Chapter 31

1. Introduction to Blood Dyscrasias and Coagulopathies

1. Fundamental Definitions

   • Blood Dyscrasias: Abnormalities in the total numbers, development, or function of blood cells.

   • Coagulopathies: Bleeding disorders resulting from defects or deficiencies in platelets or plasma clotting factors.

2. Clinical Overview

   • Disorders develop from diverse pathologic processes, many of which can be life-threatening.

   • Despite differing underlying causes, many hematologic disorders present with similar core clinical manifestations and require overlapping diagnostic approaches.

2. Gerontologic Considerations

1. Iron-Deficiency Anemia in Older Adults

   • Unusual under normal circumstances because total body iron stores naturally increase with age (the body does not eliminate excess iron).

   • Maintenance of adequate hydration is necessary.

   • Anemia in an older adult should immediately raise suspicion of chronic blood loss from the gastrointestinal (GI) or genitourinary (GU) tract.

   • Inadequate iron intake may occur due to socioeconomic and functional factors: living on a fixed income, physical inability to shop for grocery items, or lack of energy to cook complete meals.

   • Requires thorough assessment of dietary habits and targeted education on iron-deficiency prevention.

2. Pernicious Anemia & Vitamin $B_{12}$

   • Vitamin $B_{12}$ deficiency produces neurological manifestations including memory loss, dementia, confusion, and depression.

   • Neurologic changes in older adults require prompt screening for pernicious anemia to prevent irreversible neurological damage.

   • Age-related decline in gastric acidity impairs Vitamin $B_{12}$ absorption; clients taking antacids or proton pump inhibitors require careful monitoring.

3. Folic Acid Deficiency

   • Folic acid deficiency contributes to depression, cognitive decline, and psychosis in older adults.

   • Serum folic acid levels should be evaluated in older adults presenting with new-onset psychiatric or cognitive symptoms.

4. Leukemia in the Elderly

   • Both acute and chronic leukemias increase in frequency in individuals aged $50$ years and older.

   • Acute Lymphoblastic Leukemia (ALL) carries a very poor prognosis in older adults due to inability to tolerate prolonged pancytopenia.

   • Chronic Lymphocytic Leukemia (CLL) and Chronic Myelogenous Leukemia (CML) incidence rises significantly after age $50$.

   • Best management strategies for older adults with CLL require evaluating comorbidities, functional reserve, and client preferences.

3. Red Blood Cell Terminology & Normal Laboratory Values

1. Erythrocyte Pathology Descriptors (Table 31-1)

   • Normocytic: Normal cell size.

   • Microcytic: Abnormally small cell size.

   • Macrocytic: Abnormally large cell size.

   • Megaloblastic: Abnormally large, immature precursor cell.

   • Normochromic: Normal concentration of hemoglobin.

   • Hypochromic: Decreased concentration of hemoglobin (pale color).

   • Hyperchromic: Increased concentration of hemoglobin.

   • Aplastic: Severe decrease in cell production.

   • Hemolytic: Premature destruction of circulating erythrocytes.

   • Pernicious: Potentially injurious or destructive process.

2. Normal Complete Blood Count (CBC) Reference Values (Table 31-2)

   • Red Blood Cells (Erythrocytes):

     • Adult Males: $4.6-6.2 \times 10^6/\text{mm}^3$

     • Adult Females: $4.2-5.4 \times 10^6/\text{mm}^3$

   • Hematocrit:

     • Adult Males: $40-54\%$

     • Adult Females: $38-47\%$

   • Hemoglobin:

     • Adult Males: $13.5-18.0\text{ g/dL}$

     • Adult Females: $12.0-16.0\text{ g/dL}$

   • Mean Cell Volume (MCV):

     • Adult Males: $80-94\text{ }\mu\text{g/m}^3$

     • Adult Females: $81-99\text{ }\mu\text{g/m}^3$

   • Mean Cell Hemoglobin (MCH): $27-31\text{ pg/cell}$ (males and females)

   • Mean Cell Hemoglobin Concentration (MCHC): $32-36\text{ g/dL}$ (males and females)

   • Reticulocytes: $0.5\%-2.0\%$ of total RBCs (slightly higher in females)

