Module 8B: Hematology/Immunology Part 2

What is the most severe kind of sickle cell disease?

sickle cell anemia

What is the etiology of sickle cell anemia?

Inherited, autosomal recessive disorder; Characterized by an abnormal form of Hgb in the RBC

What triggers a sickling episode in sickle cell disease?

Primary Trigger: Low oxygen tension in blood

Most Common Factor: Infection

Other Triggers: Stress, dehydration, trauma

Is sickling reversible in a sickle cell crisis?

initially, yes with reoxygenation

Severe, painful acute exacerbation of sickling causing vaso-occlusive crisis

Sickle cell crisis

What are complications of a sickle cell crisis?

Tissue necrosis

Life-threatening shock (possible)

What does HOP stand for in sickle cell crisis management?

H - Hydration

O - Oxygenation

P - Pain management

What are the key prevention strategies for patients with sickle cell disease?

Vaccines (prevent infection)

Hydration (prevent dehydration)

Infection prevention (infection is most common trigger)

Repeated sickling weakens RBC membrane → RBC destruction → intravascular hemolysis →leads to?

Jaundice

What kind of pain relief will patients in a sickle cell crisis need?

opioids

What are the key manifestations of sickle cell disease (outside of crisis)?

PAIN

Pallor (assess eyes and mucous membranes)

Jaundice

Extreme fatigue

How will vital signs change in a sickle cell crisis?

Increased RR, HR, temperature

Where should you assess for jaundice in sickle cell patients?

eyes and mucus membranes

What organs in sickle cell disease have the most complications?

those with the highest O2 demands

What is the most common infection in sickle cell disease, and a major cause of mortality?

Pneumococcal pneumonia

What complications can severe infections cause in sickle cell disease?

Aplastic crisis

Hemolytic crisis

Gallstones

Shutdown of RBC production

What are the 5 key signs and symptoms of acute chest syndrome?

Fever

Chest pain

Cough

Lung infiltrates

Dyspnea

What pulmonary complications occur with acute chest syndrome?

Pneumonia

Tissue infarction

Fat embolism

What serious complications can pulmonary infarctions cause in sickle cell disease?

Cardiac: Pulmonary hypertension, MI, cor pulmonale, HF

Other Organs: Retinal detachment and blindness, renal failure, stroke

What is the primary screening test for sickle cell disease?

Newborn screen

What can be visualized on a peripheral blood smear in sickle cell disease?

Reticulocytes

Sickling (sickled RBCs)

Diagnostic test that breaks down different hemoglobin components (Hb S, Hb A, etc.)

hemoglobin electrophoresis

What imaging studies are used to diagnose complications in sickle cell disease?

Skeletal X-rays:Bone or joint deformities

MRI: Assess for stroke

Doppler studies: Assess for DVTs

Chest x-ray: Infection (acute chest syndrome)

What are the key components of medical management for sickle cell disease?

Treat infections

Pain management (opioids)

Folic acid (for chronic hemolysis causing folate depletion)

Reduce sickling episodes (medications)

Prophylactic antibiotics in crisis (even without confirmed infection)

How does hydroxyurea work in sickle cell disease?

Increases fetal hemoglobin (Hb F) concentration

Interferes with sickling process; reducing episodes

What is the most common medication to reduce sickling episodes?

hydroxyurea

What is the only cure for sickle cell disease and when is it used?

HSCT (Hematopoietic Stem Cell Transplant)

Reserved for severe patients (e.g., those who have had strokes)

Why do patients with sickle cell disease need folic acid supplementation?

Chronic hemolysis causes folate depletion

When are prophylactic antibiotics given in sickle cell disease?

sometimes during crisis, even without confirmed infection

What are the key interventions for hospitalized sickle cell patients?

Oxygen (continuous administration)

Pain management (opioids)

Fluids (prevent dehydration)

Antibiotics

VTE prophylaxis

Monitor respiratory status

Possible transfusion therapy

What VTE prophylaxis measures are used for hospitalized sickle cell patients?

