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Unit 7-chapters 32 & 33:

Assessment & Management of Patients With Nonmalignant Hematologic Disorders

Anemia

Polycythemia

Bleeding Disorders

Acquired Coagulation Disorders

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Hematologic System

Blood: 7-10% of body weight, 5-6L

Plasma: fluid portion of blood, 55-60%, More than 90% of plasma is water.
Blood cells: 40-45% of blood volume.
Erythrocytes (RBC)
Leukocytes (WBC)
Thrombocytes (Platelets)
Hematopoiesis: is the complex process of the formation and maturation of blood cells.
Hemostasis: Is the process of preventing blood loss from intact vessels and of stopping bleeding from a severed vessel.

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Blood Cells

RBC: Carries hemoglobin to provide O² to tissues.
Erythropoiesis: The process of producing RBCs (requires Erythropoietin: hormone produced by kidney)
RBCs production requires: Iron stores & metabolism, Vitamin B12, Vitamin B6, Folic acid, & Protein.
Destruction- RBC life span 120 days.
WBC: Total WBC 4,000-11,000 cells/mm³
Platelets: Play an essential role in the control of bleeding.
Lifespan 7 to 10 days.

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Assessment of Hematologic Health

Health History:
Ethnicity, Family Hx, Nutritional Hx, Medications, onset of symptoms, severity, risk factors, functional ability of patient, distress, and coping.
Physical Assessment (skin, oral cavity, lymph nodes, and spleen)
Diagnostic Evaluation

Hematologic studies

(CBC, PT, PTT, INR)

Bone marrow

aspiration and biopsy.

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Lower than normal hemoglobin and fewer than normal circulating erythrocytes (RBC); a sign of an underlying disorder.
Classification:
Hypoproliferative Anemia: Defect in production of RBCs
Caused by iron, vitamin B_12, or folic acid deficiency, decreased Erythropoietin production, cancer.
Hemolytic Anemia: Excess destruction of RBCs
Caused by abnormality within erythrocyte itself (sickle cell anemia, G6PD deficiency), or within the plasma (immune hemolytic anemia), or from direct injury to the RBC (mechanical heart valves).
Bleeding: Rapid blood loss, acute or chronic (hemorrhoids)

Anemias

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Anemia

A sign of an underlying disorder

Determinations	Reference range
Determinations	Conventional Unit	SI Unit
Red Cell Count	Males: 4,600,000–6,200,000/cu mm Females: 4,200,000–5,400,000/cu mm	Males: 4.6–6.2 x10¹²/L Females: 4.2–5.4 x10¹²/L
Hemoglobin	Males: 13–18 gm/dL Females: 12–16 gm/dL	Males:2.02–2.79 mmol/L Females:1.86–2.48 mmol/L

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Depend on the rapidity of the development of the anemia, duration, metabolic requirements of the patient, concurrent disorders (cardiac or pulmonary disease), and complications.
Fatigue, Weakness, Malaise
Pallor or jaundice of skin & mucous membraines.
Cardiac & respiratory symptoms (tachycardia, dyspnea)

Tongue changes: Smooth, sore tongue

Nail changes: Brittle and ridged
Angular cheilosis: is inflammation of one, or more commonly both, of the corners of the mouth.

Clinical Manifestations

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Pallor From Anemia

Angular Cheilosis

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Diagnostic Testing & Complications

Hemoglobin and Hematocrit (percentage of blood volume consisting of Erythrocytes)
Reticulocyte count, RBC indices
Iron studies, Vitamin B_12, Folate
Bone marrow aspiration
Anemia Complications:
Heart Failure (HF) & Angina
Paresthesias
Confusion, Delirium
Injury related to falls

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Medical Management

Correct or control the cause of Anemia
Transfusion of packed RBCs
Treatment specific to the type of Anemia:

Dietary therapy
Iron or vitamin supplementation: Iron, Folate, B₁₂
Transfusions
Immunosuppressive therapy
Other

