Hematologic Disorders- Level 2

Hematologic Disorders

Katie Faehl, MSN, RN
1443 / Spring 2025 / HLC Level 2

Hematologic Disorders

Anemias (many)
Hemochromatosis
Polycythemia
Thrombocytopenia
Neutropenia
Lymphoma
Multiple Myeloma

Hematology: Lab Values to Know (pre-lecture prep work)

Hemoglobin
Hematocrit
White Blood Cell Count
Red Blood Cell Count
Platelet Count

Hematology: Lab Vocabulary (pre-lecture prep work)

Hgb
Hct
Macrocytic
Microcytic
MCV
MCH
MCHC
Platelet Count
Neutrophil Count

Anemia

Anemia: Clinical Setting Questions

Have you taken care of patients who had anemia or were at risk of developing anemia?
What caused it?
What conditions or diagnoses did they have?
What clinical manifestations did they display?

Anemia

A deficiency in:
- Number of erythrocytes (RBCs)
- Quantity or quality of hemoglobin (Hgb)
- Volume of packed RBCs (hematocrit)

Anemia

Anemia = tissue hypoxia
$↓$ RBCs can occur for three reasons
- They are lost from the body
- Not enough of them are being produced
- Too many of them are being destroyed

Types of Anemias

Decreased RBC Production
- Nutritional Deficiencies
  - Iron
  - Vitamin B12
  - Folic Acid
- Thalassemia
- Anemia of Chronic Disease
- Aplastic Anemia
Blood Loss
- Acute vs Chronic
Increased RBC Destruction
- Acquired Hemolytic Anemia

Anemia

Mild Anemia
- Hgb 10-12 g/dL
Moderate Anemia
- Hgb of 6-10 g/dL
Severe Anemia
- Hgb of < 6 g/dL
Clinical Pearl
- Transfusions are considered when Hgb < 7

Anemia: Clinical Manifestations

Integumentary
- Pallor
- Jaundice
- Pruritus
Neuro
- Headache
- Irritability
- Vertigo
- Dizziness

Anemia: Clinical Manifestations

GI
- Glossitis (swollen/inflamed tongue)
- Hepatomegaly
- Splenomegaly
Generalized symptoms
- Cold sensitivity
- Fatigue

Anemia: Cardiopulmonary Manifestations

Result from heart and lungs trying to provide adequate O2 to tissues of the body
Lack of O2 in tissues of heart & lungs
Strain on heart and lungs
- Cardiac
  - Output maintained by $↑$ HR & stroke volume
  - Palpitations, bounding pulse, tachycardia
  - MI (extreme cases)
- Pulmonary
  - Dyspnea & tachypnea

Anemia: Decreased RBC Production

Iron, B12 & Folate Deficiencies

Iron-Deficiency Anemia

Iron
- essential for hemoglobin synthesis
- absorbed in the small intestine
  - duodenum & upper jejunum

Iron Deficiency Anemia

Causes
- Inadequate dietary intake
- Malabsorption
- Blood loss
- blood hemolysis
Labs
- $↓$ Hgb/Hct
- $↓$ Serum Iron Levels
- $↑$ TIBC (Total Iron Binding Capacity)

Iron Deficiency Anemia: Clinical Manifestations

General manifestations of anemia (+)
- Pallor
- Glossitis
- Cheilitis
  - Inflammation of lips
- Headache
- Paresthesias

Iron Deficiency Anemia: Interprofessional & Nursing Management

Iron Replacement
- Nutrition Therapy
  - Liver, lean beef, turkey, pork, chicken, fish, legumes (beans), dark green leafy vegetables, whole-grain & enriched bread & cereals

Iron Deficiency Anemia: Interprofessional & Nursing Management

Iron Replacement
- Iron Supplements (ferrous sulfate)
  - Oral iron supplements
    - nursing considerations?
  - IV or IM
    - malabsorption
    - need for high doses
    - intolerance/poor adherence to oral regimen
- PRBCs

Megaloblastic Anemias

Impaired DNA synthesis of RBCs $\rightarrow$
- Defective maturation of RBCs
- RBCs are macrocytic & abnormal
  - termed megaloblasts
- RBCs with fragile cell membranes
Most are due to deficiencies of
- Cobalamin (Vitamin B12)
- Folic Acid

Megaloblastic Anemia: Cobalamin (Vit B12) Deficiency

Cobalamin = Vitamin B12 = Extrinsic Factor
Required for DNA synthesis of RBCs
Absorbed in the ileum
Requires Intrinsic Factor (IF)

Cobalamin (Vitamin B12) Deficiency

Causes
- ↓ gastric Intrinsic Factor (IF) = ↓ B12 absorption
  - Pernicious anemia

