Blood Disorders - Final Exam Flashcards
Fluid of blood
Plasma
Cells of blood
WBC (leukocytes)
RBC (erythrocytes)
Platelets (thrombocytes)
Sites of hematopoiesis
Adults: calvarium, vertebrae, ribs, sternum, pelvis
Children: most bones
Fetus: most ones
Embryo: yolk sac, liver cords
Centrifuging of blood
Plasma at top
Buffy coat (WBC and platelets) in middle
RBC at bottom
Erythrocytes
For transportation of oxygen from lungs into peripheral tissues
Live in circulation for 120 days - broken down in spleen
Hemoglobin
Complex molecules consisting of four heme groups (Fe in middle) and four globins
97% Hemoglobin A
Fetal hemoglobin
Alpha chains and gamma chains
In children, traces in adults
Bilirubin
Heme is broken down and Fe is removed
Hemoglobin synthesis
Requires iron, Vit B12, Vit B6, folic acid
Erythropoietin
Kidney sensing O2 is low and secrete hormone to tell bone marrow to make more RBC
Thrombocytes
Platelets
Essential clotting factors
Malignant transformation of hematopoietic cells (2)
Solid tumors or leukemia
Anemia (3)
Reduction of Hb in blood - below normal levels
Less than reference range established in given population
Associated with appearance of abnormal Hb, reduction in RBC, structural abnormalities of RBC
Causes of anemia (3)
- Decreased hematopoiesis
- Abnormal hematopoiesis
- Increased loss or destruction of RBC
Decreased hematopoiesis (2)
- Bone marrow failure (aplastic anemia, myelophthisic anemia)
- Deficiencies of nutrients (vit B12, folic acid, protein)
Abnormal hematopoiesis
Usually consequence of genetic abnormalities
Sickle cell anemia
Increased loss and destruction of RBC (4)
- Bleeding
- Intrasplenic sequestration
- Immune hemolysis
- Infections
Aplastic anemia
symptoms: 5
Idiopathic, secondary
Bone marrow depletion of hematopoietic cells (consists only of fibroblasts, fat cells, scattered lymphocytes)
Anemia, leukopenia, thrombocytopenis
Uncontrollable infections, bleeding tendency, chronic fatigue, sleepiness, weakness
Iron deficiency anemia
(histology: 2
causes: 3)
Most common form
Hypochromic (pale), microcytic (small) RBC
Increased loss of iron, inadequate iron intake or absorption, increased iron requirements
Megablastic anemia
histology: 3
Caused by deficiency of vit B12 or folic acid
Large RBC (macrocytic anemia)
Bone marrow is hypercellular with numerous megaloblasts (abnormal RBC precursors)
Hypersegmentation of neutrophils
Vit B12 deficiency (3)
- Pernicious anemia
- Lack of gastric intrinsic factor
- Atrophic gastritis
Folic acid deficiency
Inadequate intake
Due to diet or malabsorption
Hemolytic anemia (4)
- Increased RBC destruction
- Intracorpuscular or extracorpuscular defects
- Compensatory erythroid hyperplasia of BM
- Hyperbilirubinemia (jaundice)
Intracorpuscular defects (3)
- Structural abnormalities
- Sickle cell anemia, thalassemia, hereditary spherocytosis
- Infection
Extracorpuscular defects
- Antibodies, infectious agents, mechanical factors
- Autoimmune hemolytic anemia, hemolytic disease of newborn, transfusion reactions, hemolytic anemia due to cardiac valve prosthesis, disseminated intravascular coagulation
Sickle cell anemia
Substitution of glutamic acid by valine Synthesis of abnormal beta chain of globin Most prevalent among African populations Multiple infarctions in various organs Jaundice
Sickle cell anemia - low oxygen
Hemoglobin S polymerizes and forms chain that deforms cells
Reversible if O2 is back up, quickly
All symptoms occur due to clotting
Long term complications of sickle cell anemia (3)
- Delayed intellectual development, neurologic deficits
Largely avoided due to medical surveillance and appropriate management or crises - Cardiopulmonary insufficiency
- Recurrent infections
Thalassemia
(histology: 1
symptoms: 5
developmental: 2)
Genetic defect in synthesis of HbA, do not produce enough
Reduced rate of globin chain synthesis
Beta or alpha chain affected
Minor (heterozygotes) or major (homozygotes, very serious)
Hypochromic (pale RBC) anemia
Splengomegali, hemosiderosis, hepatomegaly
Bone marrow: compensatory hyperplasia
Calvarium (crew cut) hair on radiographic study - a lot of hematopoiesis in skull and changes the way it looks
Jaundice
Chronic anemia that slows growth of children
Impaired normal intellectual development
Cardiorespiratory insufficiency