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Nursing Process: The Care of the Patient With Anemia—Diagnoses

Fatigue
Altered nutrition
Altered tissue perfusion
Noncompliance with prescribed therapy
Nursing Interventions:
Balance physical activity, exercise, and rest
Maintain adequate nutrition
Maintain adequate perfusion
Patient education to promote compliance with medications and nutrition
Monitor VS and pulse oximetry; provide supplemental oxygen as needed
Monitor for potential complications

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Hypoproliferative Anemias

Iron Deficiency Anemia
Anemia in Renal Disease: caused by both a mild shortening of RBC lifespan and a deficiency of erythropoietin, blood loss in the dialysis filter.
Anemia of Chronic Disease: e.g., Rheumatoid Arthritis, severe infections, and many Cancers
Aplastic Anemia
Megaloblastic Anemia

Folic Acid Deficiency
Vitamin B₁₂ Deficiency

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Iron Deficiency Anemia

The most common type of Anemia in all ages & in the world.

Causes:

Poor Diet: The intake of dietary Iron is inadequate for haemoglobin synthesis (vegetarians diets).
In men & postmenopausal women: causes are bleeding from ulcers, inflammatory bowel disease, or GI tumors.
In premenopausal women- causes are menorrhagia (excessive menstruation) & pregnancy
In Alcoholics, chronic GI bleeding
Iron malabsorption;after gastrectomy, celiac disease

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Iron Deficiency Anemia

S&S: same as Anemia in general

With severe deficiency:

Smooth, Sore tongue
Brittle and ridged nails
Angular Cheilosis.
Assessment & Diagnosis:

History and Physical Exam
Bone marrow aspiration (Definitive method for Dx)
CBC (HCt and Hb level, RBCs count, serum iron, serum ferritin)
Fecal examination for occult blood

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Iron Deficiency Anemia

Medical & Nursing Management

Identify underlying cause (except with pregnancy)
Oral Iron medications as prescribed 6 to 12 months (e.g., Ferrous Sulfate, Ferrous Gluconate)
IV administration of Iron
Diet rich with Iron (meats, beef or chicken liver, beans, leafy green vegetables & molasses).
Taking Iron rich food with a source of vitamin C
Taking Iron an hour before meals, avoid taking Antacids or dairy products with Iron
Encourages patients to continue Iron therapy.

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Aplastic Anemia

Rare disease caused by decrease in or damage to marrow stem cells and replacing it with fat leading to anemia, neutropenia, and thrombocytopenia.
Types

Congenital or Acquired (exposure to certain medications, chemicals, or radiation)
Mostly idiopathic

S&S: as anemia, infections, purpura (bruising), lymphadenopathies, splenomegaly & retinal hemorrhage
Diagnosis: bone marrow aspiration
Management:
Bone marrow transplant with immunosuppressive therapy (Cyclosporine)
Monitor S&S of infection, bleeding, & side effect of medications

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Megaloblastic Anemias

Folic Acid Deficiency
Causes:

Poor dietary intake of uncooked green vegetables
Alcoholics- poor diet intake
Increase demands of folic acid such in pregnancy, Alcoholics, & hemolytic anemia
Malabsorption GI diseases (e.g. Celiac disease)

Folate (stored folic acid) is available in green vegetable & liver.
Small stores of folate in the body, so it can depleted within 4 months.

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Megaloblastic Anemia

Vitamin B₁₂ Deficiency
Causes

Poor dietary intake especially in vegetarians
Malabsorption from GI tract (more common) such in Cronh’s disease, ileal resection, or gastrectomy.
Chronic use of proton pump inhibitors to reduce gastric acid production (e.g., Losec)
Lack of intrinsic factor (called pernicious anemia) in the stomach causing malabsorption of vit. B₁₂ in ileum.
The body has large stores of vit. B₁₂ , so anemia developed over years.

