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Hematological Disorder

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Adham Mohammed
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0% found this document useful (0 votes)
39 views36 pages

Hematological Disorder

Uploaded by

Adham Mohammed
Copyright
© © All Rights Reserved
We take content rights seriously. If you suspect this is your content, claim it here.
Available Formats
Download as PDF, TXT or read online on Scribd
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ANEMIA

▪ Anemia is a reduction in the number of RBCs, the quantity


of hemoglobin, or the volume of RBCs
▪ Because the main function of RBCs is oxygenation, anemia
results in varying degrees of hypoxia
▪ Detected or confirmed through CBC.
▪ Causes:
➢ Blood loss
➢ Decreased production of erythrocytes
➢ Increased destruction of erythrocytes
SYMPTOMS
▪ Clinical Manifestations:
1. Pallor.
2. Fatigue, weakness.
3. Dyspnea.
4. Palpitations, tachycardia.
5. Headache, dizziness, and restlessness.
6. Slowing of thought.
7. Paresthesia.
TREATMENT
▪ A successful treatment of anemia depends on
successful diagnosis of the cause.
▪ Vitamin and mineral supplements
▪ Change in diet
▪ Medication
▪ Blood transfusion
▪ Bone marrow transplant
▪ Surgery (to remove the spleen, if related to
hemolytic anemia)
▪ Antibiotics (if an infection is the causative
agent)
TYPES OF ANEMIA
• Iron Deficiency Anemia
• Thalassemia
• Megablastic Anemia
• Anemia of Chronic Disease
• Aplastic Anemia
• Anemia Caused By Blood Loss
Iron-Deficiency Anemia
▪ Etiology:
1. Inadequate dietary intake
• Found in 30% of the world’s population
2. Malabsorption
• Absorbed in duodenum
• GI surgery
3. Blood loss
• 2 mls blood contain 1mg iron
• GIT losses
4. Hemolysis
▪ Clinical Manifestations:
• Most common: pallor
• Second most common: inflammation of the
tongue (glossistis)
• Cheilitis=inflammation/fissures of lips
• Sensitivity to cold
• Weakness and fatigue
▪ Diagnostic Studies:
• CBC
• Iron levels: Total iron-binding capacity (TIBC),
Serum Ferritin.
• Endoscopy/Colonscopy
• Management:
– Treatment of underlying disease/problem
– Diet- foods rich in iron
– Drug Therapy: Iron therapy for 2-3 months after the
hemoglobin levels return to normal

– Oral iron
» Feosol, DexFerrum, etc
» Absorbed best in acidic environemtn
– Parenteral iron
» IM or IV
» Less desirable than PO
Thalassemia
▪ Etiology:
• Autosomal recessive genetic disorder of inadequate production
of normal hemoglobin
• Found in Mediterranean ethnic groups

▪ Clinical Manifestations:
• Asymptomatic → major retardation → life threatening
• pallor, icterus, weakness.
• Splenomegaly, hepatomegaly cardiac enlargement
▪ Mageanment:
• No specific drug or diet are effective in treating thalassemia
• Thalassemia minor : Body adapts to ↓ Hgb
• Thalassemia major: Blood transfusions with IV deferoxamine
Megaloblastic Anemias
• Characterized by large RBCs which are fragile
and easily destroyed
• Common forms of megaloblastic anemia
1. Cobalamin deficiency
2. Folic acid deficiency
1) Cobalamin (Vitamin B12) Deficiency
• Vitamin B12 (cobalamin) is an important water-soluble
vitamin.
• Intrinsic factor (IF) is required for cobalamin absorption
• Causes of cobalamin deficiency:
– Gastric mucosa not secreting IF
– GI surgery →loss of IF-secreting gastric mucosal cells
– Long-term use of H2-histamine receptor blockers
cause atrophy or loss of gastric mucosa.
– Nutritional deficiency
– Hereditary defects of cobalamine utilization
• Clinical manifestations:
– General symptoms of anemia
– Sore tongue
– Anorexia
– Weakness
– Parathesias of the feet and hands
– Confusion → dementia
• Diagnostic Studies:
• RBCs appear large & Abnormal shapes
• Management:
– Parenteral administration of cobalamin
– High dose oral cobalamin can use be used
2) Folic Acid Deficiency
• Folic Acid Deficiency also causes megablastic anemia
(RBCs that are large and fewer in number)
• Folic Acid required for RBC formation and maturation
• Causes:
– Poor dietary intake
– Malabsorption syndromes
– Drugs that inhibit absorption
– Alcohol abuse
– Hemodialysis
▪ Clinical manifestations : are similar to those of cobalamin
deficiency
• Insidious onset: progress slowly
• Absence of neurologic problems
▪ Treatment:
• Folate replacement therapy
• Encourage patient to eat foods with large amounts of folic
acid
• Leafy green vegetables
• Liver
• Mushrooms
• Oatmeal (‫)الشوفان المجروش‬
Anemia of Chronic Disease
• Underproduction of RBCs, shortening of RBC survival
• 2nd most common cause of anemia (after iron deficiency
anemia
• Causes
– Impaired renal function
– Splenomegaly
– Hepatitis
– Chronic, inflammatory, infectious or malignant disease
– Chronic liver disease
– Folic acid deficiencies
Aplastic Anemia
• Etiology
– Congenital: Chromosomal alterations
– Acquired: Results from exposure to ionizing radiation,
chemical agents, viral and bacterial infections
• Characterized: by Pancytopenia
– ↓ of all blood cell types
• RBCs
• White blood cells (WBCs)
• Platelets
– Can be a critical condition: hemorrhage & Sepsis
• Clinical Manifestations: Gradual development
- General manifestations of anemia
• Frequent or prolonged infections
• Unexplained or easy bruising
• Nose bleed and bleeding gums
• Prolonged bleeding from cuts
▪ Treatment:
• Identifying cause
• Blood transfusions
• Antibiotics
• Bone marrow stimulants: (Neupogen) & Bone marrow
transplantation
Anemia Caused By Blood Loss
▪ Acute Blood Loss
▪ Chronic Blood Loss

