child with pallor & jaundice (hemolytic anemia)
DESCRIPTION
Child with pallor and jaundice (hemolytic anemia)TRANSCRIPT
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اشرح لي صدري و { ر لي رب يس
اني أمري واحلل عقدة من ل س
}يفقهوا قولي
حيـــــــــــــم حمن الر بســــــــــــــم هللا الر
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Child with pallor and jaundiceby 5th year medical students_ Tripoli university
group 6
(Haemolytic anaemia)
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CONTENTS
• Introduction
• Sickle cell anemia
• Autoimmune (AIHA)
• Thalassemia
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Jaundice is yellowish discoloration of skin and mucous membranes due to ↑ in
blood bilirubin
Pallor is is a pale color of the skin which can be caused by illness, emotional shock or
stress, or anemia,,, and is the result of a reduced amount of oxyheamoglobin in skin or mucous membranes
Anemia is HB level below the normal rage according To age
Neonate <14g/dl1_12 months <10g/dl1-12 year<11g/dl
Anaemia results from the following mechanisms:
1_reduced red cell production - either due to ineffective erythropoiesis (e.g. iron deficiency, the commonest cause of anaemia) or due to red cell aplasia
2_blood loss3-increased red cell destruction (haemolysis)
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What is hemolytic anemia?
It is ↓ red cell life span due to ↑ red cell destruction in the circulation (intravascular) or in liver & spleen (extravascular)
note: BM can ↑ production about 8 fold , so symptoms & signs of hemolytic anemia appear when the BM no longer able to compensate for the premature destruction of RBCs(exeeding BM capacity for compensating)
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Haemolytic anaemia
Red cell mem
disord.
ImmuneHaemoglobinopathiesRed cell enzyme
disord.
Hereditary
Spherocytosis
G6PDH
Def.
Thalassaemia
AIHA
Sickle cell
anemia
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Hereditary spherocytosis: A genetic disorder of the red blood cell membrane
clinically characterized by anemia, jaundice (yellowing) and splenomegalyM.O.IIt is AD disease .
Defect or Deficiency of Beta Spectrin or Ankyrin Loss of membrane surface areabecomes more spherical Destruction in Spleen
Clinical picture*jaundice - usually develops during childhood but may be intermittent; may
cause severe haemolytic jaundice in the first few days of life
*anaemia - presents in childhood with mild anaemia (haemoglobin 9-11g/dl),
but the haemoglobin level may fall with an intercurrent infection; many children have 'compensated' haemolysis with a normal haemoglobin
* splenomegaly - depends on the rate of haemolysis;
* gallstones - due to increased bilirubin excretion
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InvestigationCBCBlood filmOsmotic fragility test
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• Treatment• oral folic acid as they have a raised folic acid
requirement secondary to their increased red blood cell production. .
• Splenectomy is beneficial but is only indicated for poor growth or troublesome symptoms of anaemia (e.g. severe tiredness)
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Autoimmune Hemolytic Anemia:
(AIHA) Autoimmune disease involving auto-antibodies directed toward RBC surface antigens leading to accelerated destrution (hemolysis).
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Etiology:
1. Idiopathic (most common cause).
2. Autoimmune disease: (SLE,RA).
3. Neoplastic: e.g lymphoma, myeloma
4. Drugs: methyldopa,penicillin
5. Infection: mycoplasma, EBV
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Clinical picture:
_ Anemia + jaundice.
_ hand, foot and cyanosis (cold type).
_splenomegaly (warm type).
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Two main types:
%
Site
Antibody
Active temperature
Treatment
Warm AIHA
80% of cases
Extravascular hemolysis
IgG
37 degree
Steroids & splenectomy.
Cold AIHA
20% of cases
Intravascular hemolysis
IgM
4 degree
Warming & plasmapheresis.
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Investigation:
_CBC: normocytic normochromic or macrocytic hyperchromic.
_Blood film: spherocytosis, schistocytes.
_Direct antiglobuline test(Coombs’ test) : +ve
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treatment:
_Treat underlying causes.
_Corticosteroids: response may take 3 weeks
_Splenectomy.
_Immunosuppressive therapy.
_Blood transfusion.
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ThalassemiaGenetic disorders of Hb synthesis with ↓ produc.of either α or ß
polypeptide chains of Hb molecules (α-thalass. or ß- thalass.)
M.O.I : AR
ß -thalass. Carrier Carrier
Diseased
Onset : ≥ 6 months “ complete switch from fetal
Hb α2δ2 to adult Hb α2ß2 “
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HistoryOnset of anemia > 6 months
Symptoms of anemia
FTT
History of frequent blood transfusion
not improve with iron supplement “ if minor”
positive FH
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C\P & Complic.
Thalassaemic face
Causes of mortality: high output HF from sever Anm. or iron overload
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Investigation
MCH↓ &CBC : ↑Retics. \ ↓MCV
Bld film\ target cells (T) ,
Poikilocytes (p) , microcytes (M)
Hb electrophoresis :↑↑Hb F
US “gall stone”
Imaging :
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Pathological fractureSC compression
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Management1-Monthly bld transfusion (aim : Hb >10g\dl , reduce growth failure ,
prevent bone deformation)
2-Iron chelation therapy (each unit of transfused RBCs contain 200mg
elemental iron)
3-Folic acid (hyperactive BM)
4-Splenectomy (if hyperactive \ after vaccination).
5-Cholecystectomy (for bilirubin stone)
*gene therapy “deliver globin gene into cells by viral vector”
*Emerging therapy “induce F-Hb by butyrates”
*BM transplant : (young , HLA match, no organ dysfunction.)
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What is the Difference ß- thalass.minor & IDA?
NOTE :(both are microcytic hypochromic) Iron def. anemiaß-Thalassemia minor
Reticulocytes↓Reticulocytes↑
No abnormal cells , ↓ RDWTarget cells in bld film, normal RDW
Serum ferritin↓Serum ferritin↑
IRBC↑IRBC↓
normal HbHb A2 By electrophoresis↑
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1 α-gene
Normally we have 4 α- globin genes
All α-gene2 α-gene 3 α-gene
Silent
carrier
α-thalass.
traitHHD
Hb-H
α-thalas. major
”Hb barts
hydrops
fetalis”
α- thalassaemia
Asymptomatic
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REFERENCES Illustrated textbook of pediatrics
www.Medscape.com
www.pubMed.com
www.Sehha.com