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Clinical Manifestation

Same for folic acid & Vit.B₁₂ deficiencies:

Patients with pernicious anemia develop a smooth, sore, red tongue and mild diarrhea
Extremly Pale
Confusion
Paresthesia (numbness of feet & lower leg)
May have difficulty to maintain balance
May develop HF secondary to Anemia
The symptoms are progressive

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Medical & Nursing Management

Folate deficiency is treated by:
Increasing the amount of folic acid in the diet
Administering 1 mg of folic acid daily
For people with malabsorption administered folic acid intramuscularly (IM).
Vit. B₁₂ deficiency is treated by:
Oral suplements with vit. B₁₂ - Large doses
Monthly IM injections of vit. B₁₂
To prevent recurrence of prnicious anemia, vit. B₁₂ therapy must be continued for life.
Nurse should assess patient skin, mucous membranes, tongue & patient stability.

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Hemolytic Anemias

Sickle cell Anemia
Thalassemia
Glucose-6-Phosphate Dehydrogenase (G6PD) deficiency
Immune hemolytic Anemia
Hereditary hemochromatosis

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Sickle Cell Anemia

Is a severe hemolytic anemia that results from inheritance of the sickle hemoglobin (HbS) gene, which causes the hemoglobin molecule to be defective.
With low O₂ tension (as in venous circulation) the HbS distorts itself into a sickle shape with rigid structure
These long rigid RBC can adhere to the endothelium of small vessels
Sickled cells are rapidly hemolyzed & have a very short lifespan of 10 to 20 days.
Sickled cells reduced blood flow, thus precipitating infarction of organs (spleen, CNS, eyes, lungs, liver, kidney & bones).

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Sickle Cell Anemia

Clinical Manifestations: low Hb (5 to 11 g/dl), sclera jaundice, enlarged facial and skull bones (children).
Chronic anemia is associated with tachycardia, enlarged heart, HF and dysrhythmias may occur in adult.
Very painful acute vaso occlusive crisis.
Diagnosis: Hemoglobin electrophoresis
Medical Management: manage symptoms and complications

Hematopoietic Stem cell transplant (HSCT)
Pharmacologic therapy: (Hydrea) chemotherapy agent, decreasing the formation of sickled cells
Transfusion therapy: RBC transfusion

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Nursing Process: The Patient With Sickle Cell Anemia—Assessment

Health history and physical exam
Chronic Skin Ulcers
Pain assessment
Laboratory data: S-shaped hemoglobin
Presence of symptoms and impact of those symptoms on patient’s life; swelling, fever, pain
Sickle cell crisis assessment
Blood loss: menses, potential GI loss
Cardiovascular and Neurologic assessment

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Nursing Process: The Patient With Sickle Cell Anemia—Diagnosis

Acute Pain and Fatigue
Risk for Infection
Risk for Powerlessness
Deficient Knowledge

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Collaborative Problems and Potential Complications

Hypoxia, Ischemia, Infection
Dehydration
CVA
Anemia
Acute and chronic Renal Failure
Heart Failure
Impotence
Poor compliance
Substance abuse

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Nursing Process: The Patient With Sickle Cell Anemia—Interventions

Pain management
Manage fatigue
Infection prevention
Promote coping
Education of disease process
Monitor for complications

Nursing Interventions

MANAGING PAIN

Acute pain during a sickle cell crisis can be severe and unpredictable. The patient’s subjective description and rating of pain on a pain scale must guide the use of analgesic agents (see Chapter 12). Any joint that is acutely swollen should be supported and elevated until the swelling diminishes. Relaxation techniques, breathing exercises, and distraction are helpful for some patients. After the acute painful episode has diminished, aggressive measures should be implemented to preserve function. Physical therapy, whirlpool baths, and transcutaneous electrical nerve stimulation (TENS) are examples of such modalities.

MANAGING FATIGUE

The fatigue experienced can be acute or chronic in nature. Assisting the patient to find an appropriate balance between exercise and rest can be a useful strategy. Patients will need to develop strategies to cope with daily life demands in the setting of chronic fatigue. Maximizing nutrition, hydration, healthy sleep cycles, and diminishing tissue hypoxia can all serve to minimize fatigue. Research is needed to better understand fatigue in this patient population as well as to delineate the most effective ways to ameliorate it.