SCDs (Sequential Compression Devices)

Sub Q heparin

Why is respiratory status monitoring critical in hospitalized sickle cell patients?

Acute chest syndrome (ACS)

Infection (especially pneumococcal pneumonia)

When might transfusion therapy be used in sickle cell disease?

Hemoglobin <8 g/dL or hematocrit <20%

Severe anemia

Exchange or prophylactic transfusions to increase normal hemoglobin

What is chelation therapy and when is it used in sickle cell disease?

Removes excess iron from the body after repeated blood transfusions

What pain management strategies are used in nursing care for sickle cell patients?

Pharmacologic:

Opioids (primary treatment)

Nonpharmacologic:

Relaxation techniques

Distraction

Heat application

Massage

Guided imagery

Why is hydration critical in sickle cell disease nursing management?

Prevent dehydration, which can trigger sickling episodes

What should you teach patients about avoiding sickle cell crises?

Steps to Avoid Dehydration:

Drink 8-10 glasses of water daily

Increase fluids during hot weather or exercise

Steps to Avoid Hypoxia:

Avoid high altitudes

Avoid strenuous physical activity

Seek prompt treatment for respiratory infections

Other:

Avoid extreme temperatures

Manage stress

Get adequate rest

What key points about medical care should be taught to sickle cell patients and caregivers?

Routine immunizations (especially pneumococcal and influenza)

Importance of prompt medical attention for fever, infection signs, or pain

Pain control strategies and when to seek help

Medication adherence: Hydroxyurea (take daily as prescribed)

Reasons for supportive care (prevent complications, minimize organ damage)

What should patients know about taking hydroxyurea?

Take daily as prescribed

Don't stop without consulting provider

May take 3-6 months to see full benefits

Report side effects

Requires regular lab monitoring

Second most common cause of illness and death in the US; caused by uncontrolled growth of malignant cells

Cancer

What are the top 3 most common cancers in men?

Lung

Prostate

Colorectal

What are the top 3 most common cancers in women?

Breast

Colorectal

Lung

What are the top 3 most common cancers in children?

Leukemia

CNS Tumors

Lymphoma

Where can defects occur that lead to cancer development?

Defects can occur in:

Cell proliferation

Cell differentiation

Causes can be:

Genetic

Hormonal

Viral

What are the three stages of tumor development?

Tumor Initiation: Process that produces initial cancer cells (mutations + microenvironment)

Tumor Promotion: Cancer cell population expands with diversity (additional mutations + changing microenvironment)

Tumor Progression: Spread to adjacent and distal sites/metastasis (further mutations + changing microenvironments)

Cancer that spreads from where it started to a distant part of the body

metastatic cancer

What are the most common sites of metastasis?

Lungs

Liver

Brain

Bones

How is the primary cancer site identified if cancer has already metastasized?

Biopsy differentiation

How does metastasis affect cancer prognosis?

increases risk of death significantly

Arise from specific body organs and grow into masses that invade/erode body tissue as they expand

Solid tumor

Arise from cells of the hematopoietic cell line or from secondary immune organs (lymph nodes, spleen)

Hematological malignancies

How do cancer types differ between adults and children?

Adults: Most common cancers are solid tumors

Lung, prostate, colorectal (men)

Breast, colorectal, lung (women)

Pediatrics: Most common cancers are hematological malignancies

Leukemia (28%)

CNS tumors (25%)

Lymphomas (12%)

What is the purpose of cancer staging?

Considers:

Size of the tumor

Burden of the disease

Extent of disease spread

Used to:

Plan patient assessment

Guide clinical management

Determine treatment strategies

What does TNM stand for in cancer staging?

T = Tumor size and invasiveness

N = Number of lymph nodes involved (regional spread)

M = Presence of metastases (distant organ sites)

What factors are considered when staging solid malignancies?