   • White Blood Cells (Leukocytes):

     • Adult Males: $5,000-13,000/\text{mm}^3$

     • Adult Females: $5,000-10,000/\text{mm}^3$

     • Neutrophils: $3,000-7,500/\text{mm}^3$

     • Eosinophils: $50-400/\text{mm}^3$

     • Basophils: $25-100/\text{mm}^3$

     • Monocytes: $100-500/\text{mm}^3$

     • Lymphocytes: $1,500-4,500/\text{mm}^3$ (T-lymphocytes: $60\%-80\%$, B-lymphocytes: $10\%-20\%$

   • Platelets: $150,000-450,000/\text{mm}^3$

4. Overview of Anemia

1. Definition & Pathopathologic Causes

   • Anemia refers to a reduction below normal in erythrocyte numbers, hematocrit, or hemoglobin concentration.

   • Primary mechanisms:

     • Blood loss (acute hemorrhage or chronic loss).

     • Inadequate or defective erythrocyte production.

     • Accelerated premature destruction of RBCs.

2. Clinical Manifestations (Box 31-1)

   • Inadequate RBC Volume:

     • Orthostatic hypotension, thready pulse, oliguria, heart murmur.

   • Compensatory Mechanisms for Lost RBC Function:

     • Tachycardia, tachypnea, cool and clammy skin, amenorrhea.

     • Dyspnea, chest discomfort, cellular acidosis, headache, vertigo, pallor, constipation, difficulty concentrating, decreased bowel sounds.

5. Specific Anemic Disorders

5.1 Hypovolemic Anemia

1. Pathophysiology & Etiology

   • Results from loss of circulating intravascular blood volume.

   • Acute severe loss: Trauma, gunshot wounds.

   • Chronic loss: Peptic ulcer disease, uterine fibroids, bleeding hemorrhoids.

   • Bone marrow responds by accelerating RBC production, generating microcytic, hypochromic RBCs with inadequate heme content.

2. Pharmacologic Considerations

   • Low-dose aspirin, NSAIDs, and COX-2 inhibitors increase GI bleeding risks.

   • Risk multiplies significantly when taken concurrently with SSRI antidepressants.

   • Corticosteroids, aldosterone antagonists, and anticoagulants also elevate bleeding risk when taken with aspirin or NSAIDs.

3. Assessment & Diagnostic Findings

   • Acute: Signs of hypovolemic shock (marked pallor, severe tachycardia, hypotension, oliguria, altered level of consciousness).

   • Chronic: Postural hypotension, fatigue, pallor, chills, rapid pulse and respiration.

   • Labs: Decreased RBC count, low hemoglobin and hematocrit, elevated reticulocyte count, reduced MCV and MCHC.

4. Medical & Nursing Management

   • Treatment: Transfusions for acute severe loss; treating underlying cause in chronic loss; oral/IV/IM iron therapy; supplemental oxygen.

   • Nursing Care:

     • Activity Intolerance: Limit nonessential activities, rest periods, supplemental $O_2$ for tachycardia/tachypnea. Targets: HR $< 100\text{ beats/min}$, RR $< 28\text{ breaths/min}$.

     • Hypovolemia: Monitor CBC, check vital signs every $2-4\text{ hours}$; report systolic $BP < 90\text{ mm Hg}$ or HR $> 100\text{ beats/min}$; track I&O every shift/hour; report urine output $< 30-50\text{ mL/h}$; test stool for occult blood; apply pressure to bleeding sites; increase IV flow rate if bleeding profusely; modified Trendelenburg for shock; prep for upper endoscopy.

     • Impaired Gas Exchange: Continuous oximetry; report $SpO_2 < 90\%$; administer oxygen per nasal cannula or mask.

     • Thermal Injury Risk: Draft prevention, warm blankets, increased room temp/humidity, warm fluids. Target temp: $98.6^\circ\text{F} \pm 1^\circ\text{F}$.

     • Knowledge Deficiency: Educate on aspirin/NSAID mucosal damage (prostaglandin inhibition). Enteric coating/buffering does not eliminate risk. Teach use of proton pump inhibitors (omeprazole) to keep gastric pH $\geq 6$, $H_2$ blockers (famotidine), or COX-2 inhibitors (celecoxib). Take NSAIDs with food or milk; avoid alcohol combination.

