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52 Cards in this Set

  • Front
  • Back
Hemoglobin; FUNCTION
Transport oxygen from the lungs to the tissues and CO2 from the tissues back to the lungs
The Bohr Effect
Decreased 02 affinity in an acid environment, increased affinity in basic environment
Tissues have ___ ph compared to lungs
lower
In lungs O2 taken up by Hb as environment is more ___
alkaline
beta globin gene cluster chromosome -
11
alpha globin gene cluster chromosome -
16
The combination of two alpha chains and two gamma chains form "fetal" hemoglobin, termed "
hemoglobin F
The combination of two alpha chains and two beta chains form "adult" hemoglobin, also called
hemoglobin A
The expression of the alpha and non-alpha genes is closely balanced by an unknown mechanism. Balanced gene expression is required for normal red cell function. Disruption of the balance produces a disorder called .
thalassemia
Term used to describe cell death
SENESCENCE

In the steady state the rate of red cell production per day is equivalent to the rate of red cell senescence
Complaints
Fatigue
Shortness of breath
Chest pain
Forgetfulness
Apathy

Physical Exam
Pale
Ill appearing
Icteric – yellow sclerae
Tachycardic (acute)
Pulmonary crackles (heart failure?)
Splenomegaly
Stomatitis, chelitis
Nail changes
The Anemic patient
A measure of the average size of the red blood cells
MCV
Measures the variation in red blood cell size present in a sample of blood
Red cell distribution width

When the cells are uniform in size the RDW is normal
When the cells exhibit a lot of variation in their size the RDW is high
means that the red blood cells have less color than normal when examined under a microscope or to be more scientific..
Lack of complete saturation of the erythrocyte stroma with hemoglobin, as judged by pallor of the unstained or stained erythrocytes when examined microscopically.
Hypochromia
The average normal North American diet contains ___ mg of iron. Most is present in meat and green vegetables.
Approximately 1.0 mg is absorbed each day and about an equal amount is lost in the feces and sweat.
15-20
a protein present in the blood essential in the transport of iron from the intestine into the bloodstream, making it available to the normoblasts in the bone marrow.
When not bound to iron, it is known as "_____"
apo-transferrin
a protein that stores iron and releases it in a controlled fashion

a "buffer" against iron deficiency

when it is combined with iron is called
Ferritin
What Type of Surgery Would Predispose A Patient To Iron Deficiency?
Bariatric surgery Duodenum surgery
What Medications Would Impair Iron Absorption?
Proton pump inhibitors Antacids
Iron is stored as either
ferritin or hemosiderin

Ferritin is water soluble and found mainly in the bone marrow and liver. It is preferentially used before hemosiderin
Most common anemia in the world
Iron Deficiency Anemia
Autosomal recessive
Most common genetic disorders in Northern Europeans
10% of the population are heterozygotes
0.5% homozygous (major problem)
Hereditary Hemochromatosis
Weakness,fatigue and chronic abdominal pain
Abnormal liver function with eventual cirrhosis
Congestive heart failure
Arthritis (metacarpal joints)
Diabetes
Hyperpigmentation “the bronzed diabetic”
Hereditary Hemochromatosis
___ is a must for absorption, as it releases B12 from protein *this is highly clinically significant
Acid secretion
recognizes UTP as a mismatch and excises it from DNA strand
No TTP available to replace UTP
DNA becomes fragmented leading to arrest of DNA synthesis at various stages
Uracil glycosylase
why is folate and B12 important
bc it is important in oncology - without them, we will have fragmented DNA!
Defined as defect in DNA synthesis with a lesser effect on RNA and protein synthesis
Leads to unbalanced cell growth and impaired cell division
MEGALOBLASTOSIS
Bone marrow is hypercellular with a erythroid predominance
MEGALOBLASTOSIS
Pathognomonic of
megaloblastic anemias
Hypersegmented Neutrophils

Normal neutrophils contain three or four nuclear lobes,
Hypersegmented
neutrophils contain five or more
Anemia
Pallor
Lemon yellow tint to skin
Glossitis (SHINY PAINFUL TONGUE)
Neurologic abnormalities- loss of position sense, gait disturbances, weakness, spasticity
MEGALOBLASTOSIS
Lesions can be seen throughout the length of the spinal cord and white matter of the brain