Anatomic Factors:

Anatomy (location)

Histology (cell type)

Size (tumor dimensions)

TNM Parameters:

Tumor size (T)

Lymph node involvement (N)

Metastasis presence (M)

What are the general cancer stage classifications?

0: cancer in situ

I: localized tumor growth

II: limited local spread

III: extensive local and regional spread

IV: metastasis

When is TNM staging NOT used?

Leukemias and other non-solid tumors

histologic grading of cancer

Grade I: Well-differentiated, slow growth

Grade II: Moderately abnormal, moderate growth

Grade III: Poorly differentiated, rapid growth

Grade IV: Undifferentiated/anaplastic, most aggressive

What are the 7 warning signs of cancer (CAUTION UP)

C - Change in bowel or bladder habits

A - A sore that does not heal

U - Unusual bleeding or discharge

T - Thickening or lump in breast or elsewhere

I - Indigestion or difficulty swallowing

O - Obvious change in wart or mole

N - Nagging cough or hoarseness

U - Unexplained weight loss

P - Persistent fatigue

What are the three main categories of oncology emergencies?

Obstructive: Tumor blocks organs or blood vessels

Metabolic: Hormonal/metabolic alterations from tumor or treatment

Infiltrative: Tumor invades major organs or treatment complications

Tumor obstruction of an organ or blood vessel

Ex:

Spinal cord compression - tumor pressing on spinal cord

Intestinal obstruction - tumor blocking bowel

obstructive oncology emergencies

Production of ectopic hormones from tumor OR metabolic alterations from tumor/treatment

ex: SIADH (Syndrome of Inappropriate Antidiuretic Hormone): Tumor produces excess ADH

metabolic oncology emergencies

Malignant tumors infiltrate major organs OR complications from cancer therapy

ex:

Cardiac tamponade - fluid accumulation around heart compressing it

Carotid artery rupture - tumor erodes into major artery

infiltrative oncology emergencies

What is primary prevention for cancer?

Exercise

Diet

Tobacco cessation

Sunscreen use

What is secondary prevention for cancer?

Colonoscopy

Mammogram

Pap smear

Prostate blood test

What is tertiary prevention for cancer?

Targeted therapies

Palliative care

What is the only definitive way to diagnose cancer?

Biopsy

What are the main methods used to diagnose cancer other than biopsy?

Cytology studies - Examine cells

Radiology - Chest x-ray, CT, MRI, PET scan

Laboratory studies - CBC, chemistry profile, liver function, tumor markers, genetic markers

Endoscopic examinations - Visualize internal organs

Bone marrow examination - For hematologic cancers

What test is most useful in determining the stage of a cancer and can be used to determine the effects of therapy on cancer throughout the body?

PET scan

What are the three main types of biopsy procedures?

Needle/Aspiration (FNA)

Incisional

Excisional

What makes excisional biopsy unique?

Can have dual purpose

Diagnostic - confirms cancer diagnosis

Therapeutic - treats by removing tumor

What are the three main goals of cancer treatment?

Cure - Eliminate cancer completely

Control - Manage cancer as chronic disease

Palliation - Relieve symptoms, maintain quality of life

Can patients receive palliative care while still getting curative treatment?

yes; Palliative care and curative treatment can happen concurrently

What are the four main types of cancer treatment?

Surgical procedures - Remove tumors/affected tissue

Radiation therapy - Kill cancer cells with ionizing radiation

Medical treatments - Chemo, immunotherapy, biologic therapy, targeted therapy

Hematopoietic Stem Cell Transplant (HSCT) - BMT or stem cell transplant from peripheral blood

What are the main types of medical cancer treatments?

