Thank You

Abnormal
hemoglobin

By Dr. Ehab
Khairy
 Abnormal Hemoglobin
May be due to:
1- Abnormal concentrations
High or low concentrations
2- Abnormal Structures
Sickle cell disorders
3- Abnormal Synthesis
Thalassemias
4- Abnormal Hemoglobin function
Met hemoglobin
 Abnormally low Hemoglobin
Concentration
• Iron deficiency
• Chronic disease
∀ β Thalassemia
∀ α Thalassemia
• Sickle cell
• A plastic anemia
• Autoimmune
• Folic acid deficiency
• Vit.B12 deficiency
• Distribution Of Anemia
 Physiological compensatory
Mechanisms for Low Hemoglobin
 Manifestations Of Sever
Nutritional Anemia
Iron deficiency anemia

• Respiratory distress
• Congestive heart failure
• Thrombocytopenia
• Neurologic abnormalities
 Vitamin B 12 deficiency
anemia

1- Neuropathy
* Sensory : Bilateral symmetrical parasthesia
Loss of propioceptive and vibratory sensations
* Motor: Diminished deep tendon reflexes
and un steady gait
* Mental: Impaired memory and mental
depression
2- Thyroid disorders: associated with
pernicious anemia
 Folic acid deficiency anemia
• Smooth tongue
• Hyper pigmentation
• Mental depression
• Peripheral edema
• Liver dysfunction
• With or without peripheral neuropathy
 Optimization Of Deficiency
Anemia Before Elective Surgery
Iron Deficiency Anemia

# Oral preparations 200 mg

# Parenteral preparations must be given

under close medical supervision

# Human Erythropoietin
 Vitamin B12 Deficiency Anemia

1 mg hydroxo-cobolamin Im every three

days for six injections

Folic Acid Deficiency Anemia

Oral 5 mg of folic acid for four months

 Preoperative Transfusion
Decision making
*Causes of anemia *Degree of anemia

*Duration of anemia *Urgency of surgery

*Intravascular fluid volume
*Likely hood of intra operative blood loss
*Age of the patient
*Co existing diseases
- Ischemic heart disease
- Lung disease
- Cerebrovascular disease
 Guide Line For Blood
Transfusion
- Hb conc.> 10 g/dl Transfusion rarely indicated
specially for chronic anemia
- Hb conc. < 6g/dl Almost indicate blood
transfusion specially for acute anemia
- Hb conc. Between 6 – 9 g/dl Decision of
transfusion is determined by patient risk for
complications of decreased tissue oxygenation
( patient with ischemic heart)
Adverse Effects Of Anesthesia On
Anemia
Anesthesia depress sympathetic nervous
system and decrease C.O.P. and offsets
An important physiological compensatory
mechanism for anemia

Nitrous oxide can produce megaloblastic

changes even within a short period of
administration
 Effects of Anemia On Anesthesia
and Its Choice
• Anemia theoretically reduces Mac of
Volatile Anesthetics

• Neurologic changes of vitamin B12

deficiency detract the choice of Regional
anesthesia and peripheral nerve block
 Anesthetic Goals
1- Adequate tissue Oxygen Delivery
Avoid drug induce myocardial depression
Avoid factors shifting oxygen dissociation curve
to the left (Hyperventilation & Hypothermia)

2- Minimizing blood Loss

3- Adequate Monitoring
 Polycythemia

Pathologic excess of hematocrit with or without

increased hemoglobin concentration

1- Primary Polycythemia (vera)

Increased hematocrit Iron deficiency anemia
2- Relative Polycythemia
Increased hematocrit Normal Hb conc.
3- Secondary Polycythemia
Increased hematocrit High Hb conc.
 Causes Of Secondary Polycythemia
1- Chronic anoxia
* High Altitude * Smoker’s polycythemia
* Cardiopulmonary diseases
* Obesity – hypoventilation & Sleep apnea
syndrome
2- Renal Diseases
Calculi, Cysts, Hydronephrosis, Carcinoma
3- Excessive Testosterone production
4- Iatrogenic
Exogenous Testosterone and Erythropoietin
Complications of Increased
Hb Conc.
Increased Blood Viscosity

Increased peripheral resistance

Thrombosis
 Anesthetic Goals
Reduction of Hb conc. < 15g/dl prior to
elective surgery by
1- Minimizing tissue anoxia and other
factors
enhancing erythropoietin production

