1. ANEMIAS Dr. Aamer Aleem Consultant Hematologist
Associate Prof. of Medicine
KKUH & College of Medicine
Riyadh

2. Anemia
Anemia is present when a patient has a reduced amount of hemoglobin per unit volume of blood when compared with the correct reference population for that patient.
Males Hb-13-18
Females Hb 12-17

3. ANEMIA Single cell line(RBC) problem Multiple cell line problem
(RBC,WBC,Platelet)
-Bone marrow suppression
-immunologic disorders
-peripheral destruction/sequestration

4. Anemia Anemia is generally defined as a hematocrit
<40% (hemoglobin <13.0 g/dL) in men or
<37% (hemoglobin <12.0 g/dL) in women.
(WHO definition)
Red blood cell (RBC) indices, which include the mean corpuscular volume (MCV), the mean corpuscular hemoglobin (MCH), the mean corpuscular hemoglobin content (MCHC), and the red-cell distribution width (RDW) index, are further used to define types of anemia.

5. Anemia
Despite having a set of peculiar symptoms and signs, anemia is not a disease per se, but a syndrome, as it may arise from an extensive list of causes.
It is the chronic syndrome of highest prevalence in clinical medicine.

6. Anemia Etiology
Based on Hb, red cell indices, retic count and red cell morphology
(1) Inadequate response
A. Hypochromic microcyctic
B. Normochromic Normocytic
C. Macrocytic
(2)Adequate response
R/O blood loss---Includes Hemolytic
disorders

7. Anemia-symptoms What are the symptoms of Anemia ?
General malaise, weakness, fatigue, breathlessness on exertion, palpitations, angina.
Desire to eat sand and clay.
Menorrhagia common in women.

8. Symptoms of Anemia
The central nervous system, the heart and the muscle mass are the most affected organs, since they are the ones that most need oxygen for their functions.
The symptoms increase with physical activity, as this consumes oxygen.
With hemoglobin between 9 and 11 g/dL there's irritability, headache and psychic asthenia; in the elder fatigability is observed, and angina may occur.
With hemoglobin between 6 and 9 g/dL there's tachycardia, dyspnea and fatigue upon the slightest effort.
With hemoglobin below 6 g/dL symptoms are present even in sedentary activities, and when below 3.5 g/dL heart failure is impending and any activity is unfeasible.

9. COMPLETE BLOOD COUNT Hb Conc (g/dl) Hematocrit (PCV) MCV (fl) MCH (pg)
RDW(measure of red cell size variability)
RBC Count (…x109/L )
WbC Count (…x109/L )
Platelet Count (…x109/L)
(Reticulocyte Count) ( % )

10. Red Cell Indices
MCV defines the size of the red blood cells and is expressed as femtoliters (10−15; fl) or as cubic microns (μm3). The normal values for MCV are 87 ± 7 fl.
MCH quantifies the amount of hemoglobin per red blood cell. The normal values for MCH are 29 ± 2 picograms (pg) per cell.
MCHC indicates the amount of hemoglobin per unit volume. In contrast to MCH, MCHC correlates the hemoglobin content with the volume of the cell. It is expressed as g/dl of red blood cells or as a percentage value. The normal values for MCHC are 34 ± 2 g/dl.

11. Red Cell Indices
How to calculate red cell indices

12. Glossary of Useful Blood Count Descriptors
Anisocytosis: red cells of unequal size. Reflected in increased RDW (Red cell Distribution Width.) Dimorphic Blood Film: two populations of red cells - one microcytic and the other normocytic. Seen in treated or transfused iron deficiency, and sideroblastic anemia Howell-Jolly bodies: round nuclear remnants within the red cells. Indicate splenectomy or hyposplenismMacrocytosis: large red cells Erythroblast: any nucleated red cell precursor Hypersegmented neutrophils: a neutrophil with six or more lobes. Usually (but not inevitably) means vitamin B12 or folate deficiency

13. Glossary of Useful Blood Count Descriptors
Hypochromia: pale red cells. Always accompanied by microcytosis Leukoerythroblastic: the presence of erythroblasts and myelocytes (which are precursors of mature cells) in the blood. Often indicates marrow infiltration eg by secondary cancer or fibrosis
Microangiopathy: indicates mechanical damage to red cells with red cell fragments on the blood filmMicrocytosis: small red cells Poikilocytosis: a traditional term for red cells of unequal shape
Polychromasia: grey coloured red cells on film, indicating presence of increased reticulocytes
Reticulocyte: an erythrocyte newly released from the bone marrow

