RBC Disorders Flashcards
(35 cards)
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
Thalaseemia main featurrs
Extravascular hemolysis
Anisocytosis
Microcytic, hypochromic
Poikilocytosis
Target cells
Beta thalaseemia major called as cooley anemia which has crewcut appearance on x ray
Paraxysomal nocturnal hemoglobinuria identification
It’s immunohemolytic anemia
It’s identifird by negative expression of CD 55 and 59
Mutation in phosphotidylinositol glycan complementation group A gene
PNH which type of hemolysis
Both intra and extravascular by complement fixation and spleen sequestration
Large bizzare multinuckeated marcroohages seen in
Megaloblastic anemia
Features of megaloblastic anemia
Macrocytic, normochromic
Anisocytosis, poikilocytosis
Low reticulocyte count
Hypersegmented neutrophils
Pencil cells and plummer vinson syndrome triad is seen in
Iron deficiency anemia
Microcutic, hypochromic anemia
Iron deficiency anemia
Serum ferritin is high andblow in which disorders
Anemia of chronic inflammation and iron deficiency anemia respectively
Aplastic anemia
Chronic primary hematopoietic failure and pancytopenia