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Abnormal Abnormal hemoglobinhemoglobin
By Dr. Ehab By Dr. Ehab KhairyKhairy
Abnormal HemoglobinAbnormal Hemoglobin
May be due to: May be due to:
1- Abnormal concentrations1- Abnormal concentrations
High or low concentrationsHigh or low concentrations
2- Abnormal Structures2- Abnormal Structures
Sickle cell disordersSickle cell disorders
3- Abnormal Synthesis 3- Abnormal Synthesis
ThalassemiasThalassemias
4- Abnormal Hemoglobin function4- Abnormal Hemoglobin function
Met hemoglobin Met hemoglobin
Abnormally low Hemoglobin Abnormally low Hemoglobin Concentration Concentration
• Iron deficiency Iron deficiency • Chronic diseaseChronic disease ThalassemiaThalassemia ThalassemiaThalassemia• Sickle cellSickle cell• A plastic anemiaA plastic anemia• AutoimmuneAutoimmune• Folic acid Folic acid deficiencydeficiency• Vit.B12 deficiencyVit.B12 deficiency• Distribution Of AnemiaDistribution Of Anemia
Physiological compensatory Physiological compensatory Mechanisms for Low HemoglobinMechanisms for Low Hemoglobin
Manifestations Of Sever Manifestations Of Sever Nutritional AnemiaNutritional Anemia
Iron deficiency anemiaIron deficiency anemia
• Respiratory distressRespiratory distress• Congestive heart failureCongestive heart failure• ThrombocytopeniaThrombocytopenia• Neurologic abnormalities Neurologic abnormalities
Vitamin B 12 deficiency Vitamin B 12 deficiency anemiaanemia
1- Neuropathy1- Neuropathy* Sensory : * Sensory : Bilateral symmetrical parasthesiaBilateral symmetrical parasthesiaLoss of propioceptive and vibratory sensationsLoss of propioceptive and vibratory sensations* Motor: * Motor: Diminished deep tendon reflexesDiminished deep tendon reflexesand un steady gaitand un steady gait* Mental: * Mental: Impaired memory and mentalImpaired memory and mentaldepressiondepression
2- Thyroid disorders: 2- Thyroid disorders: associated withassociated withpernicious anemiapernicious anemia
Folic acid deficiency anemiaFolic acid deficiency anemia
• Smooth tongueSmooth tongue• Hyper pigmentationHyper pigmentation• Mental depressionMental depression• Peripheral edemaPeripheral edema• Liver dysfunctionLiver dysfunction• With or without peripheral neuropathyWith or without peripheral neuropathy
Optimization Of Deficiency Optimization Of Deficiency Anemia Before Elective SurgeryAnemia Before Elective SurgeryIron Deficiency Anemia Iron Deficiency Anemia
# Oral preparations # Oral preparations 200 mg200 mg
# # ParenteralParenteral preparations preparations must be given must be given under close medical supervision under close medical supervision
# Human Erythropoietin# Human Erythropoietin
Vitamin B12 Deficiency AnemiaVitamin B12 Deficiency Anemia
1 mg hydroxo-cobolamin Im every three 1 mg hydroxo-cobolamin Im every three days for six injectionsdays for six injections
Folic Acid Deficiency AnemiaFolic Acid Deficiency Anemia
Oral 5 mg of folic acid for four monthsOral 5 mg of folic acid for four months
Preoperative Transfusion Preoperative Transfusion Decision makingDecision making
*Causes of anemia *Degree of anemia *Causes of anemia *Degree of anemia
*Duration of anemia *Urgency of surgery*Duration of anemia *Urgency of surgery
*Intravascular fluid volume*Intravascular fluid volume
*Likely hood of intra operative blood loss*Likely hood of intra operative blood loss
*Age of the patient*Age of the patient
**Co existing diseasesCo existing diseases- Ischemic heart disease - Ischemic heart disease
- Lung disease- Lung disease
- Cerebrovascular disease - Cerebrovascular disease
- - Peripheral vascular disease Peripheral vascular disease
Guide Line For Blood Guide Line For Blood TransfusionTransfusion
- Hb conc.> 10 g/dl - Hb conc.> 10 g/dl Transfusion rarely indicatedTransfusion rarely indicated
specially for chronic anemiaspecially for chronic anemia