5.2 Iron-Deficiency Anemia

1. Pathophysiology & Etiology

   • Depletion of iron stores leads to inadequate hemoglobin synthesis and microcytic, hypochromic erythrocyte formation.

   • Causes: Heme loss from bleeding, poor intake, malabsorption, increased demand (pregnancy, growth spurts, menses).

   • Diets absorb $< 10\%$ of ingested iron. Depletion forces cells into inefficient anaerobic metabolism.

2. Assessment & Medical Management

   • Manifestations: Fatigue, cold sensitivity, exertion dyspnea, resting tachycardia, decreased serum iron, low Hb/Hct.

   • Management: Address underlying cause, dietary enhancement, oral or parenteral iron, blood transfusions for severe cases.

3. Pharmacologic & Dosing Considerations

   • Oral iron supplements should be administered every other day (rather than daily or twice daily).

   • Excess iron stimulates hepcidin (a liver hormone suppressing dietary iron absorption). Alternate-day dosing keeps hepcidin levels lower, maximizing absorption efficiency and reducing GI adverse effects.

4. Nutrition Notes 31-1

   • Heme Iron: Beef, pork, lamb, egg yolks, oysters, dark meat poultry. High absorption rate, unaffected by other dietary factors. Recommend 3 lean meat servings weekly.

   • Nonheme Iron: Enriched grains, fortified cereals, legumes, nuts.

   • Enhancers: Vitamin C (citrus, strawberries, peppers, tomatoes) and heme foods.

   • Inhibitors: Tea, coffee, and wheat bran taken during meals.

5. Nursing Guidelines 31-1 & Patient Education

   • Dilute liquid oral iron with juice and drink with a straw to avoid tooth discoloration.

   • Take on empty stomach unless GI upset occurs; do not take with antacids.

   • Inform client that stools will turn dark green or black.

   • Z-Track IM Technique: Select 1.5 to 2-inch needle, draw $0.2\text{ mL}$ air seal, pull tissue laterally $1\text{ inch}$, insert at $90^\circ$, aspirate, inject slowly, hold needle for $10\text{ seconds}$, release skin, apply direct pressure without rubbing.

5.3 Sickle Cell Disease

1. Pathophysiology & Genetics

   • Autosomal recessive genetic disorder affecting $1\text{ in } 365$ Black infants; also prevalent in Mediterranean and Middle Eastern populations.

   • Replacement of normal Hemoglobin A (HbA) with abnormal Hemoglobin S (HbS) (HbF persists until 6 months of age).

   • Hypoxia, cold, infection, or dehydration triggers polymerization of HbS into crystal rods, changing biconcave RBCs into rigid, sticky, sickle shapes.

   • Repeated sickling causes vascular occlusion, tissue ischemia, acute pain, and premature hemolysis (shortened RBC lifespan).

   • Heterozygous carriers (Sickle Cell Trait) possess $\sim 40\%$ HbS and are generally asymptomatic.

2. Assessment Findings & Complications

   • Vaso-occlusive painful crises, joint swelling, fever, tissue infarction.

   • Risk of cerebrovascular accident (stroke), acute chest syndrome (pulmonary infiltrates, cough, wheezing, chest pain, tachypnea), priapism, leg ulcers, functional asplenia, pneumococcal infections.

   • Accelerated RBC hemolysis produces hyperbilirubinemia, jaundice, gallstones, and splenomegaly/infarction.

3. Diagnostics & Medical Management

   • Sickledex screening test; Hemoglobin electrophoresis (differentiates trait vs disease); elevated secretory phospholipase $A_2$ signals impending acute chest syndrome.

   • Voxelotor (Oxbryta): Inhibits cell sickling and clumping.

   • Crizanlizumab-tmca (Adakveo): IV therapy reducing pain crisis frequency.

   • Hydroxyurea (Hydrea): Stimulates Fetal Hemoglobin (HbF) production. Handling rules: Wear gloves, do not open capsules, wash spills 3 times with detergent.

   • Inhaled Nitric Oxide: Vasodilating agent promoting oxygen-hemoglobin binding.

