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Hereditary disorder characterized by Production of abnormal Hb Hbs
sickle cell AneÉ
1 Homozygous bss occur with mild hypoxia
11 Heterozygous sicklecell trait ABAS occur with severe hypoxia
HemolyticAnemia was
she
11 vaso occlusive crisis spleen Autosplenectomy No splenomegaly
Bones Pain Avascularnecrosis of femurhea
Penis Priapism
Inve Ins
1 Hb electrophoresis
K
1 symptomatic treatment Analgesics 02 therapy IV fluids
Aim to Hbsto2304 c Bloodtransfusion if stroke or visceraldamage
Pneumococcal vaccination
11 Hydroxy urea elevate Hbf Reduce Hbs
Hereditary disorder www.iI
FfFgobinchaincaouBl syneresis
Lead to Hb synthesis
Betfalassaemia coupes
production of β chains than replacedby gamma Y or Delta S
chain Production of Hbf AZT 8 HbA2 az52
1 Thalassemia major Hbf 8 HbA
1 Growth retardation sweatstitffattina
11 Young age onset Smiths Hbf HBA synthesis
111 Hepatosplenomegaly Hypersplenism
IV Bonedeformities Flat bones pathologic fractures
facial appearanceprotrusion of Frontal Parietalbon
Depression of nasal bridges
Hemochromatosis duets Iron overload
i t
ri.no
Hereditary disorder characterized by Production of abnormal Hb Hbs
sickle cell AneÉ
1 Homozygous bss occur with mild hypoxia
11 Heterozygous sicklecell trait ABAS occur with severe hypoxia
HemolyticAnemia was
she
11 vaso occlusive crisis spleen Autosplenectomy No splenomegaly
Bones Pain Avascularnecrosis of femurhea
Penis Priapism
Inve Ins
1 Hb electrophoresis
K
1 symptomatic treatment Analgesics 02 therapy IV fluids
Aim to Hbsto2304 c Bloodtransfusion if stroke or visceraldamage
Pneumococcal vaccination
11 Hydroxy urea elevate Hbf Reduce Hbs
Hereditary disorder www.iI
FfFgobinchaincaouBl syneresis
Lead to Hb synthesis
Betfalassaemia coupes
production of β chains than replacedby gamma Y or Delta S
chain Production of Hbf AZT 8 HbA2 az52
1 Thalassemia major Hbf 8 HbA
1 Growth retardation sweatstitffattina
11 Young age onset Smiths Hbf HBA synthesis
111 Hepatosplenomegaly Hypersplenism
IV Bonedeformities Flat bones pathologic fractures
facial appearanceprotrusion of Frontal Parietalbon
Depression of nasal bridges
Hemochromatosis duets Iron overload
