Hematologic Disorders

Assessment and Management Chapter 33

Blood Cells -- Overview

The hematologic system is composed of what three parts? Identify the cellular components of blood. What substances constitute the fluid portion of blood? Identify the processes that must be in balance for hemostasis. Define hematopoiesis.

Erythrocytes (RBCs)
What is important to know about RBCs?
What is the principle component of mature RBCs? What is the function of that component? What hormone is important to the production of RBCs? What other substances are required for normal erythrocyte production? What are normal lab values?

Leukocytes (WBCs)
What is important to know about WBCs?
What is overall primary function? What are the two general categories? Identify cells within each category. What is the primary function of the differentiated cells? Bands are less mature granulocytes. What does an increase in bands indicate?

Thrombocytes (Platelets)
What is important to know about platelets?
They technically are not cells, what are they? What hormone partially regulates their production? What is their essential role? What are normal lab values? What is the collaborative problem associated with thrombocytosis?

Coagulation Cascade
Initiated by triggering event Landslide effect Two pathways
Intrinsic (slower) Extrinsic (faster)

Clotting factors are activated sequentially Regardless of triggering event the outcome is formation of a fibrin clot

More on Hemostasis
What has to happen once the coagulation cascade has achieved the formation of the clot and the vessel injury has healed? How will this process be accomplished? What lab test will identify the end result of this process?

Hematologic Assessment: Lab Profile

CBC Peripheral blood smear Hemoglobin Hematocrit Mean cell volume Mean cell hemoglobin Mean cell hemoglobin concentration Reticulocyte count Total iron binding capacity Iron Serum ferritin Platelet count Hg electrophoresis Direct Coombs Indirect Coombs Bleeding time Euglobin lysis time Fibrin degredation products

Bone Marrow
Identify the diagnostic tests associated with bone marrow evaluation. What is the purpose of these diagnostic tests? Identify the most prevalent sites used. Identify important pre-procedure nursing responsibilities. What are two hazards of these procedures? Identify post-procedure nursing

Anemias
TYPE CAUSE Autosomal recessive inheritance of two defective genes for hemoglobin synthesis X-linked recessive inherited deficiency of the enzyme glucose-6-phosphate dehydrogenase Abnormal immune function; immune reactive cells fail to recognize own blood cells as self cells alcoholism, malabsorption syndromes, partial gastrectomy); Or rapid metabolic activity (pregnancy, adolescence, infection)

Sickle Cell Disease G6PD Deficiency Autoimmune Hemolytic

Iron Deficiency Inadequate iron intake (iron deficient diet, chronic

Anemias
TYPE CAUSE Dietary deficiency Failure to absorb vitamin B12 from intestinal tract (partial gastrectomy, pernicious anemia) Dietary deficiency Malabsorption syndromes Drugs (Oral contraceptives, anticonvulsants, methotrexate)

Vitamin B12 Deficiency Folic Acid Deficiency

Aplastic Anemia Exposure to myelotoxic agents

(radiation, benzene, chloromycetin, alkylating agents, anti-metabolities, sulfonamides, insecticides) Viral Infections [unproven] (Epstein-Barr virus, hepatitis B, cytomegalovirus)

Anemia: Manifestations
Identify manifestations specific to the following body systems.
Integumentary Cardiovascular Respiratory Neurologic

What will the severity of symptoms depend upon?

Care of Anemia
What is the most important treatment consideration? Review the Nursing Process: The Patient with Anemia, p. 1047 -1049

Leukopenia
Define leukopenia. Identify which two cell types are predominantly involved. The patient who is leukpenic is at increase risk of what complication? What is the ANC? How is it determined? How will leukopenia be treated?

Leukemia
Group of malignant disorders involving abnormal overproduction of a specific WBC type
Usually at an immature state In the bone marrow

May be acute or chronic Categorized by the specific maturational pathway from which the abnormal cell arose
Lymphocytic/lymphoblastic

Leukemia: Basic Physiology

Malignant transformation of the stem cells causing an abnormal proliferation of a specific type of leukocyte Proliferation shuts down normal bone marrow production Results in anemia, thrombocytopenia, and leukopenia of the unaffected WBC types

Leukemia
INFECTION and HEMORRHAGE are the causes of death Etiology: many genetic and environmental factors. . . Involving gene damage to the cells
May be viral pathogenesis Can be secondary to bone marrow damage from radiation

Leukemia
Lab assessment
Decreased H&H Decreased platelets Altered WBC (low, normal, elevated: usually 20,000 to 100,000 Bone marrow aspiration/biopsy identifies types

Leukemia
Drug therapy
Intensive combination chemotherapy Major side effects: bone marrow depression
Increases vulnerability to infection Antibiotics, antifungals, antivirals

