HEMATOLOGY
DISORDERS
Submitted to: Mam Tehseen
Submitted by: Stargazers
Slides Designed by: Abeera Munawar
1
OBJECTIVES
Sickle cell Immune Iron Vitamin DIC
anemia hemolytic deficiency B12 (disseminated
intravascular
anemia anemia deficiency coagulation)
anemia
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Sickle Cell Anemia
Sickle cell anemia is a genetic disorder that affects hemoglobin production in red blood
cells.it is characterized by Abnormal hemoglobin (HbS) causes red blood cells to become
misshapen (sickle-shaped) and rigid. These sickled cells can block blood vessels, leading
to pain crises, organ damage, and other complications.
Types
1. HbSS (Sickle Cell Disease): This is the most common and
severe form, where an individual inherits two copies of the HbS
gene (one from each parent). It leads to more frequent and
c
severe symptoms.
2. HbSC: This type occurs when an individual inherits one HbS
gene and one HbC gene. While still problematic, HbSC tends to
be milder than HbSS, with fewer and less severe crises
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Causes
Genetic
Inheritance
Mutation
Sign and Symptoms
• Chronic hemolysis and thrombosis
• Short erythrocyte lifespan (10–12 days)
• Anemia (Hb 7–10 g/dL)
• Jaundice (especially in sclerae)
• Bone marrow expansion
• Tachycardia • Cardiomegaly
• Cardiac murmurs • Dysrhythmias
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PATHOPHYSIOLOGY
1. Abnormal Hemoglobin (HbS):
Genetic mutation leads to the formation of HbS instead of
normal HbA.
2. Sickle-Shaped Cells:
Under low oxygen conditions, HbS causes red blood cells to
become stiff and sickle-shaped.
3. Blockage of Blood Flow:
Sickled cells are less flexible and can clump together,
obstructing blood flow in small blood vessels.
4. Tissue Damage:
Blocked blood flow reduces oxygen supply to tissues, leading
to ischemia, pain, and eventual tissue damage.
5. Shortened RBC Lifespan:
Sickled red blood cells have a shorter lifespan (10-20 days vs.
normal 120 days), leading to hemolysis and anemia.
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Nursing management
1. Assessment and Monitoring
2. Pain Management
3. Hydration
4. Oxygen Therapy
5. Education
6. Prevention of Complications
7. Supportive Care
8. Medication Management
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Immune hemolytic anemia (IHA)
Immune hemolytic anemia (IHA) is a condition where the
body's immune system mistakenly targets and destroys its
own red blood cells (RBCs), leading to hemolysis
(destruction of RBCs) and anemia (low red blood cell
count).
Cause:
1. Monitor Blood Counts
2. Assess for Anemia Symptoms
3. Manage Blood Transfusions
4. Medication Administration
5. Infection Prevention
6. Patient Education
7. Supportive Care
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Signs and symptoms
1. Fatigue
2. Paleness
3. Jaundice
4. Dark Urine
5. Splenomegaly
6. Chills and Fever
7. Shortness of Breath
8. Tachycardia
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Nursing management
1. Monitor Blood Counts
2. Assess for Anemia Symptoms
3. Manage Blood Transfusions
4. Medication Administration
5. Infection Prevention
6. Patient Education
7. Supportive Care
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Iron deficiency anemia
Iron deficiency anemia is a condition where there is insufficient iron in the body to
produce hemoglobin, leading to a decrease in the number of red blood cells and a
reduction in the blood's ability to carry oxygen to tissues.
CAUSES:
• Inadequate dietary intake (e.g., vegetarian diets).
• Blood loss (e.g., intestinal hookworm, ulcers, gastritis, GI tumors)
• Menorrhagia and pregnancy (in premenopausal women)
• Chronic alcoholism (GI blood loss)
• Iron malabsorption (gastrectomy, celiac disease)
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Signs and symptoms
1. Fatigue
2. Pallor
3. Shortness of Breath
4. Dizziness or Light headedness
5. Brittle Nails
6. Cold Hands and Feet
7. Headaches
8. Glossitis
9. Restless Leg Syndrome
10. A smooth, sore tongue; brittle and
ridged
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PATHOPHYSIOLOGY
Inequatead dietary intake or chronic blood loss leads to reduced iron stores in
the body.
