SHEIKHA
HEMOLYTIC HEMOLYTIC ANEMIASANEMIAS
HEMOLYTIC ANEMIAS
SHEIKHA
Abnormalities of the Environmentof the Red Cells
Abnormalities of the Red Cells
Prematuredestructionof the red cells
HEMOLYTIC ANEMIAS
SHEIKHA
Abnormalities of the Red Cells
HEMOLYTIC ANEMIAS
SHEIKHA
Membrane
HbEnzymes
120 days
HEMOLYTIC ANEMIAS
SHEIKHA
Membrane
Hb
Enzymes
HS
HE
H. Stomatocytosis
HEMOLYTIC ANEMIAS
SHEIKHA
Membrane
Hb
Enzymes
HS
HE
H.St.
Hemoglobinopathies
Thalassemia Sickle
HEMOLYTIC ANEMIAS
SHEIKHA
Membrane
Hb
Enzymes
HS
HE
H.St.
Hemoglobinopathies
Thalassemia Sickle
G6PDH ↓
PK ↓
ALL CONGENITAL
HEMOLYTIC ANEMIAS
SHEIKHA
PNHParoxysmalNocturnalHemoglobinuria
ACQUIRED
HEMOLYTIC ANEMIAS
SHEIKHA
Abn
orm
aliti
es
of th
e E
nviro
nmen
tof
the
Red
Cel
ls
HEMOLYTIC ANEMIAS due to
ABNORMAL RBC ENVIRONMENT
Abnormal Plasma Constituents
IMMUNE: DRUGS
HDN TOXINS
Drugs
AIHA
Abnormal Physical
Environment
Fragmentation
Hypersplenism
Burn
SHEIKHA
WARM CHAD
Infections
Malaria
Cl. Welchii
HEMOLYTIC ANEMIAS due to
ABNORMAL RBC ENVIRONMENT
Abnormal Plasma Constituents
IMMUNE: DRUGS
HDN TOXINS
Drugs
AIHA
SHEIKHA
WARM CHAD
HEMOLYTIC ANEMIAS due to
ABNORMAL RBC ENVIRONMENT
Abnormal Plasma Constituents
IMMUNE:
HDN
Drugs
AIHA
SHEIKHA
WARM CHAD
IMMUNEHEMOLYTIC
ANEMIAS
HEMOLYTIC ANEMIAS due to
ABNORMAL RBC ENVIRONMENT
Abnormal Plasma Constituents
IMMUNE: DRUGS
HDN TOXINS
Drugs
AIHA
SHEIKHA
WARM CHAD
AIHA
WARM
IDIOPATHICAssociated with:
SLECLL
LYMPHOMAOTHER
AUOIMMUNEDISORDERS
MISCELANEOUS
HEMOLYTIC ANEMIAS
SHEIKHA
Coomb’s Test
HEMOLYTIC ANEMIAS due to
ABNORMAL RBC ENVIRONMENT
Abnormal Plasma Constituents
IMMUNE: DRUGS
HDN TOXINS
Drugs
AIHA
SHEIKHA
WARM CHAD
AIHA
CHAD
ACUTE
CHRONIC
ATYPICALMYCO-
PLASMAPNEMONIA
IM IDIO-PATHIC
LYMPH-OMA
HEMOLYTIC ANEMIAS due to
ABNORMAL RBC ENVIRONMENT
Abnormal Plasma Constituents
IMMUNE: DRUGS
HDN TOXINS
Drugs
AIHA
SHEIKHA
WARM CHAD
AIHA
WARM CHAD
PCH
ACUTE
MeaslesMumpsFluIdiopathic
CHRONIC
Idiopathic
Management of AIHAManagement of AIHA• STEROID
• SPLENECTOMY
• RITUXIMAB
• CYCLOSPORIN
• FOLATE
HEMOLYTIC ANEMIAS due to
ABNORMAL RBC ENVIRONMENT
Abnormal Physical
Environment
Fragmentation
Hypersplenism
Burn
SHEIKHA
March Hb-uria
Abnormal Blood Vessels
MAHA Microangiopathic Hemolytic Anemia
TTP/ HUS Thrombotic Thrombocytopenic Purpura / Hemolytic Uremic Syndrome
Mechanical HA From damaged Valves
Malignancy
“MucinSecretingAdeno-
Carcinoma”
Septicemia
MalignantHTN