   • Transfusions & Iron Chelation: Transfusions reduce stroke risk; Deferoxamine (Desferal) removes excess iron from multiple transfusions.

   • Bone Marrow / Stem Cell Transplant: Curative potential ($85\%$ cure rate in matched sibling donors).

   • Pain Management: Scheduled IV opioids/PCA, buprenorphine, nalbuphine, oxygen therapy, aggressive hydration.

4. Client Teaching & Evidence-Based Practice

   • Teaching: High fluid intake, warm clothing, avoid cold drafts/high altitudes, avoid tight clothing/smoking, pneumococcal and H. influenzae immunizations, genetic counseling.

   • Evidence-Based Practice (Ambrose et al., 2023): Hydroxyurea dose escalation effectively reduces primary stroke risk in children with sickle cell anemia in resource-limited settings.

5.4 Hemolytic Anemia

1. Pathophysiology & Etiology

   • Premature erythrocyte destruction caused by cardiopulmonary bypass, heavy metals (lead, arsenic), malaria, chemical toxins, or iso-/autoantibodies (transfusion reactions or autoimmune disease).

2. Assessment Findings & Diagnostics

   • Jaundice, splenomegaly, severe anemia, potential hypovolemic shock.

   • Radioactive chromium study shows RBC lifespan $\leq 10\text{ days}$. Positive direct Coombs test (direct antiglobulin test) indicates antibody-mediated hemolysis.

3. Management

   • Remove causative agent, administer corticosteroids, blood transfusions, or perform splenectomy if unresponsive to medical therapy.

5.5 Thalassemias

1. Pathophysiology & Types

   • Hereditary hemolytic anemias divided into Alpha- and Beta-thalassemias.

   • Cooley Anemia: Severe beta-thalassemia causing bronzing of skin (excess hemolysis/iron) and severe anemia.

2. Management

   • Symptomatic treatment with frequent blood transfusions.

   • Iron Chelation Therapy: Removes trapped excess iron to prevent organ failure. Administer IV/SubQ Deferoxamine (Desferal), or oral Deferiprone (Ferriprox) / Deferasirox (Exjade).

5.6 Pernicious Anemia

1. Pathophysiology & Etiology

   • Lack of Intrinsic Factor (produced by stomach mucosa) prevents absorption of Vitamin $B_{12}$ (Extrinsic Factor) in the terminal ileum.

   • Etiology: Gastric mucosal atrophy, aging, gastrectomy, bariatric surgery, ileal resection, strict vegan diets lacking animal products.

   • Impaired $B_{12}$ leads to megaloblastic RBC production and progressive neurological degeneration.

2. Assessment Findings & Diagnostics

   • Glossitis (smooth, beefy red tongue), stomatitis, diarrhea, dyspnea, jaundice, paresthesias (numbness/tingling in hands/feet), motor ataxia, loss of vibratory/position sense, confusion, depression.

   • Schilling test confirms diagnosis. Blood smear shows large, immature megaloblastic erythrocytes.

3. Medical & Nursing Management

   • Lifelong IM Vitamin $B_{12}$ injections ($100\text{ }\mu\text{g}$ daily for 2 weeks, then $100\text{ }\mu\text{g}$ monthly). Oral $B_{12}$ is generally ineffective.

   • Nursing: Soft, bland diet; meticulous post-meal oral hygiene; physical therapy; fall prevention/assistance with ambulation.

5.7 Folic Acid-Deficiency Anemia

1. Pathophysiology & Nutrition Notes 31-2

   • Deficiency in Folate (Vitamin $B_9$) causing megaloblastic anemia. Unlike $B_{12}$ deficiency, folic acid deficiency does NOT cause irreversible neurological damage.

   • Causes: Poor diet, chronic alcoholism, malabsorption, folic acid antagonists (methotrexate, anticonvulsants), pregnancy, hemolysis.

   • Food sources: Green leafy vegetables, citrus/orange juice, fortified grains, dried beans/peas.

2. Assessment & Management

   • Sore beefy red tongue, fatigue, dyspnea, nausea, weakness, decreased serum folate.

   • Management: Oral folic acid ($1\text{ mg/day}$) or parenteral folate for malabsorption disorders.