2- Normovolemic hemo- dilution just prior to

surgery is much better than repeated
phlebotomy a week before…. Why?
 Structure Of Hemoglobin
# Fetal Hemoglobin (Hb F)
Consists of 2 α chains & 2 γ
Changes to adult Hemoglobin within 3 to 6 months
after birth to adult
# Adult Hemoglobin (Hb A)
Consists of 2 α chains & 2 β
An other form of adult Hb
# Adult hemoglobin (HB A2)
consists of 2 α chains & 2 δ
 Hereditary Disorder Of
Hemoglobin
• Hemoglobinopathies consist of abnormal
structural variants of hemoglobin , the
most important is sickle disorders (Hb S)

• Thalassemia reflects abnormal synthesis

of hemoglobin where chains of
hemoglobin are diminished or absent
 Sickle Cell Diseases
Sickle cell disease is one of the most
prevalent genetic disorders affecting
millions through out the word
It is common in India, Saudia Arabia,
Mediterranean countries, South, and
center of America
Sickle cell disease denotes all genotypes
that contain at least 1 sickle gene in which
hemoglobin S (HbS) makes up at least half
of the hemoglobin present.
 Mendelian Trait of Sickle Cell
Disease
Hb S is formed of 2 normal alpha-
and 2 abnormal beta-globin chains
• Recessive (Hb S, Hb S)
Abnormal hemoglobin
• Heterozygous (Hb A, Hb S)
Normal and abnormal hemoglobin
Normal hemoglobin more abundant
 Pathophysiology
# β chain of Hb S differs form that of Hb A only in
the substitution of valine for glutamic acid at
the sixth position.
# Hb S carries oxygen normally but it unloads
oxygen more easily as its P50 is 31mmHg
compared to 26 mmHg of normal Hb
# Once oxygen is unloaded to the tissues
It begins to form semisolid aggregate
structures, consequently RBCs acquire sickle
shape.
 Results of Sickle Cells
• Erythrostasis
• Increased blood viscosity
• Reduced blood flow
• Hypoxia
• Increased adhesion of red blood cells
• Vascular occlusion
• Further sickling
Pathophysiology of Sickle Cell
Disease
 Clinical Signs and Symptoms
Vaso occlusive Painful crisis

• Acute chest syndrome (a life-

threatening pneumonia-like illness)
• Cerebrovascular accidents
• Splenic and renal dysfunction
• Chronic anemia
• Susceptibility to infections
 Cont.
• Jaundice
• Pallor
• Hand and foot syndrome (warmth
and tenderness)
• Leg ulcers
• Organomegaly
• Cardiac failure
• Abdominal and bone pain (aseptic
necrosis)
 A plastic crisis
Characterized by
profound anemia Hb is < 3g/dl,

It may be Precipitated by
Infection, hypoxia, systemic disease,
acidosis, dehydration, trauma, and
Folic acid deficiency
Splenic Sequestration Crisis
Characterized by
Sudden pooling of blood in the spleen
due to partial occlusion of its drainage
It can cause life threatening hypotension
in infants and young children…… Why?

Repeated splenic infarction result in

fibrosis -auto spleenectomy.
 Managing a Crisis
A part of blood transfusion
- High doses of folic acid
- Analgesia for pain
- Hydration
- Enhance hemoglobin F production of
by using hydroxyurea and
erythropoietin …….. Why?
 Anesthetic Managements
Preoperative preparation
- Adequate hydration
- Infection control
- Prophylactic antibiotics
- Optimization of quality and quantity of Hb

by partial exchange transfusion

- The goal of such transfusion is to achieve
a hematocrit of 30-40% with > 50%
normal Hb A
Intra operative Managements

Avoid 6H & 1S
Hypoxia Hypercarbia
Hypotension Hypovolemia
Hypothermia Hyperthermia
Stases
 Intra Operative Monitorings
CVP is useful to monitor volume state

PAP for monitoring of mixed venous

oxygen saturation

Sickling changes do not occur if the VSO2

above 40 mmHg
 Post operative Considerations

Supplemental oxygen
Pain Control
Pulmonary physiotherapy
Early ambulation
Thalassemia
 Types Of Thalassemia
Alpha Thalassemia

• Normal αα/αα
• Silent carrier - α/αα
• Minor -α/-α
--/αα
• Hb H disease --/-α
• Barts hydrops fetalis --/--
 Beta Thalassemia
• Normal β/β
• Minor β/β0
β/β+
• Intermedia β0/β+
• Major β0/β0
β+/β+

