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In the name of God

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Title: In the name of God Author: armoafi Last modified by: alireza Created Date: 5/16/2004 7:57:31 PM Document presentation format: On-screen Show (4:3) – PowerPoint PPT presentation

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Title: In the name of God


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DEFINITION OF ANEMIA
  • Anemia may be defined as a reduction in red blood
    cell mass or blood hemoglobin concentration.
  • It is particularly important to use age and sex
    adjusted norms when evaluating a pediatric
    patient for anemia

4
Normal limit of Hb in children
5
CLASSIFICATIONS OF ANEMIA
  • Physiologic classification
  • Morphologic classification
  • Mean corpuscular volume

6
Anemia
  • Blood loss (acute) Normal spleen, high retic
    count, normal bilirubin, normal urinalysis
  • Hemolytic anemia Splenomegaly, increased
    reticulocyte count, high bilirubin (indirect),
    urobilinogen or hemoglobinuria
  • Impaired red cell formation Normal spleen, low
    retic count, normal bilirubin, normal urinalysis
  • Retic, bilirubin, spleen, U/A,

7
Approch to anemia blood loss(Acute) (Normal
spleen, high retic count, normal bilirubin,
normal urinalysis)
  • Neonatal problem (fetofetal transfusion,
    fetomaternal transfusion,)
  • Hemorrhagic disease of newborn
  • Meckels diverticulum, ..

8
Anemia
  • Blood loss (acute)
  • Hemolytic anemia Splenomegaly, increased
    reticulocyte count, high bilirubin (indirect),
    urobilinogen or hemoglobinuria
  • Impaired red cell formation

9
Approch to anemia Hemolytic anemia
(Splenomegaly, increased reticulocyte count, high
bilirubin (indirect), urobilinogen or
hemoglobinuria)
  • 1) Corupuscular
  • 2) Extra corpuscular
  • Enzyme defect,
  • Membrane defect,
  • Hemoglobin disorder
  • immune,
  • non immune

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Hemolytic anemia Corpuscular
  • 1) Enzyme defect
  • G6PD deficiency
  • PK deficiency

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Glucose 6 phosphate dehydrogenase(G6PD)
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G6PD DEFICIENCY
  • X-linked disorder
  • Affecting 200 to 400 million people
  • Contains 515 amino acids
  • Over 400 variant enzymes have been reported (90
    according to specific mutations)

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Classification of G6PD variants
  •  Class I variants are rare, have severe enzyme
    deficiency (less than 10 percent of normal) and
    have chronic hemolytic anemia(44 variants)
  • Class II variants have severe enzyme
    deficiency, but there is usually only
    intermittent hemolysis (28 variants)
  •  Class III variants have moderate enzyme
    deficiency (10 to 60 percent of normal) with
    intermittent hemolysis usually associated with
    infection or drugs (16 variants)
  • Class IV variants have no enzyme deficiency or
    hemolysis(2 variants)
  • Class V variants have increased enzyme activity
           

14
PATHOPHYSIOLOGY OF G6PD DEFICIENCY
  • The in vivo half-life of enzyme normal enzyme
    (G6PD B) 62 days, G6PD A- 13 days, G6PD
    Mediterranean in hours
  • G6PD A- Patients usually have hemolysis that is
    mild and limited to older deficient erythrocytes
    (class III).
  • G6PD B(Mediterranean) red cells of all ages are
    grossly deficient (class II).

15
DIAGNOSIS
  • Clinical presentation (should be considered in
    the differential diagnosis of any nonimmune
    hemolytic anemia)
  • Laboratory findings Hemolysis (Hb, Retic count,
    bilirubin, hemoglobinemia, hemoglobinuria), PBS
    (polychromatophlia,Heinz bodies, bite cells
    ),Rate of NADPH generation

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Hemolytic anemia Corpuscular
  • 1) Enzyme defect
  • G6PD deficiency
  • PK deficiency

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Pyruvate kinase deficiency
  • Clinical presentation
  • a broad spectrum of clinical and hematologic
    findings occurs, ranging from a mild, completely
    compensated hemolytic state to severe anemia.
  • Anemia and hyperbilirubinemia may occur in the
    neonatal period.
  • In the older patient, pallor, scleral icterus,
    and splenomegaly are usual findings.