PREVENTING AND MANAGING INFECTION

Nursing care focuses on monitoring patients for signs and symptoms of infection. Prescribed antibiotics should be initiated promptly, and patients should be assessed for signs of dehydration. If patients are to take prescribed oral antibiotics at home, they must understand the importance of completing the entire course of antibiotic therapy.

PROMOTING COPING SKILLS

This illness can leave the patient feeling powerless and with decreased self-esteem because the acute exacerbations often result in chronic health problems. These feelings can be exacerbated by inadequate management of pain and fatigue, and enhancing management of pain and fatigue can be extremely useful in establishing a therapeutic relationship based on mutual trust. Nursing care that focuses on the patient’s strengths rather than deficits can enhance effective coping skills. Providing the patient with opportunities to make decisions about daily care may increase the patient’s feelings of control. The patient needs to understand the rationale for and importance of compliance with a therapeutic medication regimen.

MINIMIZING DEFICIENT KNOWLEDGE

Patients with SCD benefit from understanding what situations can precipitate a sickle cell crisis and the steps they can take to prevent or diminish such crises. Keeping warm and providing adequate hydration can be effective in diminishing the occurrence and severity of attacks.

If hydroxyurea is prescribed for a woman of childbearing age, she should be informed that the drug can cause congenital harm to unborn children and advised about pregnancy prevention.

MONITORING AND MANAGING POTENTIAL COMPLICATIONS

Management measures for many of the potential complications have been described in previous sections. Other measures follow.

Leg Ulcers. Leg ulcers require careful management and protection from trauma and contamination. Referral to a wound-ostomy-continence nurse may facilitate healing and assist with prevention. If leg ulcers fail to heal, skin grafting may be necessary. Scrupulous aseptic technique is warranted to prevent hospital-acquired infections.

Priapism Leading to Impotence. Male patients may develop sudden, painful episodes of priapism. The patient is taught to empty his bladder at the onset of the attack, exercise, and take a warm bath. If an episode persists longer than 3 hours, medical attention, which consists of IV hydration, administration of analgesic agents, and possible penile intracavernosal aspiration, is recommended. Repeated episodes may lead to extensive vascular thrombosis, resulting in impotence.

Chronic and Acute Pain. Many patients have considerable difficulty coping with chronic pain and repeated episodes of sickle cell crisis and may find it difficult to adhere to a prescribed treatment plan. Some patients with SCD develop problems with substance abuse, although the rate of abuse in this patient population is similar to populations of patients with other diseases (Natrajan & Kutler, 2016). This results from inadequate management of acute pain during episodes of crisis, which then promotes mistrust of the health care system and (from the patient’s perspective) the need to seek care from other sources. In a qualitative study of health care system engagement and opioid pain use, patients with SCD who viewed themselves as “allies” and used interpersonal and symptom-specific strategies used the hospital setting less frequently for pain management than those who had a more defensive and reactive approach with their health care providers (Brown, Weisberg, Balf-Soran, et al., 2014). Receiving care from a single provider over time is much more beneficial than receiving care from rotating physicians and staff in an ED. Prior negative ED experiences often result in the patient delaying appropriate care during a subsequent painful episode (Jenerette, Brewer, & Ataga, 2014). When crises occur, the staff in the ED should be in contact with the patient’s primary provider so that optimal management can be achieved. Frank, open discussion can be instrumental in promoting trust and improved outcomes. Health care providers’ attitudes toward patients with SCD are also important if improved outcomes are to be achieved (Freiermuth, Haywood, Silva, et al., 2014) (see Chart 33-4).