- Hb conc. < 6g/dl - Hb conc. < 6g/dl Almost indicate bloodAlmost indicate blood
transfusion specially for acute anemiatransfusion specially for acute anemia
- Hb conc. Between 6 – 9 g/dl - Hb conc. Between 6 – 9 g/dl Decision ofDecision of
transfusion is determined by patient risk fortransfusion is determined by patient risk for
complications of decreased tissue oxygenation complications of decreased tissue oxygenation
( patient with ischemic heart)( patient with ischemic heart)
Adverse Effects Of Anesthesia On Adverse Effects Of Anesthesia On Anemia Anemia
Anesthesia depress sympathetic nervousAnesthesia depress sympathetic nervous
system and decrease C.O.P. and offsets system and decrease C.O.P. and offsets
An important physiological compensatoryAn important physiological compensatory
mechanism for anemiamechanism for anemia
Nitrous oxide can produce megaloblasticNitrous oxide can produce megaloblastic
changes even within a short period ofchanges even within a short period of
administrationadministration
Effects of Anemia On Anesthesia Effects of Anemia On Anesthesia and Its Choice and Its Choice
• Anemia theoretically reduces Mac of Anemia theoretically reduces Mac of Volatile AnestheticsVolatile Anesthetics
• Neurologic changes of vitamin B12 Neurologic changes of vitamin B12 deficiency detract the choice of Regional deficiency detract the choice of Regional anesthesia and peripheral nerve blockanesthesia and peripheral nerve block
Anesthetic Goals Anesthetic Goals
1-1- Adequate tissue Oxygen DeliveryAdequate tissue Oxygen DeliveryAvoid drug induce myocardial depressionAvoid drug induce myocardial depression
Avoid factors shifting oxygen dissociation curveAvoid factors shifting oxygen dissociation curve
to the left (Hyperventilation & Hypothermia) to the left (Hyperventilation & Hypothermia)
2-2- Minimizing blood LossMinimizing blood Loss
3-3- Adequate Monitoring Adequate Monitoring
Polycythemia Polycythemia
Pathologic excess of hematocrit with or withoutPathologic excess of hematocrit with or without
increased hemoglobin concentrationincreased hemoglobin concentration
1- Primary Polycythemia (vera)1- Primary Polycythemia (vera)
Increased hematocrit Iron deficiency anemiaIncreased hematocrit Iron deficiency anemia
2- Relative Polycythemia2- Relative Polycythemia
Increased hematocrit Normal Hb conc.Increased hematocrit Normal Hb conc.
3- Secondary Polycythemia3- Secondary Polycythemia
Increased hematocrit High Hb conc.Increased hematocrit High Hb conc.
Causes Of Secondary PolycythemiaCauses Of Secondary Polycythemia
1- Chronic anoxia1- Chronic anoxia* High Altitude * Smoker’s polycythemia* High Altitude * Smoker’s polycythemia
* Cardiopulmonary diseases * Cardiopulmonary diseases
* Obesity – hypoventilation & Sleep apnea* Obesity – hypoventilation & Sleep apnea
syndromesyndrome
2- Renal Diseases 2- Renal Diseases Calculi, Cysts, Hydronephrosis, CarcinomaCalculi, Cysts, Hydronephrosis, Carcinoma
3- Excessive Testosterone production3- Excessive Testosterone production
4- Iatrogenic 4- Iatrogenic Exogenous Testosterone and ErythropoietinExogenous Testosterone and Erythropoietin
Complications of Increased Complications of Increased Hb Conc. Hb Conc.
Increased Blood ViscosityIncreased Blood Viscosity
Increased peripheral resistanceIncreased peripheral resistance
ThrombosisThrombosis
Anesthetic Goals Anesthetic Goals
Reduction of Hb conc. < 15g/dl prior toReduction of Hb conc. < 15g/dl prior to
elective surgery byelective surgery by
1- Minimizing tissue anoxia and other factors1- Minimizing tissue anoxia and other factors
enhancing erythropoietin productionenhancing erythropoietin production
2- Normovolemic hemo- dilution just prior to2- Normovolemic hemo- dilution just prior to
surgery is much better than repeatedsurgery is much better than repeated
phlebotomy a week before…. Why?phlebotomy a week before…. Why?