Cobalamin (Vitamin B12) Deficiency

Causes
- $↓$ gastric Intrinsic Factor (IF) = $↓$ B12 absorption
  - Pernicious anemia
  - GI surgery
  - Malabsorption
- Dietary issues
Labs
- $↓$ Hgb/Hct
- $↑$ MCV
- $↓$ Serum B12

Vit B12 Deficiency: Clinical Manifestations

General Anemia symptoms (+)
- GI symptoms
  - sore, red, beefy, shiny tongue (glossitis)
  - anorexia
  - abdominal pain, nausea & vomiting
- Neuromuscular symptoms
  - weakness / muscle weakness
  - paresthesias
  - ataxia
  - impaired cognition

Vit B12 Deficiency: Interprofessional & Nursing Management

Vitamin B12 Replacement
- Parenteral (IM) or intranasal
  - Treatment of choice
  - cyanocobalamin, hydroxocobalamin

Megaloblastic Anemia: Folic Acid Deficiency

Folic Acid
- required for DNA synthesis of RBCs
- absorbed in the duodenum & upper jejunum

Folic Acid Deficiency

Causes
- Alcohol abuse
- Chronic hemodialysis
- Dietary deficiency
- Medication interactions (ex: phenytoin)
- Malabsorption
Labs
- $↓$ Hgb/Hct
- $↑$ MCV
- $↓$ Folate levels
- Normal B12

Folic Acid Deficiency: Clinical Manifestations

Similar to B12 deficiency (+)
- GI symptoms
  - stomatitis
  - dysphagia
  - flatulence
  - diarrhea

Folic Acid Deficiency: Interprofessional & Nursing Management

Folic Acid Replacement
- Nutrition
  - Green leafy vegetables, enriched grain products and breakfast cereals, orange juice, peanuts, avocado, asparagus & beans
- Supplements
  - Folate 1mg PO daily
  - vs
  - Folate 5mg PO daily

Anemia: Decreased RBC Production

Thalassemia Major & Thalassemia Minor

Thalassemia

A group of diseases involving inadequate production of normal Hgb
- Decreased RBC production
- Due to an absent or reduced globulin protein
- Autosomal recessive genetic basis

Thalassemia Minor

Thalassemia Trait
Often asymptomatic
Mild to moderate anemia
Mild additional symptoms
Body adapts

Thalassemia Major

Life-threatening
Growth & development deficits
General anemia symptoms (+)
- Jaundice
- Splenomegaly
- Bone marrow hyperplasia

Thalassemia Major: Interprofessional Care

Blood transfusions or exchange transfusions
(+) iron chelation therapy for life
- Chelating agents
  - IV or PO
  - Bind iron $\rightarrow$ excreted in the kidneys
No iron supplements
Splenectomy
Stem Cell Transplant

Anemia: Decreased RBC Production

Aplastic Anemia

Pancytopenia ( $↓$ RBCs, WBCs & platelets)
- Causes
  - Autoimmune
  - Acquired
    - Toxic injury to bone marrow stem cells
  - Inherited stem cell defect

Aplastic Anemia

Clinical Manifestations
- General manifestations of anemia (+)
- Neutropenia
- Thrombocytopenia
Labs
- $↓$ Hgb/Hct
- $↓$ WBC’s
- $↓$ Platelets

Aplastic Anemia: Interprofessional & Nursing Management

Treatment
- Identify and remove causative agent
- Hematopoietic Stem Cell Transplant (HSCT)
- Immunosuppressive therapy

Aplastic Anemia: Interprofessional & Nursing Management

Supportive care
Prevent complications
- infection
- bleeding
Monitor
- Labs: RBCs, WBCs & Platelets
- Vitals: Temperature
- Bleeding / bruising

Anemia: Blood Loss

Acute Blood Loss & Chronic Blood Loss

Anemia: Acute Blood Loss

Caused by sudden hemorrhage
- Trauma
- Surgery complications
- Blood vessel rupture
Clinical concerns
- Hypovolemic shock
- Compensatory increased plasma volume with diminished O2-carrying RBCs

Acute Blood Loss: Clinical Manifestations

Based on % of total blood volume lost
Mild to severe
Tachycardia, hypotension, decreased cardiac output, thready pulse, shock, organ failure & death
Pain
- Internal bleeding
- Retroperitoneal bleeding

Acute Blood Loss: Interprofessional & Nursing Management

Replace blood volume
- prevent shock
Find source & stop blood loss
Postoperative patients
- Monitor blood loss
- Pain assessments
- Give blood products for anemia
No need for long-term treatment

Anemia: Chronic Blood Loss

Sources of chronic blood loss
- Bleeding ulcer
- Hemorrhoids
- Menstrual and postmenopausal blood loss
Management involves
- Identifying the source / stop bleeding
- Providing supplemental iron as needed

Anemia: Increased RBC Destruction

Acquired Hemolytic Anemia.