HEMOLYTIC ANEMIAS due to
ABNORMAL RBC ENVIRONMENT
SHEIKHA
Infections
Malaria
Cl. Welchii
HEMOLYTIC ANEMIAS due to
ABNORMAL RBC ENVIRONMENT
Abnormal Plasma Constituents
IMMUNE: DRUGS
HDN TOXINS
Drugs
AIHA
Abnormal Physical
Environment
Fragmentation
Hypersplenism
Burn
SHEIKHA
WARM CHAD
Infections
Malaria
Cl. Welchii
HEMOLYTIC ANEMIAS
SHEIKHA
HEMOLYTIC ANEMIAS
LABORATORY FINDINGS:LABORATORY FINDINGS:
HyperbilirubinemiaHyperbilirubinemia “mild & unconjugated”“mild & unconjugated”
AnemiaIncreased Urinary UrobilinogenReduced HaptoglobinReticulocytosis & PolychromasiaIncreased Marrow Activity
RBC shape changes: RBC shape changes: SpherocytosisSpherocytosis
FragmentationFragmentation
SHEIKHA
HEMOLYTIC ANEMIAS
SHEIKHA
CLINICAL FEATURES
Jaundice “usually mild”PallorAcholuric jaundiceSplenomegalyGall stonesLeg ulcers
Crises:
Aplastic
Hemolytic
Sequestration
HEMOLYTIC ANEMIAS
SHEIKHA
LABORATORY FINDINGS:LABORATORY FINDINGS:
HyperbilirubinemiaHyperbilirubinemiaAnemiaIncreased Urinary UrobilinogenReduced HaptoglobinReticulocytosis & PolychromasiaIncreased Marrow ActivityRBC shape changes RBC shape changes
CLINICAL FEATURES
Jaundice “usually mild” Pallor
Acholuric jaundice Splenomegaly Gall stones Leg ulcers
HEMOLYTIC ANEMIAS
SHEIKHA
Intravascular Hemolysis
Hemoglobinemia Hemoglobinuria
Hemosiderinuria
HEMOLYTIC ANEMIAS
SHEIKHA
Hemoglobinuria
HEMOLYTIC ANEMIAS
SHEIKHA
Hemosiderinuria
HEMOLYTIC ANEMIAS
SHEIKHA
HEMOLYTIC ANEMIAS
SHEIKHA
HEMOLYTIC ANEMIAS
SHEIKHA
HEMOLYTIC ANEMIAS
SHEIKHA
HEMOLYTIC ANEMIAS
SHEIKHA
HEMOLYTIC ANEMIAS
SHEIKHA
HEMOLYTIC ANEMIAS
SHEIKHA
HEMOLYTIC ANEMIAS
SHEIKHA
HEMOLYTIC ANEMIAS
SHEIKHA
HereditarySpherocytosis
HEMOLYTIC ANEMIAS
SHEIKHA
HEMOLYTIC ANEMIAS
SHEIKHA
HEMOLYTIC ANEMIAS
SHEIKHA
HEMOLYTIC ANEMIAS
SHEIKHA
HEMOLYTIC ANEMIAS
SHEIKHA
HEMOLYTIC ANEMIAS
SHEIKHA
HEMOLYTIC ANEMIAS
SHEIKHA
HereditaryElliptocytosis
MANAGEMENT OF HS
SPLENECTOMY
OP
SI
PN
EU
MO
VA
X
H
ib
Folate
HEMOLYTIC ANEMIAS
SHEIKHA
HEMOLYTIC ANEMIAS
SHEIKHA
Glucose 6 Phosphate Dehydrogenase
Deficiency
HEMOLYTIC ANEMIAS
SHEIKHA
Glucose 6 Phosphate Dehydrogenase Deficiency
Sex-linked200 million
Middle East MediterraneanAfrica
Saudis 13% Kurds 40% Suly Study 6%
Malaria protection
SHEIKHA
G-6-P
F-6-P
F 1,6 DP
DHAP Ga3P 1,3 DPG
3 PG
2 PG PEP
Lactate Pyruvate
Glucose
Glycolysis
Embden-Meyerhof Pathway
SHEIKHA
G-6-P
F-6-P
F 1,6 DP
DHAP Ga3P 1,3 DPG
3 PG
2 PG PEP
