β2-microglobulin and albumin.
Interprofessional and Nursing Management
◦ corticosteroids (dexamethasone or
prednisone), chemotherapy,
immunotherapy, targeted therapy, and
HSCT
◦ Bisphosphonates, such as pamidronate
and zoledronic acid, inhibit bone
breakdown and are used to treat
skeletal pain and hypercalcemia (given
monthly by IV infusion)
◦ use caution when moving and
ambulating the patient. A slight twist or
strain in the wrong area may cause a
fracture
◦ Kyphoplasty is a minimally invasive
procedure (usually done in an
interventional radiology area) that
injects cement to stabilize the vertebral
compression.
◦ High-dose chemotherapy followed by
HSCT has evolved as the standard of
care in eligible patients
◦ Immunomodulators: thalidomide,
 lenalidomide, and pomalidomide. Proteosome inhibitors include bortezomib, carfilzomib, and
ixazomib.
◦ Daratumumab is a
monoclonal antibody
against CD38
◦ IV furosemide promotes
renal excretion of calcium.
◦ Ambulation and adequate
hydration are used to treat
hypercalcemia,
dehydration, and potential
renal damage. Weight
bearing helps the bones resorb some calcium. Maintaining adequate hydration helps minimize
problems from hypercalcemia and prevent protein precipitates from causing renal tubular
obstruction.
◦ Analgesics, such as nonsteroidal
antiinflammatory drugs,
acetaminophen, or an
acetaminophen/opioid combination
for bone pain
◦ Patients are at risk for DVT related to
chemotherapy and immobility

Disorders of the Spleen

◦ Many illnesses can cause some degree
of splenomegaly (enlarged spleen)
◦ hypersplenism refers to the
occurrence of splenomegaly and
peripheral cytopenias (anemia,
leukopenia, and thrombocytopenia)
◦ A slight to moderate enlargement of
the spleen is usually asymptomatic
and found during routine
examination of the abdomen.
◦ patient may have abdominal
discomfort and early satiety.
◦ Assess the size of the spleen: adionuclide colloid liver spleen scan, CT or PET scan, MRI, and
ultrasound scan
◦ splenectomy is done as part of the evaluation or treatment of splenomegaly. This is done for
splenic rupture
◦ Splenomegaly may be painful. The patient may need analgesics; care in moving, turning, and
positioning; and evaluation of lung expansion because spleen enlargement may impair
diaphragmatic excursion. Immunoglobin deficiencies may develop and patients have a lifelong
risk for infection
Blood Component Therapy