↓
Depleted iron stores result in insufficient iron available for hemoglobin synthesis.
↓
Insufficient hemoglobin production leads to microcytic (small-sized)
erythrocytes.
↓
Microcytic erythrocytes result in decreased mean corpuscular volume (MCV).
↓
Decreased hemoglobin levels cause reduced oxygen-carrying capacity of
blood.
↓
Onset of symptoms
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Nursing management
1. Assess and Monitor
2. Dietary Education
3. Iron Supplementation
4. Manage Side Effects
5. Identify and Address Underlying Causes
6. Follow-up Care
7. Patient Empowerment
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Vitamin B12 deficiency anemia
A condition where the body lacks sufficient vitamin B12, leading to
impaired red blood cell production and anemia. This deficiency can
cause fatigue, weakness, shortness of breath, and neurological symptoms
like numbness and tingling
causes:
1. Inadequate Dietary Intake
2. Malabsorption
3. Intrinsic Factor Deficiency
4. Chronic Alcoholism
5. Medications
6. Age
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Signs and symptoms
1. Fatigue
2. Pallor
3. Glossitis
4. Neurological Symptoms
5. Sore Mouth or Ulcers
6. Shortness of Breath
7. Weakness8. Dizziness
9. Cognitive Impairment
10. Elevated Methylmalonic Acid (MMA) and
Homocysteine
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PATHOPHYSIOLOGY
Decreased thymidylate and purine synthesis
↓
Impaired DNA synthesis
↓
Formation of immature nucleus that appears large and dense
+
Asynchronous cytoplasmic development due to normal RNA
synthesis
↓
Megaloblast formation
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Nursing management
1.Assess and Monitor
2. Dietary Education
3. Vitamin B12 Supplementation
4. Manage Symptoms
5. Identify and Address Underlying Causes
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DIC (disseminated intravascular coagulation
)
Disseminated intravascular coagulation (DIC) is a serious condition
characterized by abnormal activation of the coagulation cascade,
leading to widespread clot formation and simultaneous bleeding due to
the consumption of clotting factors and platelets
causes:
• Sepsis (most common cause)
• Trauma or burns- Obstetric complications (e.g., abruptio placentae, amniotic fluid embolism)
• Cancer (especially leukemia and metastatic tumors)
• Severe infections (viral, bacterial, fungal)
• Shock or hypoxia
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Signs and symptoms
• bleeding
• Petechiae
• purpura, ecchymosis
• prolonged bleeding from wounds
• Thrombosis
• Cyanosis, gangrene, signs of stroke, or
organ failure
• Hypotension & shock
• Due to widespread clotting and
bleeding
• Respiratory distress
• Due to microvascular clotting in the
lungs.
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PATHOPHYSIOLOGY
• Triggering Event: (e.g. infection, trauma, cancer)
↓
• activates coagulation pathway
↓
• Widespread Clot Formation (Microthrombi form in small blood vessels)
↓
• Consumption of Clotting Factors & Platelets: Coagulation proteins and
platelets get used up.
↓
• Bleeding Tendency: Since clotting materials are exhausted, bleeding
occurs
• ↓
Organ Ischemia
↓
• Clots block blood supply, damaging organs like kidney, brain, lungs.
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Nursing management
❑ Monitor for bleeding & thrombosis
❑ Check mucosal sites, IV lines, and skin.Prevent injury
❑ Use soft toothbrushes, avoid IM injections, handle
patient gently
❑ Assess vital signs frequently
❑ Detect signs of shock or organ dysfunction.Monitor
lab values
❑ PT, aPTT, fibrinogen, platelets, and D-dimer
❑ Provide emotional support
❑ Address anxiety and educate the family.
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