Lactate Pyruvate
Glucose
Glycolysis
ATP
ATP
ADP
ADP
ATPATP
ATP
ADP
ADP
OXIDATIVEDAMAGE
SHEIKHA
G-6-P
F-6-P
F 1,6 DP
DHAP Ga3P 1,3 DPG
3 PG
2 PG PEP
Lactate Pyruvate
6PG Ru5P
NADP NADPH*GlucoseNADP NADPH
*
OXIDATIVEDAMAGE
G6PDH
PPP
SHEIKHA
G-6-P
F-6-P
F 1,6 DP
DHAP Ga3P 1,3 DPG
3 PG
2 PG PEP
Lactate Pyruvate
6PG Ru5P
NADP NADPH*GlucoseNADP NADPH
*G6PDH
A- Mediterranean
10% of Blacks10% Activity
HEMOLYTIC ANEMIAS
SHEIKHA
Glucose 6 Phosphate Dehydrogenase Deficiency
Favism
Highly Palatable
EasilyCultivated
Staple Food in G6PDH
areas
HighProtein
High Nutritional Value
HEMOLYTIC ANEMIAS
SHEIKHA
Glucose 6 Phosphate Dehydrogenase Deficiency
FavismSpecific to Fava beans
Peas & others are safe
ErraticOnly in 25% of
G6PDH Deficient Patients
Same Patient“Only Occasionally”
Dosage“Children”
Bean Maturity
FoodProcessing
HEMOLYTIC ANEMIAS
SHEIKHA
Acute drug-induced hemolysis
Favism
Chronic hereditary non-Spherocytic Anemia
Neonatal jaundice
Glucose 6 Phosphate Dehydrogenase Deficiency
HEMOLYTIC ANEMIAS
SHEIKHA
HEMOLYTIC ANEMIAS
SHEIKHA
SHEIKHA
G-6-P
F-6-P
F 1,6 DP
DHAP Ga3P 1,3 DPG
3 PG
2 PG PEP
Lactate Pyruvate
Glucose
2,3 DPG
PK
PK Deficiency
SHEIKHA
Drugs and Chemicals Associated with Hemolysis in G6PDH-Deficient Subjects
Drugs Definite Association Possible Association Doubtful Association
Antimalarials Primaquine Chloroquine Quinacrine
Pamaquine -- Quinine
Sulfonamides Sulfanilamide Sulfadimidine Sulfoxone
Sulfacetamide Sulfasalazine Sulfadiazine
Sulfapyridine Glyburide Sulfisoxalone
Sulfamethoxazole -- --
Sulfones Dapsone -- --
Nitrofurans Nitrofurantoin -- --
Antipyretic/analgesic Acetanilid Aspirin Acetaminophen
-- -- Phenacetin
SHEIKHA
Drugs Definite Association
Possible Association
Doubtful Association
Other drugs Nalidixic acid Ciprofloxacin PAS
Niridazole Chloramphenicol Doxorubicin
Methylene blue Vitamin K analogs Probenecid
Phenazopyridine Ascorbic acid Dimercaprol
Septrin PAS --
Other chemicals Naphthalene Acalypha indica --
Trinitrotoluene -- --
Drugs and Chemicals Associated with Hemolysis in G6PDH-Deficient Subjects
HEMOLYTIC ANEMIAS
SHEIKHA
HEMOLYTIC ANEMIAS
SHEIKHA
HEMOLYTIC ANEMIAS
SHEIKHA
HEMOLYTIC ANEMIAS
SHEIKHA
HEMOLYTIC ANEMIAS
SHEIKHA
HEMOLYTIC ANEMIAS
SHEIKHA
HEMOLYTIC ANEMIAS
SHEIKHA
HEMOLYTIC ANEMIAS
SHEIKHA
HEMOLYTIC ANEMIAS
SHEIKHA
HEMOLYTIC ANEMIAS
SHEIKHA
HEMOLYTIC ANEMIAS
SHEIKHA
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