Bone marrow transplantation (BMT) Peripheral Blood Stem Cell Transplant (PBSCT)

Malignant Lymphoma
Malignancies characterized by a proliferation of committed lymphocytes rather than stem cell precursors Solid tumors- particularly affecting lymph nodes and spleen Types
Hodgkins Non-Hodgkins

Hodgkins Lymphoma
Incidence:
Peaks in mid to late 20s And > 50 (males affected more in > 50)

Causes
Probably viral or chemical Usually originates in a single or chain of nodes Reed-Sternberg cells (characteristic marker)

Assessment: enlarged painless node or nodes, fever, malaise, night

Hodgkins Lymphoma
Diagnosis: Biopsy reveals R-S cells Staging: crucial treatment is based on extent of disease Prognosis: one of most curable forms of cancer Treatment
Stage 1-2: extensive radiation More extensive: radiation with aggressive multiagent chemotherapy Nursing management focuses on side effects

Non-Hodgkins Lymphoma
Cancers of the lymph tissue that are not Hodgkins Most occur in older adults Causes unknown
Suspect viral, radiation, chemical

Prognosis:
Dependent on cell type Ranges from excellent to poor

Treatment and nursing care similar to Hodgkins lymphoma

Platelet Disorders
What is thrombocytopenia? What can it result from?

Autoimmune Thrombocytopenic Purpura

Cause- autoimmune Patho- body makes an anti-platelet antibody that destroys platelets What manifestations would you expect? Diagnosis: platelet count < 20,000 Collaborative care
Identify cause if possible and treat Provide safe environment Immunosuppressants IVIG Splenectomy

Thrombotic Thrombocytopenic Purpura

Rare Platelets clump
Clinical picture similar to DIC

Cause: suspect autoimmune Treatment:

Immunosuppressants Plasma exchange Platelet aggregation inhibitors

Clotting Factor Disorders

Most are congenitally transmitted gene abnormalities of one clotting factor
Hemophilia A & B von Willebrands

Disseminated Intravascular Coagulation

An acquired disorder Triggers: sepsis, trauma, cancer, shock,
abruptio placenta, toxins, allergic reactions

Patho thrombi form in microcirculation consuming all platelets and clotting factors Fibrinolytic system triggered to release fibrin degredation products (potent anticoagulants) further bleeding

DIC
Characterized by:
Low platelet and fibrinogen levels Prolonged PT, aPTT, thrombin time Elevated D-dimer

Clinical manifestations:
Bleeding from mucous membranes, venipuncture sites, GI and urinary tracts

Treatment:
Most important: identify and treat cause Correct secondary effects of tissue hypoxia Replace depleted coagulation factors Heparin infusions (controvesial)

Transfusion Therapy
Blood component that is removed from a door and transfused to benefit a recipient. Components may be transfused individually or collectively. Pretransfusion responsibilities:
Review and understand agency policies and procedures Review physicians order Evaluate clinical condition of patient and lab values

Transfusion Therapy
Normal saline is solution of choice Must determine that blood components to be administered are correct
Must check components with another RN

Transfusion responsibilities
Measure and record vital signs initially and at specified intervals Initiate infusion slowly and observe for signs of reaction

Transfusion Reactions
Hemolytic Transfusion Reaction
Caused by blood type or Rh incompatibility May range from mild to life threatening Clinical signs: fever, chills, low back pain, nause, chest tightness, dyspnea, anxiety Onset: may be immediate or may not occur until subsequent transfusion

Transfusion Reactions
Allergic Transfusion Reaction
Most often in those with h/o allergies Clinical signs: urticaria, itching, flushing, bronchospams, and occasionally anaphylaxis Onset- during or up to 24 hours after transfusion

What pharmacologic intervention may prevent future reactions?

Transfusion Reactions
Febrile Transfusion Reaction
Occurs commonly with those with antiWBC antibodies Clinical signs chills, muscle stiffness, tachycardia, fever, hypotension, tachypnea

How will the transfusion procedure be adapted to prevent febrile reactions?

Transfusion Reaction
Bacterial Transfusion Reactions
Seen after transfusion of contaminated blood or blood products Clinical signs-- fever, chills, hypotension Onset is rapid

What interventions are essential to preventing a bacterial transfusion reaction.

Transfusion Reaction
Circulatory Overload
In what patient population would you anticipate this reaction? Most common with whole blood transfusions or with multiple transfusions What clinical signs would you expect?

When Transfusion Reaction Occurs

STOP THE TRANSFUSION Maintain 0.9% NS at KVO rate Notify physician Document according to agency policy/protocol Teat symptoms per physician order Monitor VS Collect specimens (blood, urine) and send to lab with transfusion administration set Thoroughly document