14. Glossary of Useful Blood Count Descriptors
Rouleaux: red cells in stacks, as coins. Indicates high ESR, eg infection, myeloma, cancer, collagen disease, TB etc.
Schistocyte: a red cell which has undergone mechanical damage - synonymous with red cell fragment
Spherocyte: a spherical red cell due to disproportionate membrane loss. Either inherited, or acquired from (usually) immune causes
Sickle cell: a crescent-shaped red cell characteristic of Sickle Cell Anemia
Target cell: red cell with central area of Hb giving the appearance of a target. Seen in many conditions, including IDA, hemoglobinopathy and liver disease

15. Rouleaux
Rouleaux (singular is rouleau) are stacks of red blood cells. They occur when the plasma protein concentration is high, and because of them the ESR (erythrocyte sedimentation rate) is also increased. Conditions which do this include infections, inflammatory and connective tissue disorders, and cancers. In this case, the diagnosis is probably multiple myeloma, as a plasma cell can be seen on the film.

16. Erythroblasts & other BM cells
Erythroblasts are the precursor cells of erythrocytes. They contain nuclei, and in adults are only found in the bone marrow under normal circumstances. Their presence in the blood may indicate either marrow 'stress', as in hypoxia, severe sepsis, or hemolysis, for example, or some fundamental bone marrow pathology such as replacement by secondary cancer.

17. Anisocytosis
Anisocytosis means that the red cells are of unequal size. It is a feature of many anemias, and other blood conditions, and does not have much diagnostic value. The 'red cell distribution width' (RDW) is a quantitative measure of the degree of anisocytosis. The RDW is useful in the differential diagnosis of microcytic anemia. Most cases of iron deficiency have a raised RDW, and most cases of thalassemia trait have a normal RDW.

18. Dimorphic Red Blood Cells
There are two populations of red cells present. One is normocytic, and the other is microcytic. This occurs either because an iron-deficient patient has been transfused or treated with iron, or in the Sideroblastic Anemias.

19. Anemia-diagnosis How can we diagnose Anemia ?
Blood tests to detect the various components of blood is carried out for proper diagnosis.
Red blood cell count may be normal or decreased.
Peripheral blood smear shows pale small cells.
White blood cell count normal or decreased

20. Classification of anemia
Anemia can be either acute or chronic.
In acute anemia (sudden loss of blood), the lack of blood volume in the circulatory system is more important than the deficiency of hemoglobin. A loss up to 10% of blood volume, as that taking place upon blood donation, is well tolerated. Losses between 10 and 20% cause postural hypotension, dizziness and faint. In losses above 20%, there's tachycardia, cold extremities, extreme paleness and hypotension, followed by shock; should the loss surmount 30%, without immediate replacement of intravenous fluids, the shock rapidly becomes irreversible and fatal.
In chronic anemia, there's no decrease in blood volume, which is compensated by an increase in plasma volume.

21. Classification of anemia
Based on MCV
Microcytic anemia
Normocytic anemia
Macrocytic anemai

22. Classification of anemia
Based on underlying process
Blood loss or def. of nutrients
Hemolysis
Failure of production

23. A diagnostic scheme for anemias
Determine the MCV
The most useful initial approach to anemia is based on red cell size. The Mean Corpuscular Volume (MCV) represents a direct measurement of red cell size. When this is reduced, the anemia is referred to as microcytic. It is macrocytic when the MCV is increased, and normocytic when the MCV is normal.

24. Anemia-classification
One way to classify anemia is by RBC size (i.e., MCV), as microcytic, macrocytic, or normocytic. For the microcytic anemias, the etiologic possibilities are iron deficiency, thalassemia, sideroblastic anemia, and the anemias of chronic disease. Severe microcytic anemia (MCV <70 fL) is caused mainly by iron deficiency or thalassemia. Macrocytic anemia may be the result of megaloblastic (folate or vitamin B12 deficiency) or nonmegaloblastic causes. Folate deficiency can in turn be due to either reduced intake or diminished absorption. Severe macrocytic anemia (MCV >125 fL) is almost always megaloblastic. In some rare cases, macrocytic anemia is related to the myelodysplastic syndromes prior to or after chemotherapy.
The causes of normocytic anemias include aplastic anemia, bone-marrow replacement, pure red-cell aplasia, anemias of chronic disease, hemolytic anemia, and recent blood loss. A number of anemias have a genetic etiology. Examples of such inherited disorders include hereditary spherocytosis and sickle-cell (SC) anemia

26. Hypochromic Microcytic Anemia D/D
Microcytic anemias
the etiologic possibilities are
Iron deficiency
Thalassemia
Sideroblastic anemia
Anemias of chronic disease.
Severe microcytic anemia (MCV <70 fL) is
caused mainly by iron deficiency or thalassemia.