6. Erythrocytosis: Polycythemia Vera

1. Pathophysiology & Etiology

   • Unregulated marrow proliferation of erythrocytes, leukocytes, and platelets. (High altitude erythrocytosis is physiologic, but Polycythemia Vera is neoplastic).

   • RBC destruction releases intracellular potassium (hyperkalemia) and uric acid (goutlike symptoms).

   • Blood hyperviscosity leads to hypertension, heart failure, stroke, and deep vein thrombosis.

2. Assessment Findings

   • Reddish-purple face/lips, fatigue, severe pruritus, exertional dyspnea, dizziness, splenomegaly, gouty painful joints, elevated RBC/WBC/platelet counts, hyperkalemia, hyperuricemia.

3. Medical & Nursing Management

   • Therapeutic phlebotomy ($500\text{ mL}$ removed several times weekly).

   • Anticoagulants, radiophosphorus, antineoplastics (mechlorethamine).

   • Nursing: Fluid intake of at least $3\text{ quarts (3 L) per day}$; avoid crossing legs or tight clothing; encourage movement and leg elevation; perform isometric quadriceps/gluteal exercises; wear thromboembolic support hose; rest immediately if chest pain occurs.

7. Leukocytosis and Leukemias

7.1 Leukemia Types & Classification (Table 31-3)

1. Acute Lymphocytic Leukemia (ALL): Immature lymphocytes; onset $< 5\text{ years}$, uncommon after $15$.

2. Chronic Lymphocytic Leukemia (CLL): Immature/abnormal lymphocytes; onset $> 40\text{ years}$, most common adult leukemia.

3. Acute Myelogenous Leukemia (AML): Proliferation of immature myeloid cells; affects all age groups.

4. Chronic Myelogenous Leukemia (CML): Proliferation of myeloid line; onset $> 20\text{ years}$, associated with Philadelphia chromosome genetic marker in $90\%-95\%$ of cases.

7.2 Stem Cell Transplant Types (Table 31-4)

1. Adult Stem Cells: Harvested via bone marrow aspiration, peripheral apheresis, or umbilical cord blood. Autologous (self), Syngeneic (identical twin), Allogeneic (donor).

2. Embryonic Stem Cells: Harvested from $4-5\text{ day-old}$ blastocysts; pluripotent; restricted to research.

7.3 Nursing Process for Leukemia

1. Assessment & Diagnostics

   • Weakness, fatigue, frequent infections, epistaxis, joint pain, CNS infiltration symptoms (headache, confusion), lymphadenopathy, hepatosplenomegaly.

2. Absolute Neutrophil Count (ANC) Calculation (Box 31-2)    $\text{ANC} = \frac{\text{WBC} \times (\%\text{ neutrophils} + \%\text{ bands})}{100}$

   • $\text{ANC } 500-999$: Infection risk.

   • $\text{ANC } 100-499$: High risk for infection.

   • $\text{ANC } < 100$: Severe/almost certain infection.

3. Neutropenic Precautions (Box 31-3)

   • Private room; strict hand hygiene.

   • Daily showering; mask worn by client outside room.

   • No raw fruits, raw vegetables, or cut flowers.

   • Minimize invasive procedures; cluster blood draws.

4. Bleeding Risk & Patient Education (Client Teaching 31-2)

   • Monitor platelet count; inspect skin for petechiae/bruising; report epistaxis, melena, or hematuria.

   • Use electric razors and soft toothbrushes; apply prolonged pressure to puncture sites.

   • Educate on strict drug schedules, prompt medical evaluation for fever/bleeding/mouth sores, avoiding ill contacts.

8. Multiple Myeloma

1. Pathophysiology & Etiology

   • Malignancy of plasma cells (B-lymphocytes) releasing osteoclast-activating factor, triggering osteoclasts to break down bone tissue.

   • Produces osteolytic "punched-out" or "honeycombed" lesions in spine, ribs, skull, pelvis, femurs. Vertebral collapse causes compression and severe pain.

   • Bence Jones proteins damage renal tubules during excretion, leading to kidney failure.

   • M-type globulin suppresses normal antibody production.