19
Pyruvate kinase deficiency
  •  Laboratory findings
  • Elevated reticulocyte count.
  • A few small spiculated erythrocytes
  • No increased number of spherocytes
  • Osmotic fragility is normal.

20
Hemolytic anemia Corpuscular
  • Enzyme defect
  • Membrane defect
  • Spherocytosis,
  • Elliptocytosis

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Membrane defect Spherocytosis
  •  Pathophysiology A deficiency or abnormality of
    the erythrocyte membrane structural protein
    spectrin , ankyrin, band 3, and protein 4.2.
  • The spherocyte is relatively rigid and
    non-deformable

22
Membrane defect Spherocytosis
  • Clinical presentation anemia, hyperbilirubinemia,
    splenomegaly Expansion of the marrow cavities .
  • Laboratory findings reticulocytosis, anemia,
    hyperbilirubinemia, spherocyte in PBS, Erythroid
    hyperplasiain BMA, osmotic fragility test,
    Autohemolysis,

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Hemolytic anemia Corpuscular
  • 1)Enzyme defect
  • 2) Membrane defect
  • 3) Hemoglobin disorder
  • Normal variant,
  • Functional disorder,
  • Structural problem,
  • Thalassemia

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Hemolytic anemia Hemoglobin disorder
  • Normal variant,
  • Functional disorder,
  • Structural problem,
  • Thalassemia

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Hemolytic anemia Extracorpuscular
  • Immune
  • Nonimmune
  • Alloimmune,
  • isoimmune,
  • Autoimmune
  • HUS syndrome,
  • TTP,
  • DIC,
  • Infection,
  • Burn,
  • Hypersplenism, ..

38
Typical Hemolytic Uremic Syndrome
  • In the majority of cases, Stx HUS is associated
    with strains of Escherichia coli that produce a
    Shiga toxin
  • D HUS associated with Shigella dysenteriae
    serotype 1 will be included in the discussion of
    Stx HUS
  • Cases of HUS in children due to Shiga
    toxin-producing E. coli infections other than
    colitis (e.g. UTI) can occur .

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Anemia
  • Blood loss (acute)
  • Hemolytic anemia
  • Impaired red cell formation Normal spleen, low
    retic count, normal bilirubin, normal urinalysis

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Approch to anemia Impaired red cell formation
(Normal spleen, low retic count, normal
bilirubin, normal urinalysis)
  • 1)Deficiency
  • 2)Bone marrow failure
  • 3)Bone marrow infiltration

Iron deficiency Megaloblastic anemia vitamins,
Thyroxine deficiency
Failure of a single cell line Failure of all
cell line
Malignant Non malignant
43
Approch to anemia Impaired red cell formation
(Normal spleen, low retic count, normal
bilirubin, normal urinalysis)
  • 1)Deficiency
  • 2)Bone marrow failure
  • 3)Bone marrow infiltration

Iron deficiency Megaloblastic anemia
vitamins Thyroxine deficiency

44
Clinical manifestation of IDAHematological
symptoms
  • The most common presentation of IDA is an
    otherwise asymptomatic, well nourished infant or
    child who has a mild to moderate microcytic,
    hypochromic anemia

45
Clinical manifestation of IDA Non hematological
symptoms
  • Neurodevelopmental and Cognitive function
  • Immunity
  • Exercise capacity
  • Pica and pagophagia
  • Thrombosis
  • Epithelial change dysphagia, esophageal web,
    atrophic glossitis, spoon nails, blue sclerae

46
Approch to anemia Impaired red cell formation
(Normal spleen, low retic count, normal
bilirubin, normal urinalysis)
  • 1)Deficiency
  • 2)Bone marrow failure
  • 3)Bone marrow infiltration

Failure of a single cell line CPRA, TEC,
A.crisis Failure of all cell line Aplastic anemia
47
Fanconi AnemiaClinical features
  • Incidence is 3/1,000,000
  • Heterozygote frequency 1/300 in U.S. and Europe
  • Median age at diagnosis is 5-7
  • Median survival is 20-30 yrs.
  • Phenotypic variability occurs even within families