27. Normochromic Normocytic Anemia D/D
Chronic inflammatory disease— (1)infection (2)collagen vascular disease (3)inflammatory bowel disease
Recent blood loss
Malignancy/Marrow infiltration
Chronic renal failure
Transient erythroblastopenia of chidhood
Marrow aplasia/hypoplasia
HIV infection
Hemophagocytic syndrome

28. Macrocytic Anemia D/D Megaloblastic anemias
• Vit.B12 def. - (1) pernicious anemia
(2) malabsorption
• Folate def. - (1) malnutrition (2) malabsorption
(3) chronic hemolysis (4)drugs - phenytoin, sulfa
Hemolysis
Myelodysplastic syndrome
Marrow failure - Aplastic anemia
Chronic liver disease
Hypothyroidism

29. Macrocytic anemia D/D
Macrocytic anemia may be the result of megaloblastic (folate or vitamin B12 deficiency) or nonmegaloblastic causes. Folate deficiency can in turn be due to either reduced intake or diminished absorption. Severe macrocytic anemia (MCV >125 fL) is almost always megaloblastic.

30. The Normocytic Anemias
These may be classified as follows:
underproduction of erythrocytes due to
(1) the anemia of chronic disease
(2) marrow failure
(3) renal failure (decreased erythropoietin)
loss or destruction of erythrocytes due to
(1) hemolysis
(2) acute blood loss
The reticulocyte count is useful in drawing this distinction, being elevated in (b) and reduced in (a).

31. The Normocytic Anemias
The causes of normocytic anemias include aplastic anemia, bone-marrow replacement, pure red-cell aplasia, anemias of chronic disease, hemolytic anemia, and recent blood loss. A number of anemias have a genetic etiology. Examples of such inherited disorders include hereditary spherocytosis, sickle-cell (SC) anemia, and thalassemia

32. Hemolytic Disorders Hemoglobinopathy– Hb SS,SC,S-Bthal
Enzymopathy--G6PD def, PK def
Membranopathy—Hereditary spherocytosis, elliptocytosis
Extrinsic factors—DIC, TTP, HUS,
Immune hemolytic anemia---Autoimmune, Drug induced

33. Diagnostic Approach-History
Age : Iron def rare without blood loss before 6 months in term infants.
Family Hist &Genetics:
(1)X-linked: G6PD deficiency
(2)Aut dominant: Spherocytosis
(3)Aut recessive:Sickle cell,Fanconi anemia
(4)Family member with early age of
cholecystectomy/splenectomy
(5)Ethnicity (Thalassemia: Mediterranean;
G6PD def: (Greeks, Blacks, Middle eastern) (6)Race:B-thal:Mediterranean,African,Asian;
A-thal: Blacks, Asians

34. Diagnostic Approach-History
Nutrition: (1)Cows milk diet:iron def.
(2)Strict vegetarian:Vit B12 def.
(3)Goats milk: Folate def.
(4)Pica: Plumbism,Iron def.
(5)Cholestasis, malabsorption:Vit E def
Drugs: (1)G6PD: oxidants (sulfa, primaquine) (2)Immune mediated hemolysis(penicillin) (3)Bone marrow suppression(chemotherapy) (4)Phenytoin increases folate requirement

35. Diagnostic Approach-History
Diarrhoea-Malabsorption of VitB12/E/Fe Inflammatory bowel disease and anemia of chronic disease with or without blood loss Milk protein intolerance induced blood loss Intestinal resection: Vit B12 def
Infection: Giardia:iron malabsorption Intestinal bacterial overgrowth:Vit B12 def Fish tapeworm:Vit B12 def EBV,CMV,Parvovirus:BMsuppression Mycoplasma,Malaria:hemolysis Hepatitis:aplastic anemia Endocarditis, HIV