   • CRAB Acronym:

     • C: Calcium elevated (hypercalcemia)

     • R: Renal insufficiency

     • A: Anemia

     • B: Bone lesions

2. Drug Therapy Options (Drug Therapy Table 31-1)

   • Chemotherapy (Cell Cycle-Specific): Vincristine, Etoposide.

   • Chemotherapy (Cell Cycle-Nonspecific): Cyclophosphamide, Melphalan, Doxorubicin, Bendamustine.

   • Corticosteroids: Dexamethasone, Prednisone (Inhibit inflammation; give with food, monitor blood glucose).

   • Immunomodulators: Lenalidomide, Pomalidomide, Thalidomide (Severe birth defects; administered under strict FDA REMS program; DVT risk requires aspirin/warfarin prophylaxis).

   • Proteasome Inhibitors: Bortezomib, Carfilzomib, Ixazomib.

   • HDAC Inhibitor: Panobinostat.

   • Monoclonal Antibodies: Daratumumab, Elotuzumab.

   • Bisphosphonates: Pamidronate, Zoledronic acid (Reduce bone loss; mandatory dental check/repair prior to therapy to prevent osteonecrosis of the jaw).

   • Allopurinol (Zyloprim): Prevents uric acid renal calculi.

3. Nursing Priorities

   • Maintain fluid intake up to $4,000\text{ mL/day}$ to prevent renal tubule damage from hypercalcemia and protein precipitation.

   • Safety and gentle handling during transfers to prevent pathologic fractures.

   • Administer analgesics prior to movement/bathing.

9. Agranulocytosis and Pancytopenia

1. Agranulocytosis

   • Severe reduction in granulocytes (neutrophils, basophils, eosinophils).

   • Etiology: Drug toxicity (sulfonamides, chloramphenicol, antineoplastics, psychotropics).

   • Assessment: Fever, chills, severe fatigue, opportunistic oral/rectal/vaginal infections.

   • Management: Withdraw offending drug; administer Filgrastim (Neupogen) or Pegfilgrastim (Neulasta); enforce neutropenic precautions.

2. Pancytopenia & Aplastic Anemia

   • Bone marrow failure leading to simultaneous deficiency of erythrocytes, leukocytes, and platelets.

   • Etiology: Autoimmune stem cell destruction, chemical toxins, radiation, medications.

   • Assessment: Severe anemia symptoms, opportunistic infections, petechiae, ecchymoses, splenomegaly. Bone marrow aspiration shows suppressed stem cell production.

   • Management: Withdraw causative agent, whole blood/packed RBC/platelet transfusions, high-dose corticosteroids, bone marrow / autologous stem cell transplantation, laminar airflow isolation, soft food diet, gentle oral hygiene.

10. Coagulopathies

1. Thrombocytopenia

   • Deficiency of platelets/thrombocytes caused by decreased production or splenic destruction. Idiopathic Thrombocytopenia Purpura (ITP) has an unknown etiology. Drug causes include heparin.

   • Manifestations: Purpura, petechiae, mucosal bleeding, epistaxis, GI bleeding, fatal internal hemorrhage risk.

   • Management: Eliminate cause, corticosteroids, platelet transfusions, splenectomy (creates lifelong infection risk due to loss of bacterial filtration).

2. Hemophilia

   • Classification:

     • Hemophilia A: Deficiency of Factor VIII (most common; includes von Willebrand disease variant).

     • Hemophilia B: Deficiency of Factor IX (Christmas disease).

     • Hemophilia C: Deficiency of Factor XI (Rosenthal disease).

   • Genetics: X-linked recessive disorder passed from mothers to male offspring.

   • Manifestations: Persistent oozing, spontaneous bleeding, hemarthrosis (bleeding into joints resulting in permanent deformity/limitation of motion).

   • Medical Management: Recombinant Factor VIII, IX, or XI concentrates; Aminocaproic acid (Amicar) to hold clots; fresh frozen plasma; topical thrombin/fibrin.

   • Client Teaching 31-3:

     • Eliminate aspirin and NSAIDs completely.

     • Avoid activities resulting in injury; wear MedicAlert bracelet.

     • Use soft toothbrush, rinse with warm water.

     • Support painful joints on pillows.

     • Take temperature via temporal or tympanic route (avoid oral/rectal trauma).