used to manage hematologic diseases

Blood component therapy only temporarily supports the patient until the underlying problem is
resolved
Should only be used only if needed
Whole blood is rarely used except
in cases of massive hemorrhage,
severe coagulopathy, shock, or in
military settings
Administration Procedure
◦ May be given through 22
gauge IV needle, cannula,
or catheter
◦ Smaller gauges, such as
25-gauge, may be used in
neonates.39 Larger sizes
(e.g., 18- or 16-gauge) in
adults may be preferred if
rapid transfusions are
given or if the infusion is
sluggish.
◦ Improper product-to-
patient identification is
the most common cause of
hemolytic transfusion
reactions, thus placing a
great responsibility on nurses to carry
out the identification procedure
appropriately.
◦ However, after multiple platelet
transfusions, a patient may develop
anti-HLA antibodies to the transfused
platelets.
◦ With the use of lymphocyte typing to
match HLA types of the donor and
recipient, multiple platelet
transfusions can be given with fewer
complications to those who develop
antibodies to platelets.
◦ Take the patient’s vital signs before
beginning the transfusion so that you
have a baseline measure.
◦ Do not refrigerate it on the nursing
unit. If the blood is not started within
30 minutes, return it to the blood
bank for storage. During the first 15
minutes or 50 mL of blood infusion,
stay with the patient.
◦ The rate of infusion during this
period should be no more than 2 mL/
min.
◦ Other blood components, such as
fresh frozen plasma and platelets, may be given over 15 to 30 minutes.
◦ After the first 15 minutes, vital signs are usually retaken.
◦ Observe patient every 30 min and up to 1hr after the transfusion
◦ The transfusion should not take more than 4 hours to give because of the increased risk for
 bacterial growth in
the product once it
is out of
refrigeration.
Blood Transfusion
Reactions
◦ If reactions occur
do the following:
(1) stop the
transfusion; (2)
maintain a patent
IV line with saline
solution; (3) notify
the blood bank and
the HCP
immediately; (4)
recheck identifying tags and numbers; (5) monitor vital signs and urine output; (6) treat
symptoms per HCP order; (7) save the blood bag and tubing and send them to the blood bank
for examination; (8) collect required blood and urine specimens at intervals based on the
hospital policy to evaluate for hemolysis; and (9) document
◦ Acute Transfusion Reactions: Mislabeling specimens and giving blood to the wrong person can
cause this
◦ Delayed Transfusion Reactions: infections, and iron overload
Autotransfusion
• involves removing whole blood from a person and transfusing that blood back into the same person.
This avoids problems of incompatibility, allergic reactions, and disease transmission. monitoring
coagulation studies in the patient receiving an autotransfusion is important.
◦ Autologous donation or elective phlebotomy (predeposit transfusion). A person donates blood
before a planned surgical procedure. The blood can be frozen and stored for up to 10 years.
Beneficial for patients with rare blood type or need limited blood support during a major
surgical procedure
◦ Autotransfusion. A method for replacing blood volume that involves safely and aseptically
collecting, filtering, and returning the patient’s own blood lost during a major surgical
procedure or from a traumatic injury.
Non-Hodgkin's Lymphoma
• group of cancers of primarily B, T, natural killer (NK), histiocytic, and dendritic cells
• Most common subtypes are diffuse large B-cell lymphoma, follicular lymphoma, marginal zone
lymphoma, mantle cell lymphoma, and peripheral T-cell lymphoma not otherwise specificed
Etiology and Pathophysiology
• may result from chromosomal translocations, infections, environmental factors (pesticides,
herbicides, solvents, organic chemicals, wood preservatives), and immunodeficiency states.
• Viruses and bacteria implicated
in the pathogenesis of NHL,
include HTLV-1, EBV, human
herpesvirus 8, hepatitis B and C,
H. pylori, Chlamydophila psittaci,
Campylobacter jejuni, and
Borrelia burgdorferi
• More common in people who
have inherited immunodeficiency
syndromes, received
chemotherapy or radiation
therapy
Clinical Manifestations
• painless lymph node enlargement
• can manifest in nonspecific ways,
such as an airway obstruction,
hyperuricemia and renal failure
from tumor lysis syndrome,
pericardial tamponade, and GI
symptoms.
• Patients with high-grade
lymphomas may have
lymphadenopathy and B
symptoms, such as fever, night
sweats, and weight loss.
Diagnostic and Staging Studies
◦ MRI or lumbar puncture to
rule out CNS disease, a bone
marrow biopsy to determine bone marrow infiltration, or a barium enema or upper endoscopy
to look for GI involvement
◦ In early NHL, the CBC may be normal, but some lymphomas manifest in a “leukemic” phase.
◦ blood tests for tumor lysis; screening for hepatitis, HIV, and other infections; skin biopsies; bone
marrow biopsies; and lumbar punctures.
Interprofessional and Nursing Management
◦ chemotherapy, biotherapy, radiation, and sometimes phototherapy and topical therapy
◦ more aggressive lymphomas (diffuse large B cell) are generally more responsive to treatment
◦ Patients with low-grade (indolent) lymphoma may live 10 years or more without treatment.
◦ Those that have an infectious basis, such as H. pylori gastric lymphomas, may be treated with
antibiotic or antiviral therapy.
◦ Rituximab, a monoclonal antibody against the CD20 antigen on the surface of normal and
malignant B cells
◦ T-cell lymphomas may be treated with topical corticosteroids or topical chemotherapy for
limited-stage disease. For more diffuse disease, treatment may include methotrexate, α-
interferon, brentuximab vedotin, or other drugs