Hemolytic Disease, Thalassemias, Sickle Cell

 Hydrops fetalis  Icterus gravis neonatorum  Kernicterus (outcome)  Erythroblastosis fetalis

 Destruction of RBC  Neonates and infants  Intrinsic (membrane defect,

enzymes)  Acquired (infection, alloimmunization)  Platelets and Granulocytes

DAT

 ABO HDN  Rh HDN  Others

65% 33% 2%

 Maternal Antibody  Fetal antigen  Cross the placental barrier  IgG1 and IgG3  High and low responders  Primary and secondary response  Anamnestic response

 Mom  Baby  Mom

lacks an antigen has an antigen produces Antibody (IgG)

Hereditary disorders that can result in moderate to severe anemia • Basic defect is reduced production of selected globin chains •

•

•

Found most frequently in the Mediterranean, Africa, Western and Southeast Asia, India and Burma Distribution parallels that of Plasmodium falciparum

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Terminology • Silent carrier • Minima • Minor • Intermedia • Major

Hereditary disorders that can result in moderate to severe anemia • Basic defect is production of an abnormal globin chain •

Abnormal hemoglobins discovered earlier have been given letter designations:

Hb S

=

+ Hb S

Hb C

+

Hb SC

=

Sheared in microcirculation

Irreversible Sickle Cell

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Hemoglobin electrophoresis • Major test for identifying thalassemia and

hemoglobinopathy • Types

• Cellulose acetate: Alkaline pH • Citrate agar: Acid ph