Incoordination
Difficulty walking
Sensory disturbances
Diminished position and vibration sense
Weakness
Spasticity
B12 Deficiency
Making the Diagnosis of B12 and or Folate Deficiency
Clinical suspicion
Measure serum B12 and folate levels prior to feeding patient
One meal can normalize serum folate levels in a deficient patient
Red blood cell folate will be low in folate deficiency
Red blood cell folate will be normal in B12
T cell infiltrate gastric mucosa leads to Atrophy of gastric mucosa leading to absence of intrinsic factor and HCL acid secretion
Pernicious Anemia
The Schilling’s Test
1.0 mcg of radioactively labeled B12 orally and 1000 mcg B12 IM
Determine radioactivity excretion in urine
If low, oral B12 not properly absorbed
Give Intrinsic factor, if corrects then PA
Part 2 give 7-10 d antibiotics, if corrects then bacterial overgrowth
Part 3 give pancreatic enzymes, if corrects then pancreatic dysfunction (insufficiency)
Splenectomy as abnormal cells are destroyed in the spleen
Relapses due to accessory spleen
Life threatening infections can occur in splenectomized patients
Vaccines crucial
Treatment of HS
Most common type of AIHA


Autoantibodies attach to the RBC surface
Can occur w/o underlying cause but may also be first presentation of lymphoma, lupus and CLL, also infections such as TB and CMV, chronic inflammatory disorders such as Crohn’s disease
IgG
Used to determine if antibodies are present
The Coombs test

Direct Coombs measures antibodies on the RBC
Indirect measures antibodies in the serum (used for blood typing for transfusion)
tx of IgG AIHA
Corticosteroids
Gamma globulin
Splenectomy
Immunosuppressive agents
Transfusions may be difficult because of presence of antibodies and cross reactivity with donor red blood cells
a rare disorder of the coagulation system, causing extensive microscopic blood clots to form in the capillaries

Red blood cells passing the microscopic clots are subjected to shear stress which damages their membranes, leading to intravascular hemolysis.
TTP

Thrombotic thrombocytopenic purpura (TTP
Classically, the ("pentad") is indicative of TTP
**** know the pentad!
Fluctuating neurological symptoms, such as bizarre behavior, altered mental status, stroke or headaches
Kidney failure
Fever
Thrombocytopenia, leading to bruising or purpura
Hemolytic anemia
symptoms:
Fluctuating neurological symptoms, such as bizarre behavior, altered mental status, stroke or headaches
Kidney failure
Fever
Thrombocytopenia, leading to bruising or purpura
Hemolytic anemia
TTP!!! ****
tx of TTP
Early and aggressive plasmapheresis
Steroids
Rituxan
prognosis of TTP
The mortality rate is approximately 95% for untreated cases ****************************************
(80-90%) for patients with idiopathic TTP diagnosed and treated early
Secondary TTP still has a dismal prognosis, with mortality rates despite treatment being reported as 59% to 100%.
Mutation of B (beta) globin gene
Sickle Cell Anemia
Two major physiologic processes of sickle cell anemia
Two major physiologic processes,
shortened red cell survival and
vasoocclusion, account for most of the clinical manifestations of sickle cell disease.
Results from rigid, sickled red cells leading to the obstruction of blood vessels .
Vasoocclusive
in a hemolytic crisis why does the pt look yellow?
The resulting increase in jaundice is associated with a falling hemoglobin level and an elevated reticulocyte count.
Doesn't cure sickle cell disease, but it can reduce the number of painful events and other complications and may prolong life
Increases fetal hemoglobin(HbF) production and raises the total hemoglobin concentration
Hydroxyurea
stem cell disorder, characterized by increased progenitor cell sensitivity to growth-promoting cytokines and is strongly associated with an activating somatic mutation in the JAK2 tyrosine kinase.
Polycythemia Vera
Thrombosis and Bleeding are the major causes of morbidity and mortality from what
polycythemia vera
talk about creation of RBCs
kidneys sense how much oxygen is in the blood... produces erythropoetin... sends it to BM which stimulated production of RBCs
if more iron is required what happens to the amount of apoferritin manufactured in eat mucosal cell
decreased apoferritein manufactured