Acquired Hemolytic Anemia

RBC destruction (hemolysis) > RBC production
RBCs normal but external factors damage them
Main Causes
- Physical destruction
- Antibody reactions
- Infections

Acquired Hemolytic Anemia

Clinical Manifestations
- General anemia symptoms (+)
- Jaundice
- Spleen & liver enlargement
Treatment Focus
- Supportive care until causative agent removed
- Renal function

Hemochromatosis

Iron Overload

Hemochromatosis

Iron overload disorder characterized by increased intestinal iron absorption
Etiology
- Iron accumulated at an increased rate
  - $\rightarrow$ Iron toxicity
- Genetic defect
- Chronic blood transfusions

Hemochromatosis Clinical Manifestations

Early: fatigue, joint pain, abdominal pain, weight loss
Later: liver enlargement, skin pigment changes (bronzing), organ failure Diagnostics
$↑$ serum iron, $↓$ TIBC
Genetic testing

Hemochromatosis: Treatment

Goal
- Remove excess iron
- Minimize symptoms
Iron removal
- Via phlebotomy
  - Blood removal weekly
  - Until iron stores depleted
  - Then less often
- Iron chelation therapy

Polycythemia

Circulation Overload

Polycythemia Vera

Primary Polycythemia = Polycythemia Vera
Chronic myeloproliferative disorder
Production & presence of $↑$ RBCs, $↑$ WBCs & $↑$ Platelets
- Causes impaired circulation
  - Hyperviscosity
  - Hypervolemia

Polycythemia Vera

Congestion in tissues and organs
Blood vessel distention
Impaired blood flow
Circulatory stasis
Thrombosis
Tissue hypoxia

Polycythemia: Clinical Manifestations

HTN
HA, dizziness, visual changes
Plethora (ruddy complexion)
Bleeding
Most common serious complication
Stroke due to thrombosis embolism

Polycythemia: Interprofessional & Nursing Management

Treatment Goal
- $↓$ blood volume
- $↓$ blood viscosity
Phlebotomy
Hydration therapy
Aspirin

Thrombocytopenia

Reduction in platelets below 150,000/μL
- Range in severity
- Platelet disorders caused by
  - Impaired production
  - Increased destruction
  - Abnormal distribution
  - Inherited
  - Usually acquired

Immune Thrombocytopenic Purpura (ITP) Thrombotic Thrombocytopenic Purpura (TTP) Heparin-Induced Thrombocytopenia (HIT)

Acquired autoimmune disease
Platelets get coated with antibodies
Platelets destroyed by the spleen Treatment
- Corticosteroids
- Immunosuppressants
- IVIG
- Splenectomy
Caused by
- autoimmune disorders
- drug toxicity
Platelets stick together & form micro-clots.
Clots deposit into vasculature
Platelets can’t stop bleeding Treatment
- Identify cause
- Plasmapheresis
Immune-mediated response to heparin
Destruction of platelets
Formation of platelet- fibrin thrombi (clots) Treatment
- Discontinue all heparin
- Thrombin inhibitors

Thrombocytopenia: Clinical Manifestations

Bleeding
- Nose, mouth, gums
- Petechiae, purpura, ecchymoses
- Prolonged bleeding with injections
Major Complications
- Hemorrhage
- Cerebral hemorrhage

Thrombocytopenia: Clinical Manifestations

Internal Bleeding
- Weakness
- Fainting
- Dizziness
- Abdominal pain
- $↓$ BP, $↑$ HR
Clotting Concerns (HIT)
- DVT
- CVA
- PE

Thrombocytopenia: Nursing Management

Acute Care
- Confirm no aspirin, heparin, blood thinners
- Monitor labs
  - platelets, coags, Hgb, Hct
- SQ / No IM
- Safety
- Platelet transfusions
- Vitals
- Assessments

Thrombocytopenia: Patient Teaching

Notify HCP of any bleeding
Fall prevention
Avoid nose blowing / nosebleed care
Prevent constipation
Only electric razors
No aspirin, NSAIDS or blood thinners
Soft toothbrush
Menstrual flow: count pads / no tampons

Neutropenia

Neutrophil count < 1000
- Severe neutropenia < 500
Causes
- Chemotherapy & immunosuppressive medications
Predisposition to infection
- Opportunistic pathogens
- Nonpathogenic organisms
Typical signs/symptoms of infection may be absent