48
Frequency of abnormalities in FA
Abnormality Frequency ()

Skeletal (radial ray, hip, vertebral scoliosis, rib) 71
Skin pigmentation (café au lait, hyper- and hypopigmentation) 64
Short stature (median height 5th ile) 63
Eyes (microphthalmia) 38
Renal and urinary tract 34
Male genitalia 20
Mental retardation 16
Gastrointestinal (eg, anorectal, duodenal atresia) 14
Cardiac abnormalities 13
Hearing 11
Central nervous system (eg, hydrocephalus, septum pellucidum) 8
No abnormalities 30


49
Approch to anemia Impaired red cell formation
(Normal spleen, low retic count, normal
bilirubin, normal urinalysis)
  • 1)Deficiency
  • 2)Bone marrow failure
  • 3)Bone marrow infiltration

Malignant Leukemia Non malignant Metabolic
disease Osteopetrosis
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Fast Cases
53
  • A 15 yo girl with a history of systemic lupus
    erythematosus comes to the clinic for evaluation
    of fatigue and pallor.
  • Hgb is 7.2, MCV 85, Retic 10, WBC 3.5 (50
    polys, 40 lymphs, 10 atypicals), and plts of
    125.
  • The smear shows microspherocytes and rouleaux
    formation. She does not have a history of blood
    loss.
  • What does she have?
  • Autoimmune hemolytic anemia

54
  • 17 mounts age infant with an unexplained
    persistent anemia
  • History of neonatal jaundice
  • Requirement of pRBC transfusion 2 early infancy
  • Hb stable 7-9 gr/dl from age 3-17 months
  • spleen 5 cm below costal margin
  • Retic 20, with indirect hyperbilirubinemia
  • What does she have?
  • Pyruvate kinase deficiency

55
  • A 3 yo girl has had diarrhea, sometimes bloody,
    for several days. Now, she has fever, edema,
    petechiae hypertension.
  • The CBC shows Hgb 7.5, MCV 79, Retic 15, Plts
    35, and WBC 13.5 with a normal differential. The
    smear shows several helmet cells and
    polychromasia and confirms the thrombocytopenia
  • What does she have?
  • Hemolytic uremic syndrome

56
  • A 3 month old boy is brought to the ED lethargic
    and with a fever of 40 C. While attending to
    the airway, correcting hypotension and initiating
    antibiotics, the nurse informs you of the CBC
    results Hgb 5.8, MCV 81, Retic 16, WBC 23.5
    (56 polys, 24 bands, 10 lymphs, 10 atypical
    lymphs) and plts of 350.
  • He has blood on dipstick analysis of his urine
    with no RBCs on microscopic analysis.
  • The blood shows polychromasia, and several bite
    cells.
  • What does he have?
  • G6PD deficiency

57
  • A 9 month old boy comes the Dr.s office for
    evaluation of a cold. Further hx reveals
    introduction of whole cows milk at 5 months and
    no well baby visits.
  • He appears quite pale and has the following
    findings Hgb 5.3, MCV 48, plts 780, WBC 12.5
    and retic 1.7
  • What does he have?
  • Iron Deficiency anemia

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  • An 18 month old girl was in good health until she
    developed a cold 10 days ago.
  • Now she is pale but without visible jaundice.
  • The CBC shows a Hgb of 6.8, MCV of 78 and retic
    of 0.1, WBC Plts are normal as is the
    peripheral blood smear.
  • What does she have?
  • Transient Erythroblastopenia of
    Childhood

59
  • The CBC of an 8 yo girl reveals the following
    Hgb 10, MCV 102, Retic 0.4, WBC normal, Plts
    125.
  • She is slightly short, has several large
    café-au-lait spots, is performing below average
    in school and has short thumbs.
  • What does she have?
  • Fanconis Anemia

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  • You are asked to assist in the care of political
    refugees from Afghan. On the screening entrance
    exam you note that one of them, a 2 yo girl, is
    very pale and lethargic. Her spleen is down to
    the level of the umbilicus.
  • The labs reveal Hgb 6.3, Retic 6, MCV 55, with
    normal WBC plts.
  • The smear shows many target cells, polychromasia,
    basophilic stippling, red cell fragment,
    anisocytosis and hypochromia.
  • What does she have?
  • ? thalassemia major

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People may doubt what you say
but they will believe what you do
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