RBC Disorders Flashcards
What is hemoglobin
Conjugated protein that serve as vehicle for transportation of o2 and co2
Packed red cell volume
Raised blood cells of RBC to the total blood called
Hematocrit
Anemia characteristics and features
Reduce in total circulating RBC
May be MCV IS micro, normo, macrocytic
MCHc is hypo or normochromic
Microcytic anemia disease
T-Thalaseemia
A-Anemia of chronic disease
I- Iron deficiency
L-Lesd poisoning
Macrocytic disease
Megaloblastic and non megaloblastic
Defective in DNA synthesis, they are
Folate deficiency
Vit B12 deficiency
Orotic aciduria
Fanconi anemia
Hemolytic anemia common features
Shortened life span
Elevated erythropoietin
Reticulocytosis
Accumulation of hemoglobin degraded products like jaundice, gallstones and hemosiderosis
Hereditary sperocytosis
It’s a dominant disorder.
Defect in cell membranes skeleton like spectrin, ankyrin, band 4.2,and band 3
Features of hereditary sperocytosis
Normocytic
Hyperchromic
Intrinsic damage and extravascular hemolysis in spleen
Anisocytosis
Spherocytes
Howen jolly bodies
Glucose 6 phosphate dehydrogenase deficiency
X linked recessive
Causes of hemolysis in G6PD
Intrinsic and episodic hemolysis
Both intra and extravascular hemolysis
In intra heinz bodies cause membrane damage
Extravascular has heinz bodies are bittenoff by spleen
Main features seen in G6PD deficiency
Heinz bodies
Bite cells or degmacytes
Protective against p. Falciparum
Sickle cell disease
Autosomal recessive
Point mutation on 6th codon of beta globin chain glutamine to valine
Extramedullary hematopoisis is seen
Sickle cell disease features
Sickle cells called drepanicytes
Target cells called codocytes
Howen jolly bodies
None marrow expansion
Anisocytosis and poikilocytosis
Vasoocculisive crisis
Alpha -Thalaseemia results in mutation in which chromosome
Ch 16 of 4 genes
Beta thalaseemia mutation in
Ch 11 of 2 genes