RBC and Bleeding Disorders Flashcards
(25 cards)
Autosomal dominant Defects in membrane skeleton Extravascular Hemolysis Spherical cells without central pallor Splenectomy
Hereditary spherocytosis
Hereditary spherocytosis is thorugh a defect in membrane skeleton such as
Spectrin
Ankyrin
X linked recessive
Decreased NADPH for glutathione reduction
Intravascular and extravascular hemolysis
Heinz bodies
Bite cells
G6PD Deficiency
Autosomal recessive
Mutation on the 6th codon of B globin gene
Howell Jolly bodies- nuclear remnants
Target cells
Protective against malaria
Increased susceptibility to encapsulated org.
Treatment: hydroxyurea
Sickle cell anemia
What is the mutation of the 6th codon in the B globin gene?
Glutamate ➡️Valine
Protective against malaria Decreased synthesis of glibin chains Intrinsic extravascular hemolysis Symptoms at 5-6 months of age Shift from HbF to HbA synthesis
Beta thalassemia
Protective against malaria Decreased synthesis of glibin chains Intrinsic extravascular hemolysis Symptoms at birth Hemoglobin Barts More stable
A- thalassemia
Most common nutritional disorder in the world
Iron Deficiency Anemia
Principal site of iron absorption
Proximal duodenum
Serum Fe- dec
Transferrin- inc
Ferritin- dec
IDA or chronic disease?
IDA
Serum Fe- dec
Transferrin- dec
Ferritin- inc
IDA or chronic disease?
Anemia of chronic disease
Chronic primary hematopoietic failure with pancytopenia
Most common known cause is drugs
Normocytic, normochromic anemia
Hypocellular marrow
Aplastic anemia
Prothrombin time measures what pathway of coagulation?
Extrinsic
Activated partial thromboplastin time measures what pathway of coagulation?
Intrinsic
Childhood Due to postviral illness Antibodies against gpIIb-IIIa and gbIb-IX Increased megakaryocytes Splenic congestion, large platelets Self limited
Acute Idiopathic Thrombocytopenic Purpura
40 years old Due to SLE, HIV, CLL Antibodies against gpIIb-IIIa and gbIb-IX Increased megakaryocytes Splenic congestion, large platelets Glucocorticoid treatment Splenectomy
Chronic Idiopathic Thrombocytopenic Purpura
Microangiopathic hemolytic anemia ADAMTS13 level is decreased Thrombocytopenia Renal failure Neurologic manifestations
Thrombotic thrombocytopenic purpura
HUS is caused by
E coli O157:H7
Deficiency of gp1b-IX
No platelet adhesion
Thrombocytopenia
Bernard Soulier
gpIIb-IIIa deficiency
Prevent platelet aggregation
Normal platelet count
Glanzmann Thrombasthenia
Most common inherited bleeding disorder
Von Willebrand disease
Autosomal dominant coagulation disorder
Normal platelet count
Impaired platelet function
von Willebrand disease
Most common hereditary syndrome associated with life threatening bleeding
Hemophilia
X linked recessive coagulation disorder
Platelet count and function normal
Hemophilia