Structure Of Hemoglobin Structure Of Hemoglobin
# Fetal Hemoglobin (Hb F)# Fetal Hemoglobin (Hb F)Consists of 2Consists of 2 chains & 2 chains & 2 Changes to adult Hemoglobin within 3 to 6 monthsChanges to adult Hemoglobin within 3 to 6 months
after birth to adult after birth to adult
# Adult Hemoglobin (Hb A)# Adult Hemoglobin (Hb A)Consists of 2Consists of 2 chains & 2 chains & 2 An other form of adult HbAn other form of adult Hb
# Adult hemoglobin (HB# Adult hemoglobin (HB A A22))
consists of 2consists of 2 chains & 2 chains & 2 δδ
•
Hereditary Disorder Of Hereditary Disorder Of Hemoglobin Hemoglobin
• Hemoglobinopathies consist of abnormal Hemoglobinopathies consist of abnormal structural variants of hemoglobin , the structural variants of hemoglobin , the most important is sickle disorders (Hb S) most important is sickle disorders (Hb S)
• Thalassemia reflects abnormal synthesis Thalassemia reflects abnormal synthesis of hemoglobin where chains of of hemoglobin where chains of hemoglobin are diminished or absenthemoglobin are diminished or absent
Sickle Cell Diseases Sickle Cell Diseases
Sickle cell disease is one of the most Sickle cell disease is one of the most prevalent genetic disorders affecting prevalent genetic disorders affecting millions through out the word millions through out the word
It is common in India, Saudia Arabia, It is common in India, Saudia Arabia, Mediterranean countries, South, and Mediterranean countries, South, and center of America center of America
Sickle cell disease denotes all Sickle cell disease denotes all genotypes that contain at least 1 sickle genotypes that contain at least 1 sickle gene in which hemoglobin S (HbS) makes gene in which hemoglobin S (HbS) makes up at least half of the hemoglobin present.up at least half of the hemoglobin present.
Mendelian Trait of Sickle Cell Mendelian Trait of Sickle Cell Disease Disease
Hb S is formed of 2 normal alpha-Hb S is formed of 2 normal alpha-and 2 abnormal beta-globin chainsand 2 abnormal beta-globin chains
• • Recessive (Hb S, Hb S)Recessive (Hb S, Hb S) Abnormal hemoglobin Abnormal hemoglobin
• • Heterozygous (Hb A, Hb S)Heterozygous (Hb A, Hb S) Normal and abnormal hemoglobinNormal and abnormal hemoglobin
Normal hemoglobin more abundantNormal hemoglobin more abundant
PathophysiologyPathophysiology# # chain of chain of Hb S differs form that of Hb A only inHb S differs form that of Hb A only in the substitution of valine for glutamic acid atthe substitution of valine for glutamic acid at the sixth position.the sixth position.# Hb S carries oxygen normally but it unloads# Hb S carries oxygen normally but it unloads
oxygen more easily as its Poxygen more easily as its P50 50 is 31mmHgis 31mmHg compared to 26 mmHg of normal Hbcompared to 26 mmHg of normal Hb# Once oxygen is unloaded to the tissues# Once oxygen is unloaded to the tissues It begins to form semisolid aggregateIt begins to form semisolid aggregate structures, structures, consequently RBCs acquire sickleconsequently RBCs acquire sickle shape.shape.
Results of Sickle CellsResults of Sickle Cells
• • ErythrostasisErythrostasis
• • Increased blood viscosityIncreased blood viscosity
• • Reduced blood flowReduced blood flow
• • HypoxiaHypoxia
• • Increased adhesion of red blood cellsIncreased adhesion of red blood cells
• • Vascular occlusionVascular occlusion
• • Further sicklingFurther sickling
Pathophysiology Pathophysiology of Sickle Cell of Sickle Cell DiseaseDisease
Clinical Signs and SymptomsClinical Signs and Symptoms
Vaso occlusive Painful crisisVaso occlusive Painful crisis
• • Acute chest syndrome (a life-Acute chest syndrome (a life-threatening pneumonia-like illness)threatening pneumonia-like illness)
• • Cerebrovascular accidentsCerebrovascular accidents
• • Splenic and renal dysfunctionSplenic and renal dysfunction
• • Chronic anemiaChronic anemia
• • Susceptibility to infectionsSusceptibility to infections
Cont.Cont.