PROMOTING HOME, COMMUNITY-BASED, AND TRANSITIONAL CARE

Educating Patients About Self-Care. Because patients with SCD are typically diagnosed as children, parents participate in the initial education. As the child ages, educational interventions prepare the child to assume more responsibility for self-care. Most families can learn about vascular access device management and chelation therapy. Nurses in outpatient facilities or home care nurses may need to provide follow-up care for patients with vascular access devices.

Continuing and Transitional Care. The illness trajectory of SCD is highly varied, with unpredictable episodes of complications and crises. Care is often provided on an emergency basis, especially for some patients with pain management problems (see previous section). All health care providers who provide services to patients with SCD and their families need to communicate regularly with each other. Alternate methods of care delivery are being explored, such as day hospitals for acute pain management or patient-centered medical homes, where multidisciplinary care is emphasized (Raphael & Oyeku, 2013). Nurses are in an excellent position to serve as coordinators and facilitators between health care providers in a variety of settings so that the care of these patients is optimized. Patients need to learn which parameters are important for them to monitor and how to monitor them. Guidelines should also be given regarding when it is appropriate to seek urgent care.

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Thalassemia

A group of hereditary Anemias characterized by:

Hypochromia: Abnormal decrease in the Hb of RBCs
Extreme Microcytosis: Smaller-than-normal RBCs
Hemolysis: Destruction of blood elements
Variable degrees of Anemia

Thalassaemias are associated with defective synthesis Hb
The production of one or more globulin chain within the Hb is reduced
Causing rigidity & thus premature destruction of RBC

Management: Recurrent blood transfusion of PRBCs
Hematopoietic Stem cell transplant (HSCT)

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Glucose-6-Phosphate Dehydrogenase Deficiency (G6PD)

G6PD is gene responsible of producing enzyme that is essential for RBC membrane stability. (e.g., Favism)
All types of G6PD deficiency are inherited as X linked defects
Patient may have hemolysis under specific conditions
Clinical Features: a sever hemolytic episode can result from ingestion of: oxidant drugs, fava beans, menthol, & tonic water
Laboratory Diagnosis : screening test or assay of G6PD
Treatment:

PRBCs transfusion only if severe hemolysis
Avoid precipitants

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Polycythemia

Increased volume of RBCs
Primary Polycythemia (Polycythemia Vera)- Proliferative disorder of the myeloid stem cells.
Secondary Polycythemia: caused by excessive production of Erythropoietin.
Causes: reduced amounts of oxygen, neoplasms (renal cell carcinoma) & hemoglobinopathies
Medical Management:

Treatment not needed if condition is mild
Treat underlying cause
Therapeutic phlebotomy to reduce blood viscosity and volume.

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Bleeding Disorders

Failure of hemostatic mechanisms can result in bleeding.
Causes:

Trauma
Platelet abnormality
Coagulation factor abnormality

Clinical Manifestation: with platelet defect: Petechiae (local bleeding on the skin & mucous membranes).
Medical Management: Specific blood products
Nursing Management: limit injury, assess for signs of bleeding (Petechiae, Ecchymoses), bleeding precautions

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Bleeding Disorders

Secondary Thrombocytosis
Thrombocytopenia
Immune Thrombocytopenic Purpura (ITP)
Platelet Defects
Hemophilia
Von Willebrand disease

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Bleeding Disorders

Secondary Thrombocytosis: High number of Platelets

Causes

Infection, chronic inflammatory disorders, Iron deficiency, malignant disease, acute hemorrhage, and splenectomy.

Management: treat underlying causes

Thrombocytopenia: Low platelets level

Causes: Decreased production, or increased destruction or increased consumption.
S&S: Petchiae, nasal & gingival bleeding, excessive bleeding after surgery or dental extractions.
Diagnosis: Bone marrow biopsy, CBC (platelet count)
Management: treat cause, Platelet transfusion

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Immune Thrombocytopenic Purpura (ITP)

Autoimmune disorder characterized by a destruction of normal platelets by an unknown stimulus.
More among children and young Women.
S&S: low platelet count (less than 30.000/mm³) easy bruising, heavy menses, and petechiae on extremities or trunk.
Diagnosis: history, physical exam, CBC (platelet count), test for Hep. C and HIV
Medical Management: Improve platelet count, immunosuppressive agents.
Nursing Management : Assess life style, medication history, assess recent illnesses, and patient education.