Neutropenia: Infection

Common entry points
- mucous membranes of mouth/throat, skin, perianal area & pulmonary system
Clinical Manifestations
- sore throat, mouth lesions, diarrhea, rectal tenderness, vaginal itching/discharge, SOB, NP cough, new pain
- fever (>100.4), chills

Neutropenia: Fever Management

Neutropenia with fever > 100.4
What should you anticipate?
- Cultures
- Start antibiotics within 1 hour
- Ongoing assessment & monitor vital signs
- Neutropenic precautions

Neutropenia: Nursing Management & Patient Education

Hand hygiene
Assessment
Social isolation
Diet
Patient hygiene
Other home considerations

Lymphomas

Hodgkin's & Non-Hodgkin's

Lymphomas

Cancers that originate in the bone marrow & lymphatic structures
Results in proliferation of lymphocytes
Hodgkin’s vs Non-Hodgkin’s lymphoma

Hodgkin’s Lymphoma

Long term survival ~ 85%
Clinical Manifestations
- Reed-Sternberg cells
- Enlarged, non-tender, moveable lymph node
- Starts in one node and spreads to others
- Usually starts in the cervical, axillary, inguinal or mediastinal nodes

Hodgkin’s: Clinical Manifestations

General Symptoms
- Weight loss
- Fatigue & weakness
- Fever & chills
- Tachycardia
- Night sweats
- Pain at site of disease with ETOH

Hodgkin’s: Clinical Manifestations

“B Symptoms”
- Worse prognosis
  - Fever > 100.4 &
  - Drenching night sweats &
  - Weight loss

Hodgkin's Lymphoma

Diagnostics

CBC
Lymph node excision & biopsy
Bone marrow exam
Radiological studies

Hodgkin’s Lymphoma

Interprofessional Management
- Chemo
- Radiation
- Curative treatment (once in remission)
  - Intense chemo
  - (+) stem cell transplant

Non-Hodgkin’s Lymphomas (NHL)

Group of cancers involving B, T, & natural killer cells
- Affects all ages
- No Reed-Sternberg cells
- “B symptoms” uncommon
- Lymphocytes arrested in various states of development
- Disseminated & unpredictable method of spread

NHL: Clinical Manifestations

Painless lymph node enlargement
- Primary manifestation
- Lymph node enlargement can wax and wane
Symptoms based on location of spread of disease
High grade lymphomas
- May have “B symptoms”

Non-Hodgkin’s Lymphomas

Diagnostics
- Lymph node biopsy
- MRI, lumbar puncture, bone marrow exam
Tumor Clinical Behavior
- Indolent vs aggressive
- More aggressive lymphomas respond better to treatment

Non-Hodgkin's Lymphomas

Interprofessional Management

Chemotherapy
Biotherapy
Radiation

Lymphomas: Nursing Management

Focus on problems related to the disease and side effects of therapy
- Chemo & radiation
- Pain
- Monitor for pancytopenia
- Tumor lysis syndrome
- Superior vena cava syndrome
- Non-Hodgkin’s lymphoma more extensive

Lymphoma Stages

Stage I
- Localized disease; single lymph node region or single organ
Stage II
- Two or more lymph node regions on the same side of the diaphragm
Stage III
- Two or more lymph node regions above and below the diaphragm
Stage IV
- Widespread disease; multiple organs, with or without lymph node involvement

Multiple Myeloma

Cancer of the Plasma Cells

Multiple Myeloma (MM)

Condition where cancerous plasma cells proliferate in the bone marrow and destroy bone
- Excessive production of mutated plasma cells
- Pathologic process of bone destruction
- Bence-Jones Proteins

Multiple Myeloma: Clinical Manifestations

No early symptoms
Skeletal pain
Osteoporosis
Bone degeneration $\rightarrow$ hypercalcemia
- $\rightarrow$ renal, GI, neuro & heart complications
Hyperviscosity syndrome
- $\rightarrow$ Renal tubular obstruction
Anemia, thrombocytopenia, neutropenia, immune dysfunction

Multiple Myeloma: Diagnostics

Labs
- Bence-Jones protein in the urine
- Pancytopenia
- $↑$ calcium
- $↑$ creatinine
Radiologic
- MRI, PET & CT Scans
Bone marrow exam

MM: Interprofessional Management

Chemotherapy & corticosteroids
Stem cell transplant
Immunotherapy
Targeted therapy
Bisphosphonates
furosemide
allopurinol

Multiple Myeloma: Nursing Management

Safety
Ambulation, weight bearing exercise
Pain management
Hydration therapy
Infection risk
Psychosocial concerns