• • JaundiceJaundice
• • PallorPallor
• • Hand and foot syndrome (warmth Hand and foot syndrome (warmth and tenderness)and tenderness)
• • Leg ulcersLeg ulcers
• • OrganomegalyOrganomegaly
• • Cardiac failureCardiac failure
• • Abdominal and bone pain (aseptic Abdominal and bone pain (aseptic necrosis)necrosis)
A plastic crisisA plastic crisis
Characterized by Characterized by profound anemia Hb is < 3g/dl, profound anemia Hb is < 3g/dl,
It may be Precipitated by It may be Precipitated by IInfection, hypoxia, systemic disease, nfection, hypoxia, systemic disease,
acidosis, dehydration, trauma, and acidosis, dehydration, trauma, and Folic acid deficiency Folic acid deficiency
Splenic Sequestration CrisisSplenic Sequestration Crisis
Characterized byCharacterized by Sudden pooling of blood in the spleen Sudden pooling of blood in the spleen
due to partial occlusion of its drainagedue to partial occlusion of its drainage
It can cause life threatening hypotension in It can cause life threatening hypotension in infants and young children…… Why?infants and young children…… Why?
Repeated splenic infarction result in Repeated splenic infarction result in fibrosis -auto spleenectomy.fibrosis -auto spleenectomy.
Managing a CrisisManaging a Crisis
A part of blood transfusionA part of blood transfusion
- High doses of folic acid- High doses of folic acid
- Analgesia for pain- Analgesia for pain
- Hydration- Hydration
- Enhance hemoglobin F production of - Enhance hemoglobin F production of by using hydroxyurea and by using hydroxyurea and erythropoietin …….. erythropoietin …….. Why?Why?
Anesthetic ManagementsAnesthetic Managements
Preoperative preparationPreoperative preparation- Adequate hydration- Adequate hydration
- Infection control- Infection control
- Prophylactic antibiotics- Prophylactic antibiotics- Optimization of quality and quantity of HbOptimization of quality and quantity of Hb
by partial exchange transfusionby partial exchange transfusion- The goal of such transfusion is to achieve The goal of such transfusion is to achieve
a hematocrit of 30-40% with > 50% a hematocrit of 30-40% with > 50% normal Hb Anormal Hb A
Intra operative ManagementsIntra operative Managements
Avoid 6H & 1SAvoid 6H & 1SHypoxia HypercarbiaHypoxia Hypercarbia
Hypotension Hypovolemia Hypotension Hypovolemia
Hypothermia HyperthermiaHypothermia Hyperthermia
StasesStases
Intra Operative MonitoringsIntra Operative Monitorings
CVPCVP is useful to monitor volume state is useful to monitor volume state
PAP PAP for monitoring of mixed venous for monitoring of mixed venous oxygen saturation oxygen saturation
Sickling changes do not occur if the Sickling changes do not occur if the VSOVSO22
above 40 mmHgabove 40 mmHg
Post operative ConsiderationsPost operative Considerations
Supplemental oxygenSupplemental oxygen
Pain ControlPain Control
Pulmonary physiotherapy Pulmonary physiotherapy
Early ambulationEarly ambulation
ThalassemiaThalassemia
Types Of ThalassemiaTypes Of Thalassemia
Alpha ThalassemiaAlpha Thalassemia
• NormalNormal // • Silent carrierSilent carrier - - // • MinorMinor --/-/-
--/--/• Hb H diseaseHb H disease --/---/-• Barts hydrops fetalisBarts hydrops fetalis --/-- --/--
Beta ThalassemiaBeta Thalassemia
• NormalNormal //• MinorMinor //00
//++
• IntermediaIntermedia 00//++
• Major Major 00//00
++//+ +
0 = no production of the chain0 = no production of the chain