Chemotherapy - Drugs that kill rapidly dividing cells

Immunotherapy - Harnesses immune system to fight cancer

Biologic therapy - Uses living organisms or substances

Targeted therapy - Attacks specific cancer cell markers

Allows high-dose chemo/radiation by restoring bone marrow function

Hematopoietic Stem Cell Transplantation (HSCT)

Wasting syndrome with unintended weight loss, muscle atrophy, anorexia (common in UGI, esophageal, pancreatic cancers)

Cancer cachexia

Management for cancer cachexia

Best option: Treat the underlying cancer

Increase caloric intake (doesn't fully reverse wasting)

Megestrol acetate (appetite stimulant)

Why is infection the leading cause of death in cancer patients?

Immunosuppression from chemotherapy

Neutropenia (low WBC count)

Compromised skin/mucous membranes

Invasive procedures (central lines, catheters)

How should cancer pain be managed?

Be liberal with opioid use

What are the most common side effects of cancer treatment?

Nausea/Vomiting

Hair loss

Bone marrow suppression - Leads to neutropenia, thrombocytopenia, anemia

Fatigue

GI effects - Diarrhea, constipation, mucositis

Skin reactions - Rash, dryness, sensitivity (especially with radiation)

What are the key nursing concerns with bone marrow suppression?

Neutropenia (low WBCs)

Thrombocytopenia (low platelets)

Anemia (low RBCs)

How should nurses help patients manage cancer-related fatigue?

Fatigue is common and expected

Balance rest with activity

Plan activities during high-energy times

Use energy conservation strategies

What are the four types of leukemia?

Acute Myelogenous Leukemia (AML)

Chronic Myelogenous Leukemia (CML)

Acute Lymphocytic Leukemia (ALL)

Chronic Lymphocytic Leukemia (CLL)

Identify the leukemia:

Abrupt, dramatic onset

Very immature myeloblasts

1/3 of all leukemias

Most common acute leukemia in adults

Acute Myelogenous Leukemia (AML)

Identify the leukemia:

More mature cells than AML

Overproduction of myeloid cells

Philadelphia chromosomes present!

Can progress to blast crisis

Chronic Myelogenous Leukemia (CML)

Identify the leukemia:

Most common in children

Less common in adults

Acute Lymphocytic Leukemia (ALL)

Identify the leukemia:

Most common leukemia in adults

Accumulation of immature lymphocytes

"Smudge cells" on blood smear

Chronic Lymphocytic Leukemia (CLL)

What is unique about Chronic Myelogenous Leukemia (CML)?

Philadelphia chromosomes!

What is unique about Chronic Lymphocytic Leukemia (CLL)?

smudge cells

What's the difference between acute and chronic leukemia?

Acute (AML, ALL):

Abrupt, dramatic onset

Very immature blast cells

Rapid progression

Requires immediate treatment

Bone marrow often 80-100% blasts

Chronic (CML, CLL):

Gradual onset

More mature cells

Slower progression

May be asymptomatic initially

Can be monitored before treatment

What are the main clinical manifestations of leukemia?

Anemia (fatigue), thrombocytopenia (bleeding), decreased WBC function (infection), splenomegaly/hepatomegaly, lymphadenopathy, and bone pain.

WBC >100,000 causes thick blood with poor perfusion, leading to stroke and respiratory distress.

leukostasis

What are signs of CNS involvement in leukemia?

Early morning headaches, nausea/vomiting, and lethargy from meningeal infiltration.

How does bone pain present in leukemia patients?

Migratory, vague pain without swelling; can mimic rheumatoid arthritis.

What will be seen on a peripheral blood evaluation in leukemia?

premature leukocytes due to cells not functioning the way they should

What 2 diagnostic evaluations should be done for leukemia?

Peripheral blood evaluation

Bone marrow evaluation

What may cause a feeling of fullness in chronic myeloid leukemia?

hepatosplenomegaly

What are common ways that chronic myelogenous leukemia might present?

fatigue, night sweats, weight loss, early satiety, high WBCs

What can cause a progression from CML to acute leukemia?

blast crisis