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Platelet Defects

Platelets are not functioning normally (e.g., Aspirin, NSAID)

S&S: Bleeding (mild or severe), ecchymosis, at risk of significant bleeding after trauma or invasive procedure.
Diagnosis: Platelet function analyzer, bleeding time
Medical Management: Stop medications, transfusion of normal platelets if needed.
Nursing Management : Avoid medications causing dysfunction, alcohol, and education
Instructed patient to inform their health care providers before any invasive procedure.

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Hemophilia

Two (A&B) inherited bleeding disorder, X-linked

A: Caused by genetic defect in factor VIII
B: Genetic defect that causes deficient or defective factor IX
S&S: Hemorrhage mainly into joints (75%), hematomas, pain in the joint.
Medical Management: Administer factors VIII or IX, activated Prothrombin concentrates can be used, Aminocaproic Acid (inhibits fibrinolysis)
Nursing Management : Diagnosed in childhood

Require assistance to cope
Patient and family education
Avoid medications (e.g., Aspirin, NSAID)
Assess bleeding if it happens
Administer Analgesia if required

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Therapies for Blood Disorders

Splenectomy: for two reasons:

Splenomegaly may cause excessive destruction of blood cells & thrombocytopenia.
May necessary after abdominal trauma as spleen rupture may cause sever hemorrhage.

Therapeutic Apheresis: Blood is taken from the patient & passed through a centrifuge, where a specific component is separated from the blood and removed. The remaining blood is returned to the Pt. also used to obtain platelets from donors.
Hematopoietic Stem Cell Transplantation (HSCT): (Bone marrow transplant): for Aplastic anemia, Leukemia, and Thalassemia.

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Therapeutic Approaches

Therapeutic Phlebotomy: removal of certain amount of blood (1 unit, about 500ml) under controlled condition (e.g., Polycythemia, excessive absorption of iron)

Can deplete iron sources in the body to decrease RBCs production.

Special Preparations: using a specific blood component to treat a specific condition (e.g., Factor VIII concentrate to treat Hemophilia).
Blood Component Therapy: a Single unit of blood contains 450 mL of blood and 50 mL of anticoagulant.
(Whole Blood, PRBCs, Platelets, Fresh Frozen Plasma).

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Blood Transfusion

Donor Requirements

Assess health status, weight, normal temperature, regular pulse (50-100), BP (90-180 mm Hg systolic, 50-100 mm Hg diastolic), Hemoglobin (12.5g/dL for Women, 13.5g/dL for Men))
Pre-transfusion Assessment: Determine blood type and Rh antigen, history and physical assessment.

Patient Education: Review S&S of transfusion reaction, even with patients with previous transfusion (e.g., itching, swelling, fever, chills, respiratory distress, back pain, nausea, pain at the IV site).

Reassurance- the blood is carefully tested

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Transfusion Complications

Febrile nonhemolytic reaction
Acute hemolytic reaction: blood incompatible with recipient blood. Life-threatening
Allergic reaction: reaction to plasma protein
Circulatory overload
Bacterial contamination

Transfusion-related acute lung injury
Delayed hemolytic reaction: occur within 14 days of transfusion
Disease acquisition
Long-term transfusion therapy

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Nursing Management for Reactions

Stop: Maintain IV line using normal saline.
Assess: VS, Oxygen saturation, breath sounds, dyspnea, change in mental status, chills, diaphoresis, back pain, urticaria, JVD.
Notify Physician: Implement prescribed treatment, continue to monitor VS, cardiovascular and renal status

Notify the blood bank of possible reaction

Return: Blood container and tubing for retesting of type and culture.
Treat
Documentation according to unit’s policy

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Blood Group