36. Physical exam reveals presence and potential causes of anemia
Fever-acute infection,intravascular disease,collagen vascular disease
Jaundice suggests hemolysis
Petechia & Purpura—bleeding tendency
Hypertension & edema-renal disease
Hepatosplenomegaly and lymphadenopathy—infiltrative disease
Growth failure or poor wt. gain—Anemia of chronic disease or organ failure
Examine stool for blood; urine for hemoglobinuria
(Admit & tranfuse if signs of CHF)

37. Physical Findings in Anemia
Skin: Hyperpigmentation, café-au-lait spots Fanconi anemia Petechia & purpura-BM infiltration, autoimmune hemolysis & thrombocytopenia Erythematous rash-Parvovirus, EB virus Butterfly rash-SLE ; Vitiligo-Vit B12 def.
Head:Frontal bossing-Thalassemia major,
Microcephaly-Fanconi anemia
Mouth: Glossitis-B12 def, iron deficiency
Angular stomatitis-Iron deficiency Pigmentation-Peutz Jeghers syndrome Telangiectasia-Osler Weber Rendu syndrome

38. Physical Findings in Anemia
Extremities:Absent thumb-Fanconi anemia -Spoon nails-Iron deficiency Dystrophic nails-Dyskeratosis congenita
CNS-Irritable, apathy-Iron def Peripheral neuropathy-lead poisoning Ataxia, post.column signs-Vit B12 def Stroke-Sickle cell anemia
Short stature-Fanconi anemia, Malnutrition

40. Laboratory Evaluation
Hematology: Complete Blood Count, Retic count, Peripheral smear, ESR, G6PD, Sickling(+/- inf),
Hb electrophoresis, Coombs Test, Osmotic fragility test, BM aspiration
Biochemistry: LFT, UE, RFT, S.Ferrtin, S.Haptoglobin, Iron, VitB12, Folate, Ceruloplasmin
Serology:Heterophil antibody, ANA,Viral
Urinalysis, microscopy, culture/sensitivity
Stool exam. for ova, parasites, occult blood
Endoscopy: upper and lower bowel
Imaging: US Abdomen, Skeletal radiographs, Tc pertechnetate scan for Meckels diverticulum
Tissue biopsy: skin, lymph node, liver

41. Laboratory Evaluation
Hematology: Complete Blood Count, Retic count, Peripheral smear, ESR, G6PD, Sickling(+/- inf),
Hb electrophoresis, Coombs Test, Osmotic fragility test, BM aspiration
Biochemistry: LFT, UE, RFT, S.Ferrtin, S.Haptoglobin, Iron, VitB12, Folate, Ceruloplasmin
Serology:Heterophil antibody, ANA,Viral
Urinalysis, microscopy, culture/sensitivity
Stool exam. for ova, parasites, occult blood
Endoscopy: upper and lower bowel
Imaging: US Abdomen, Skeletal radiographs, Tc pertechnetate scan for Meckels diverticulum
Tissue biopsy: skin, lymph node, liver

42. Microcytic Anemia TEST Iron def ThalMin An of ch dis
S.Iron low normal normal
S.Ferritin low N/H N/H
Marrow iron low N/H N/H
Hb A2 or F N H=Bthal N
N=Athal
RDW high normal N/H
MCV ÷ RBC - > < 13
Sickle/B-thal – Hb S > Hb A
Absence of microcytosis in both parents excludes B-thal or Sickle/B-thal but not A-thal

43. Anemia-treatment
How can Anemia be treated ? As there are many factors causing anemia, the main treatment is for the cause.
Specific treatment of disorder causing anemia.
Replacement of missing factors (iron, vitamin B12, folate)
In many chronic anemias regular blood transfusions are needed (thalassemia, myelodysplastic syndrome)
Blood transfusion is usually needed in acute blood loss or if the patient has severe symptoms or in heart failure
(Problems of blood transfusion)

44. Iron deficiency anemia (IDA)
It is a condition when supply of iron in the body to bone marrow falls short of that required for the production of red blood cells. It is the commonest cause of anemia throughout the world.

45. Iron Deficiency Anemia
The incidence of anemia in the general population is about 1.5%.
Iron deficiency related to inadequate replacement of lost iron is the most frequent cause of asymptomatic anemia and has a variety of causes.
Iron deficiency is common among women of childbearing age; 10% to 20% of menstruating women have abnormally low concentrations of hemoglobin (usually <12 g per 100 mL).

