Chapter 11 Flashcards
Where does hematopoiesis occur in children?
Marrow of long bones
Where does hematopoiesis occur in adults?
Pelvis, cranium, vertebra, sternum, ribs
Where does extra medullary hematopoiesis occur?
Spleen and liver, lymph nodes, thymus
What is hemolysis?
Ruptured RBCs
What is erythropoiesis?
Red bone marrow
What is a reticulocyte?
Immature RBC
What is polythemia? Q
Increased RBC
What is reduced red blood cells with reduced o2 capacity more common in women?
Anemia
Hemorrhagic Anemia causes what?
Hypoxia
Hypoxia in hemorrhagic anemia results in what?
Increased erythropoietin (EPO)
If hemorrhagic anemia results in a loss of more that_____% of blood what can happen?
Hypovololemic shock
Chronic blood loss in hemorrhagic anemia leads to a depletion of what which can cause what?
Iron stores, IDA
What does hypoxia for 3 days result in?
Increased plasma (hemodilution)
Hypoxia for 7 days results in what?
Increased reticulocytes (reticulocytosis)
What are symptoms of anemia?
Pallor, fatigue, weakness (lassitude) decreased growth, cachexia, ossenous malformations
Hemolytic anemia can lean to what?
Jaundice/gall stones
Anemia can produces tissue hypoxia that can increase erythropoiesis how much?
Up to 8x
What is hemolytic anemia?
Decreased RBC life span (less than 120 days)
Is iron retained in hemolytic anemia?
Yes, it’s recycled
What type of hemolytic anemia is hereditary and has abnormal RBC membranes, enzyme def
Intracorpuscular defect
What hemolytic anemia is acquired, RBC trauma, and antibodies, malaria?
Extra corpuscular defect
Where is the hemolytic anemia most common? Q
Extra vascular hemolysis (in spleen and liver
What form of hemolysis is also acted with physical or biochemical damage, (heart) ?
Intravascular hemolysis
What is an abnormally shaped RBC?
Poikilocyte
What is a irregular shaped RBC from hemolysis ?
Schistocyte
What is a tear drop shaped RBC?
Dacrocyte
What is an autosomal dominant abnormal RBC membrane problem?
Hereditary spherocytosis
What form of anemia is fragile RBC that causes them to shed pieces?
Hereditary spherocytosis
A possible aplastic crisis (parvovirus b19) is associated with what form of anemia ?
Hereditary spherocytosis
What is the treatment for hereditary spherocytosis ?
Splenectomy
What is associated with B-global mutation?
Sickle cell anemia
Is sickle cell anemia autosomal dominant or rescessive?
Recessive
What is heterozygous sickle cell anemia mean?
Sickle cell train, carries, not affected. 8% of African Americans
What is homozygous sickle cell anemia?
Means they have the disease, 1 in every 600 African Americans
What 4 thing are associated with sickle cell anemia?
Acute chest syndrome, stroke, infection/septicemia, fatty changes
Stasis increases what?
Sickling
What are symptoms of sickle cell anemia, ?
Chronic low level joint pain., priapism, gallstones stunted growth
Is you have homozygous sickle cell anemia only 50% live beyond_____?
5th decade
What condition is assopacted with an H shaped vertebrae (Lincoln log vertebra) ?
Sickle cell anemia
What is associated with mutated alpha or beta blobin genes ?
Thalassemia
Is thalassemia auto dominated or recessive ?
Recessive
Beta thalassemia is associated with what chromosome?
11
Alpha thalassemia is associated with what chromosome?
16
What areas are commonly have a lot of thalassemia?
Africa, SE Asia, Mediterranean because of malaria
What condition causes a excess of opposite globin chain?
Thalassemia
What form of beta thalassemia is more severe?
Major (2 alleles)
Asymptomatic thalassemia =
Minor ( 1 allele)
What is associated with beta thalassemia major?
Skeletal deformities
What form of beta thalassemia is microcytic and hypochromic?
Minor
How is beta thalassemia minor diagnosed?
Electrophoresis
What is the treatment for beta thalassemia major?
Blood transfusions and iron chelation
What eventually happens in beta thalassemia major?
Hemochromatosis (iron overload)
What is the lethal side affect of beta thalassemia?
Dilated cardiomyopathy
What type of facies is associated with beta thalassemia major?
Chipmunk facies
Alpha thalassemia is a mutated alpha globin gene that creates an excess of what?
B-globin chains (less damaging that excessive alpha)
Which is more severe alpha or beta thalassemia?
BETA
How many genes need to be altered for alpha thalassemia?
4
Abnormal Hb in alpha thalassemia results in what?
Decreased o2 capacity
If you have 4 deletions in alpha thalassemia what is the outcome?
No o2 capacity, lethal hydrops fetalis
What protects RBCs against hemolysis ?
Glutathione (GSH)
A G6PD results in what and is most common in who:?
Decreased GSH, older RBCs at risk for hemolysis, males
Bite cells and Heinz bodies are associated with what?
G6PD
What are causes of G6PD?
Infections (MC), fava beans (favism) , ADR
How long after exposure does hemolysis in
G6PD?