+ = diminished production of the chain+ = diminished production of the chain
Clinical manifestationsClinical manifestations
1- Anemia : 1- Anemia :
Microcytic and hypochromicMicrocytic and hypochromic
2- Excessive 2- Excessive hemolysishemolysis
3- Hyperplasia of the bone marrow3- Hyperplasia of the bone marrow
and extramedullary erythropoiesisand extramedullary erythropoiesis
Excessive hemolysis:Excessive hemolysis:- Many RBCs fail to mature and early destroyed- Many RBCs fail to mature and early destroyed
By the reticuloendothelial systemBy the reticuloendothelial system
- Excess - Excess globins of globins of Thalassemia (Target cell) Thalassemia (Target cell)
- This Hb is poorly soluble, highly reactive and- This Hb is poorly soluble, highly reactive and
cause free radical cellular Injurycause free radical cellular Injury
- Excess hemolysis result in Excess hemolysis result in
Splenomegaly, Hepatomegaly, Cholelithiasis, Splenomegaly, Hepatomegaly, Cholelithiasis, and Jaundiceand Jaundice
3- Hyperplasia Of The Bone 3- Hyperplasia Of The Bone MarrowMarrow
Caused by Caused by vigorous secretion of erythropoietinvigorous secretion of erythropoietin
Result in Result in
* Skeletal abnormalities and facial* Skeletal abnormalities and facial
dysmorphism.dysmorphism.
*Development of extramedullary marrow in *Development of extramedullary marrow in
Pleura HemothoraxPleura Hemothorax
Sinuses EpistaxisSinuses Epistaxis
Epidural space Epidural hematomaEpidural space Epidural hematoma
Blood Transfusion Therapy for Blood Transfusion Therapy for ThalassemiaThalassemia
1- Palliative transfusion:1- Palliative transfusion:
It is just to keep patient surviving It is just to keep patient surviving
2- hypertransfusion:2- hypertransfusion:
Maintain a hemoglobin level of 9 to 10 g/dlMaintain a hemoglobin level of 9 to 10 g/dl
To reduce extramedullary erythropoiesisTo reduce extramedullary erythropoiesis
3- Supertransfusion: 3- Supertransfusion:
Maintain a hemoglobin level >12 g/dlMaintain a hemoglobin level >12 g/dl
in an effort to suppress all erythropoiesis.in an effort to suppress all erythropoiesis.
Complications of regular Complications of regular Blood Transfusion Blood Transfusion
HemosiderosisHemosiderosis
Cardiac and Hepatic iron toxicity Cardiac and Hepatic iron toxicity (cirrhosis)(cirrhosis)
Require Require
CChelation therapy with Deferoxaminehelation therapy with Deferoxamine
Expected Anesthetic Expected Anesthetic Difficulties Difficulties
1- Difficult Air Way:1- Difficult Air Way:Hyperplasia of facial bones and narrowing of Hyperplasia of facial bones and narrowing of
nasalnasal
passagepassage
2- 2- Hemosiderosis:Hemosiderosis:Resulting in cardiac dysfunction, supraResulting in cardiac dysfunction, supra
ventricular arrhythmias as well as hepaticventricular arrhythmias as well as hepatic
dysfunctions dysfunctions
Regional Anesthesia Regional Anesthesia
Epidural, spinal, and intrapleural anesthesiaEpidural, spinal, and intrapleural anesthesia
are relatively contraindicated as the presenceare relatively contraindicated as the presence
of extramedullary bone marrowof extramedullary bone marrow increases theincreases the
likelihood of bleeding and hematoma formationlikelihood of bleeding and hematoma formation
Patients receiving palliative transfusionPatients receiving palliative transfusion
therapy are at greatest risktherapy are at greatest risk
Met-hemoglobinMet-hemoglobinMet Met = beyond= beyond