0 = no production of the chain

+ = diminished production of the chain
 Clinical manifestations
1- Anemia :
Microcytic and hypochromic

2- Excessive hemolysis

3- Hyperplasia of the bone marrow

and extramedullary erythropoiesis
 Excessive hemolysis:
- Many RBCs fail to mature and early destroyed
By the reticuloendothelial system
- Excess α globins of β Thalassemia (Target cell)
- This Hb is poorly soluble, highly reactive and
cause free radical cellular Injury

- Excess hemolysis result in

Splenomegaly, Hepatomegaly, Cholelithiasis,
and Jaundice
 3- Hyperplasia Of The Bone
Marrow
Caused by vigorous secretion of erythropoietin
Result in
* Skeletal abnormalities and facial
dysmorphism.
*Development of extramedullary marrow in
Pleura Hemothorax
Sinuses Epistaxis
Epidural space Epidural hematoma
 Blood Transfusion Therapy for
Thalassemia
1- Palliative transfusion:
It is just to keep patient surviving
2- hypertransfusion:
Maintain a hemoglobin level of 9 to 10 g/dl
To reduce extramedullary erythropoiesis
3- Supertransfusion:
Maintain a hemoglobin level >12 g/dl
in an effort to suppress all erythropoiesis.
 Complications of regular
Blood Transfusion
Hemosiderosis

Cardiac and Hepatic iron toxicity

(cirrhosis)
Require
Chelation therapy with Deferoxamine
 Expected Anesthetic
Difficulties
1- Difficult Air Way:
Hyperplasia of facial bones and narrowing of
nasal
passage
2- Hemosiderosis:
Resulting in cardiac dysfunction, supra
ventricular arrhythmias as well as hepatic
dysfunctions
 Regional Anesthesia

Epidural, spinal, and intrapleural anesthesia

are relatively contraindicated as the presence
of extramedullary bone marrow increases the
likelihood of bleeding and hematoma formation

Patients receiving palliative transfusion

therapy are at greatest risk
 Met-hemoglobin
Met = beyond
Ferric iron (fe+3) = one oxidation state
beyond ferrous iron (Fe+2)
Ferric form of iron
Unable to bind to O2,
Decreasing O2 carrying capacity

Shifts O2 dissociation curve to the

left Worsening tissue O2 release
 Etiology
Hereditary
Congenital absence of
NADH methemoglobin reductase
Acquired
Drug induced
Chemical induced
Drugs Causing Methemoglobin
All begin with letter A
1- Anti hypertensive Vaso dilator drugs
2- Anesthetics
Benzocaine, Lidocaine, Prilocaine
3- Analgesics
Phenacetin, Phenazopyridine
4- Antiemetic Metoclopramide
5- Anti leprosy Dapsone
6- Anti malarial Chloroquine
7- Antibiotics Sulfamethoxazole
 Sign & Symptoms Associated
with Methemoglobin blood Conc.
Met Hb conc. %total Hb Clinical

< 1.5g/dl < 10% None

1.5-3 g/dl 10-20% Cyanosis
3-4.5g/dl 20-30% Anxiety
Tachycardia
7.5-10.5 50-70% Acidosis
g/dl Arrhythmia
> 10.5g/dl >70% Death
 Diagnosis
1- Tachyphylaxis
2- Cyanosis despite high FIo2
3- Low fixed reading of pulse oximetry
4- Normal PO2 of ABG, but higher
calculated hemoglobin saturation than
that measured by pulse oximetry
5- Low PH of ABG
6- Higher than normal O2 venous tension
7- Development of cardiac arrhythmias
 Confirmation Of Diagnosis
Co- oximetry
Masseurs light absorbance at four
Different wave lengths so it is able to
quantify % of

Met Hb Co Hb

Oxy Hb Deoxy Hb
 Management Of Acquired
Methemoglobin
Mild Methemoglobinemia

Withdrawal of the offending agent

Oxygen administration

No specific therapy
 Methylene Blue
Mechanism :Transfers electron to methemoglobin
reducing it to normal
Dosage :1-2mg/kg iv over 3-5 minutes
Can be repeated at a dose of 1mg/kg after 30
minutes
Symptoms expected to diminish within 30-60
minutes
Cautions: Not effective in G6PD deficiency and
may cause hemolysis in these cases