2-3 days
What are symptoms of G6PD?
Fatigue, splenomegaly, BACK PAIN, dark urine
What are risks for G6PD?>
Males, Africans, areas of malaria
Paroxysmal nocturnal hemoglobinuria (PNH) is associated with what gene and in who?
PIGA, X chromosome
What is associated with increased complement fixation, decreased pH and respiration while sleeping and increase thrombosis?
Paroxysmal nocturnal hemoglobinuria
What is the treatment for PNH?
Antibodies that inhibit the Mac
What disorder is associated with Immue attack on RBCs?
Immunohemolytic anemias
How does one diagnose immunohemolytic anemia?
DIRECT COOMBS anti globulin test
For form of immunohemolytic anemia is spontaneous and idiopathic ?
Endogenous
Exogenous immunohemolytic anemia is caused by what?
Toxic exposure or ADR
Warm antibody immunohemolytic anemia is also acted with what ?
IgG opsonization
Cold antibody immunohemolytic anemia occurs at what temp, and because of what?
Less that 86 degrees, IgM opsonization
What in particular is associated with immunohemolytic anemia?
Raynaud phenomenon
What are causes of traumatic hemolysis?
Prosthetic heart valves “blender effect “
Narrowing of the blood vessels can cause what kind of anemia?
Microangiopathic hemolytic anemia
What cells are associated with traumatic hemolysis ?
Burr cells, helmet cells (schistocytes)
How many people does malaria affect each year and how many die?
500 million, 1 million die.
What is the name of the protozoan that causes malaria?
Plasmodium falciparum
What transmits malaria?
female Anopheles mosquito
What is another name for malaria ?
Black water fever
Merozoite showers happen how often in malaria?
Every 48 hours
What are symptoms of malaria?
Epsodic shaking, chills, fever, splenomegaly, jaunmadce, headache, joint paint
Cerebral malaria is associated with what?
Seizures coma death, lethal in days
Anemias of diminished erythropoiesis are a combination of what to things?
Anemia and reticulocytopenia
What are to nutritional deficiencies associated with anemias of de insisted erythropoiesis?
Iron deficiency anemia, mealoblastic anemias :(folate, b12)
What are the four anemias of diminished erythropoiesis?
Nutritional deficiencies, an exam of chronic disease, aplastic anemia, myelopththisic anmea,
What is the worlds most common nutritional def?
Iron
What is the most common cause of anemia?
IRON DEF
Iron def affects what perfect of developed counties? Developing countries. ?
10, 25-50
Who stores more iron Men or woman?
Men 3.5 to woman 2.5
How is iron transported ?
Transferrin
What is the most common cause of iron def in developing nations?
Low intake
What is the most common cause of iron def in developed countries>?
Chronic blood loss
What 3 things cause iron def?
Poor bioavailability, increased metabolic demand, chronic blood loss
In what way does iron def cause anemia?
Reduced hemoglobin synth
What are symptoms of iron deficiency anemia?
Pica, fingernail spooning, fatigue, decreased immunity, insidious onset
What are to common causes of chronic blood loss?
GI tract, female genital tract
Someone who is deficient in either folate, or vit b12 could have what?
Mealoblastic anemia
Mealoblastic anemia produce what kind of RBCs?
Macrocytic, hyperchromic
Pancytopenia is seen in what condition?
Megaloblastic anemia
What type of disjunction do we not see with folate deficiency anemia?
Neurologic dysfunction
Vit B12 def (pernicious anemia) can effect what?
PNS and spinal cord
What can chronic malabsorption of vit b12 cause ?
Autoimmune gastritis (decrease in intrinsic factor
Vitamin b12 def is most common in what population?
Old
What are features of b12 def?
Fatigue, neuropathy, ataxia
Is neurological recovery likely in someone who has had a B12 def anemia?
No
Vit B12 def anemia puts one at a slight risk for what kind of cancer?
Gastric carcinoma
Anemia of chronic disease is most common seen where?
Hospitalizations
What are 3 things anemia of chronic diseases can cause?
Infections, autoimmunity, cancer
What is associated with suppression of myeloid stem cells?
Aplastic anemia
Pancytopenia is seen in what ?
Aplastic anemia
What attacks the marrow in aplastic anemia ?
T cells
What are the causes of aplastic anemia ?>
1/2 idiopathic, rest myelotoxic agent
80% of aplastic anemia patients respond to what treatment?
Immunosuppressive medication
What is the worst prognosis for aplastic anmea ?
If it is idiopathic
What are features of aplastic anemia?
Thrombocytopenia, graulocytopenia
What is characteristicly absent from aplastic anemia?
Splenomegaly
What type of anemia is normocytic, normochromic, reticulocytopenia?
Aplastic
For form of anemia is associated with extensive marrow infiltration
Myelophthisic anemia
What is the most common cause of myelophthisic anemia?
Mets to bone (breast, lung, prostate)
What are 2 other causes of myelophthisic anemia besides mets to bone?
Granulomatous disease (TB, bridges good syndrome,) and lipid storage disease ( niemann pick type C)
Dacrocytes are seen in what kind of anemia ?
Myelophthisic anemia