46. Iron Deficiency Anemia
What are the causes of IDA ?
Increased physiological demand for more red blood cells
eg: increased physical activity.
In children, during spurts of growth.
In women during menstruation, pregnancy, parturition
and lactation.
Inadequate dietary intake due to poor economic reasons
or deficient foods.
Decreased absorption due to disorders in the digestive
system.
GI blood loss Peptic ulcer, piles, hiatus hernia, carcinoma of
stomach, carcinoma colon, chronic ingestion of a certain type
of pain relievers, hookworm infestation.

47. Iron Deficiency Anemia
Between 20% and 60% of pregnant women have hemoglobin levels <11 g per 100 mL. Anemia was found in 6% of white women and 17% of black women during the first trimester and in 25% of white women and 46% of black women during the third trimester.

48. Iron Deficiency Anemia
The high rates of anemia in pregnant women are not attributable to iron deficiency alone, however. In a large study of low-income, urban, mostly minority pregnant women, only 12.5% were iron deficient. Women progressing through pregnancy develop a lowered hematocrit as a result of physiologic hydremia related to a disproportionate increase in plasma volume.

49. Iron Deficiency Anemia
The daily oral iron requirement for men and postmenopausal women is 0.5 and 1 mg
Menstruating and pregnant women have higher requirements: 2 mg and 2.5 mg, respectively.
The replacement of lost iron is complicated by the fact that only 5% to 10% of the 10 to 20 mg of the iron in the average adult diet is absorbed.
Anemia is less likely to occur in women taking birth control pills and more likely to occur in women with intrauterine devices.

50. Iron Deficiency Anemia
Because men and postmenopausal women rarely develop iron deficiency that is not related to gastrointestinal blood loss (often occult), an evaluation of gastrointestinal tract must be performed when an iron deficiency is detected in these individuals

51. IDA-Practice Point Iron def. is common in children 9mo-3yr
Iron def. anemia in a child over 3yr should prompt consideration of occult blood loss.
Infants less than 6months generally do not develop iron def.; the exception to this rule is premature infants,who are at risk of iron def.at 4mo, if iron supplementation is not given.

52. Iron Deficiency Anemia-Diagnosis
Red blood cell count may be normal or decreased.
Peripheral blood smear shows pale small cells.
Aniso-poikilocytosis
White blood cell count usually normal
Serum iron is reduced
Total iron binding capacity of blood shows an increase.
Low serum ferritin

53. Iron Deficiency Anemia-Treatment
Correction of iron deficiency - to restore hemoglobin level.
To replenish iron stores.
Oral iron administration is advised. (Side effects of oral iron)
Parenteral iron may be needed occasionally
Treat the underlying cause

54. Anemia of Chronic Disease
Patients with cancer, infection, or inflammation, commonly have a mild-to-moderate anemia caused by red cell underproduction. This 'anemia of chronic disease' is very common, and is usually normocytic. Some cases develop abnormalities of iron metabolism, in which case there may be a microcytosis.

55. Hemolysis
Hemolysis is defined as the premature destruction of red blood cells, from whatever cause.
Recognising the presence of hemolysis
The simplest tests are
Raised reticulocyte count
Raised indirect (unconjugated) bilirubin
Raised serum LDH
Diminished serum haptoglobin concentration. Further more sophisticated testing may be needed in some cases.

56. Hemolysis
A Direct Coombs' Test tells you whether the red blood cells are antibody-coated, and, in the presence of hemolysis, indicates an immune-mediated process (Autoimmune hemolytic anemia).
Note that many patients with a positive Direct Coombs' test do not have hemolysis.

57. Hemolysis Principal causes Inherited abnormalities
Membrane (Hereditary Spherocytosis)
Hemoglobin (Sickle Cell Anemia)
Enzymes (Glucose-6-phosphate
dehydrogenase (G6PD) deficiency)

58. Hemolysis Acquired causes Immune
Warm and Cold Autoimmune Hemolytic Anemia
Non-immune
Mechanical Damage from leaky heart valves
Microangiopathic hemolytic anemia (MAHA) like TTP, HUS & DIC

59. Lead Poisoning Hypochromic microcytic anemia
Associated iron deficiency
Child has pica and is exposed to lead paint or lead dust
Blood smear shows basophilic stipling and blood lead is elevated.
Removal from exposure,chelation therapy and correction of iron deficiency are important.