Ferric iron Ferric iron (fe(fe+3+3) ) = one oxidation state = one oxidation state
beyond ferrous iron (Febeyond ferrous iron (Fe+2+2) )
Ferric form of ironFerric form of ironUnable to bind to OUnable to bind to O22, ,
Decreasing ODecreasing O22 carrying capacity carrying capacity
Shifts OShifts O22 dissociation curve to the dissociation curve to the
left Worsening tissue Oleft Worsening tissue O22 release release
EtiologyEtiology
HereditaryHereditaryCongenital absence of Congenital absence of
NADH methemoglobin reductaseNADH methemoglobin reductase
Acquired Acquired Drug inducedDrug induced
Chemical inducedChemical induced
Drugs Causing MethemoglobinDrugs Causing Methemoglobin
All begin with letter AAll begin with letter A
1- Anti hypertensive 1- Anti hypertensive Vaso dilator drugsVaso dilator drugs
2- Anesthetics2- Anesthetics
Benzocaine, Lidocaine, PrilocaineBenzocaine, Lidocaine, Prilocaine
3- Analgesics3- Analgesics
Phenacetin, PhenazopyridinePhenacetin, Phenazopyridine
4- Antiemetic 4- Antiemetic MetoclopramideMetoclopramide
5- Anti leprosy 5- Anti leprosy DapsoneDapsone
6- Anti malarial 6- Anti malarial ChloroquineChloroquine
7- Antibiotics 7- Antibiotics Sulfamethoxazole Sulfamethoxazole
Sign & Symptoms Associated Sign & Symptoms Associated with Methemoglobin blood Conc.with Methemoglobin blood Conc.
Met Hb conc. %total Hb Clinical Met Hb conc. %total Hb Clinical
< 1.5g/dl < 10% None< 1.5g/dl < 10% None
1.5-3 g/dl 10-20% Cyanosis1.5-3 g/dl 10-20% Cyanosis
3-4.5g/dl 20-30% Anxiety3-4.5g/dl 20-30% Anxiety
TachycardiaTachycardia
7.5-10.5 50-70% Acidosis7.5-10.5 50-70% Acidosis
g/dl Arrhythmiag/dl Arrhythmia
> 10.5g/dl >70% Death> 10.5g/dl >70% Death
DiagnosisDiagnosis1- Tachyphylaxis1- Tachyphylaxis
2- Cyanosis despite high FIo2- Cyanosis despite high FIo22
3- Low fixed reading of pulse oximetry3- Low fixed reading of pulse oximetry
4- Normal PO4- Normal PO22 of ABG, but higher of ABG, but higher calculated hemoglobin saturation than calculated hemoglobin saturation than that measured by pulse oximetrythat measured by pulse oximetry
5- Low PH of ABG5- Low PH of ABG
6- Higher than normal O6- Higher than normal O2 2 venous tensionvenous tension
7- Development of cardiac arrhythmias 7- Development of cardiac arrhythmias
Confirmation Of DiagnosisConfirmation Of Diagnosis
Co- oximetryCo- oximetryMasseurs light absorbance at fourMasseurs light absorbance at four
Different wave lengths so it is able toDifferent wave lengths so it is able to
quantify % ofquantify % of
Met Hb Co HbMet Hb Co Hb
Oxy Hb Deoxy Hb Oxy Hb Deoxy Hb
Management Of Acquired Management Of Acquired MethemoglobinMethemoglobin
Mild MethemoglobinemiaMild Methemoglobinemia
Withdrawal of the offending agentWithdrawal of the offending agent
Oxygen administrationOxygen administration
No specific therapyNo specific therapy
Methylene BlueMethylene Blue
Mechanism :Mechanism :Transfers electron to methemoglobinTransfers electron to methemoglobin
reducing it to normalreducing it to normal
Dosage :Dosage :1-2mg/kg iv over 3-5 minutes1-2mg/kg iv over 3-5 minutes
Can be repeated at a dose of 1mg/kg after 30 Can be repeated at a dose of 1mg/kg after 30
minutesminutes
Symptoms expected to diminish within 30-60Symptoms expected to diminish within 30-60
minutesminutes
Cautions: Cautions: Not effective in G6PD deficiency and Not effective in G6PD deficiency and
may cause hemolysis in these casesmay cause hemolysis in these cases