1) Acute Blood Loss: Result of sudden hemorrhage


– Trauma
– Surgery.

2) Chronic Blood Loss:


– Similar to iron deficiency anemia
– GI bleeding, hemorrhoids, menstrual blood loss
Anemia caused by Increased Erythrocyte Destruction
1-Hemolytic Anemia :Third major cause of anemia
• Destruction or hemolysis of RBCs at a rate that exceeds
production
• Intrinsic hemolytic anemia
– Abnormal hemoglobin
– Enzyme deficiencies
– RBC membrane abnormalities
• Extrinsic hemolytic anemia
– Normal RBCs
– Damaged by external factors
• Liver
• Spleen
• Toxins
• Mechanical injury (heart valves)
Anemia
Case Study #1

A 72 year old
male has the
CBC findings
shown.
Peripheral
RBCs are
hypochromic
& microcytic.
Anemia
Case Study #1

• What test would you order for this


patient?
• A-Hemoglobin Electrophoresis
• B-Retic count
• C-Stool for occult blood
• D-B12 Assay
• E-Bone marrow biopsy
Anemia
Case Study #1
• Two questions:

– What is your diagnosis?

– What is the next step for this patient?


Anemia
Case Study #1
• Answers
– Question 1
• Likely Iron Deficiency Anemia
– Question 2
• Colonoscopy
Anemia
Case Study #2
A 48 year old male
has become
progressively more
fatigued at the end of
the day. This has been
going on for months.
In the past month he
has noted paresthesias
with numbness in his
feet. A CBC
demonstrates the
findings shown.
Anemia
Case Study #2

A peripheral blood
smear (the slide is
representative of this
condition) shows red
blood cells displaying
macro- ovalocytosis
and neutrophils with
hypersegmentation.
Anemia
Case Study #2
Which of the following tests would be most
useful to determine the etiology?
A. Hemoglobin electrophoresis
B. Reticulocyte count
C. Stool for occult blood
D. Vitamin B12 assay
E. Bone marrow biopsy
Anemia
Case Study #2
• Questions:

• What is the diagnosis from these findings?

• How do you explain the neurologic findings?


Anemia
Case Study #2
• Answers:
– Question 1
• This is a macrocytic (megaloblastic) anemia. The
neurologic findings suggest vitamin B12 deficiency
(pernicious anemia).
– Question 2
• The B12 deficiency leads to degeneration in the
spinal cord (posterior and lateral columns).
Case 1
• 3 yr old male child presenting with
pallor,jaundice,
• Severe pain of long bones, fever
• CBC-anemia,reticulocytosis,increased
WBC
• LAB - LDH -600 (normal upto 200)
S.bilirubin- 5mg%
Peripheral smear
Diagnosis?
SICKLE cell anemia
DIAGNOSIS – OTHER TESTS

• Hemoglobin electrophoresis
-HbS >80%
-HbF -1-20%
-HbA2 -2- 4.5%

• Sickling test POSITIVE


CASE 4
• 14 YR old female present with anemia,
jaundice
• Rt hypochondrial pain
• o/e- vitals
stable.pallor+,icterus+,splenomegaly +
• Usg- cholilithiasis
• Lab; elevated ,LDH, S.Bilirubin
• Peripheral smear shows-
Differential diagnosis
• Hereditary spherocytosis

• Autoimmune hemolytic anemia

• Other diagnostic tests- osmotic fragility

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