60. THALASSEMIA Normal Hb is a tetramer of 2 alpha and 2 beta chains
Alpha-thalassemia:decrease or total lack of alpha globin synthesis
Beta-thalassemia:decrease or total lack of beta globin synthesis

61. THALASSEMIA Clinical classification- Silent carrier(AorB):normal CBC
Thal trait(AorB):mild anemia(HM)
HbHdisease(A-thal):moderately severe hemolytic anemia,icterus,splenomegaly
Severe Beta-thal:severe anemia,growth retardation,hepatosplenomegaly,bony def.
Thalassemia major:tranfusion dependent
Thal-intermedia:no regular transfusions

62. THALASSEMIA-complications
HbH disease:severe hemolytic anemia,spenomegaly,hypersplenism
Thal-major:poorly trasfused-skeletal abnormalities,growth retardation,CHF
Thal-major:well transfused with iron overload-(1)Endocrine disturbances:delayed puberty,growth retardation,diabetes mellitus,hypothyroidism (2)Cardiac:arrhythmias, congestive heart failure (3)Hepatic:cirrhosis,liver failure

63. THALASSEMIA-Lab Thal trait:Hb 9-10 g/dl HbH disease:Hb 6-7 g/dl
Thal intermedia:Hb 7-8 g/dl
Thal major:Hb less than 5 g/dl
Peripheral smear:hypochromic,microcytic, anisopoikilocytosis,target cells
Hb electrophoresis: (1)Thal trait-HbF 1-5%, HbA %,rest HbA (2)Thal major- HbF %,HbA2 2-7%,HbA 0-60%

64. THALASSEMIA-therapy Red cell transfusion 3-4 weekly-Hb 9-10
Chelation therapy with desferrioxamine
Splenectomy if transfusion >200ml/kg/yr
Folic acid 5mg daily
Penicillin prophylaxis to all splenectomised
Pneumococcal and Hib vaccine before sply.
Cholecystectomy for gall stones
Bone marrow transplantation is curative
Genetic counselling

65. SICKLE CELL DISEASE SA :Sickle cell trait-asymptomatic
SS :Sickle cell anemia
S-Bthal:Sickle cell-beta thal
SC :Hb SC disease
Pathophysiology:Valine replaced by glutamic acid at Beta 6 position With deoxygenation HbS crystallises&gels

66. Clinical features Anemia:chronic,onset at 3-4 mo
Aplastic crisis:parvo virus B12
Sequestation crisis:usuallyspleen
Hemolytic crisis
Dactylitis:Hand foot syndrome(infant)
Painful crisis:muscle,bone,bone marrow,lung, intestines
Cerebrovascular accidents
Acute chest syndrome:infection,infarction,emb
Chronic lung disease:pulmonary fibrosis,restictive lung disease,cor pulmonale

67. Clinical features Priapism Ocular:retinopathy
Gall bladder disease:stones,cholecystitis
Renal:hematuria,conc.deficit,nephropathy
Cardiomyopathy
Skeletal:avascular necrosis of femoral head
Leg ulceration:in older pts
Infections:pneumococcal pneumonia,meningitis, arthritis,Hinf sepsis,salmonella&staph osteomyelitis,mycoplasma pneumonia,viral infe
Growth failure,delayed puberty
Psychologic problems:chronic illness,chronic pain

68. THERAPY Anemia is usually chronic&compensated
Blood transfusion only given based on clinical condition,Hb level&retic count
Crisis:Splenic sequestration crisis,aplastic crisis,hyperhemolytic crisis-in all of these PRBC is indicated when anemia is sympto.
Pain crisis:IVF,analgesia with narcotics,NSAIDs
Acute chest syndrome:O2,judicial use of analgesics&fluids,antibiotics,PRBC
Stroke:O2,fluids,exchange transfusion
Hydroxyurea:decrease number&severity of VOC
Bone marrow transplantation

69. G6PD deficiency Episodic hemolysis on exposure to oxidants
Severity of hemolysis depends on the enzyme variant
Gene for G6PD is on X chromosome
Jaundice,dark urine(bilirubin,hemoglobin),
Red cells appear blistered
G6PD levels may be normal with hemolysis
Therapy:PRBC,IVF,urine alkalinisation
Prevention:avoid oxidants,fava beans,henna