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robertojr's version from 2015-11-25 17:32

RBC disorders

Question Answer
Are red blood cell deficiencies common?YES (that's why it's important for us to know about them)
What are 5 potential dental complications for patients with RBC deficiencies?1. Susceptibility to INFECTIONS; 2. mucosal epithelial ATROPHY; 3. BLEEDING problems
What two organs functions are especially affected by RBC deficiencies (listed in complication)Liver & Spleen
Do RBC disorders manifest in the head and neck?Yes
General umbrella term for RBC disorders involving deficienciesAnemia
"lack of blood" (latin); term means something deficient in bloodAnemia
What is the (first) ORIGIN of Erythrocytes?Pluripotent Hematopoietic Stem Cells (Or Pluripotent Stem Cells) in Bone Marrow
Pluripotent Hematopoietic Stem Cells can differentiate directly into what two types of cells?Common Myeloid SC (Stem Cell) & Common Lymphoid SC
T/F: Erythrocytes develop from Common Lymphoid Stem CellsFALSE (Lymphocytes not RBC's come from Lymphoid Stem Cells)
T/F: Erythrocytes develop from Common Myeloid Stem CellsTRUE (and these develop from Pluripotent Hematopoietic SC's)
decreased oxygen carrying capacity of the peripheral blood is a type of ____Anemia
according to WHO, severe anemia = anyone with Hb < ___ g/dL7.0 g/dL
memorize

 

Question Answer
3 Compensatory mechanisms for Anemia (all increases)Increase in Plasma Volume, Cardiac Output, & Respiratory Rate
3 Main organs affected by long term anemiaLiver, Myocardium, & Kidneys (cells involved in lipid metabolism)
accumulation of fatty vacuoles in the cell cytoplasm in response to hypoxia is a ____ (reversible/irreversible) consequence of long term anemiaReversible
T/F: There is a reversible accumulation of fatty vacuoles in the cell cytoplasm in response to long term anemiaTrue
Around the world, the most common mechanistic cause of Anemia is ___ ____ of RBC'sDecreased Production (often malnutrition, iron deficiency)
RBC's whose pale area makes up 1/3rd diameter of cell (under microscope) would be referred to as ____Normochromic
RBC's whose pale area makes up 2/3rd's diameter of cell (under microscope) would be referred to as ____Hypochromic
Normal RBC cell size is _________ to ___ microns6-8 microns (7 is safe avg)
Inadequate intake of iron results in insufficient hemoglobin synthesis and ____ (color) and ____ (size) RBC's.Hypochromic; Microcytic
Inadequate intake of ___ results in insufficient hemoglobin synthesis and hypochromic and microcytic RBC.Iron
Caused by production of inflammatory cytokines, which cause iron to be sequestered in macrophages, and deregulate iron absorption, usually resulting in ___ (color) and ___(size) RBC's.Normo-chromic; Normocytic
Which Anemia is Caused by deficiencies of folate or vitamin B12Megaloblastic Anemia
Which anemia results in enlarged abnormal hematopoietic precursors in the bone marrow, ineffective hematopoiesis, and (in most cases) pancytopeniaMegaloblastic Anemia (Folate or B12 deficiency)
Which anemia Caused by bone marrow failure (hypocellularity)Aplastic Anemia
inherited defects in DNA repair and the enzyme telomerase can lead to which Anemia?Aplastic Anemia (bone marrow failure; hypocellularity)
exposures to toxins and radiation can lead to which Anemia?Aplastic Anemia (bone marrow failure; hypocellularity)
Which anemia is caused by replacement of the bone marrow by infiltrative processes such as metastatic cancer and granulomatous diseaseMyelophthisic Anemia
Which anemia release of early erythroid and granulocytic precursors (leukoerythroblastosis) and the appearance of tear-drop red cells in the peripheral bloodMyelopthisic Anemia
leukoerythroblastosis is a sign associated with which anemia?Myelopthisic Anemia (metastatic infiltration of bone marrow)
term for release of early erythroid and granulocytic precursors into bloodLeuko-erythro-blastosis
3 non-specific symptoms of anemiaPallor (pale), Tired, & Weak
Hyperbilirubinemia, jaundice & pigment gallstones are specific to what type of Anemia?Hemolytic
In Anemia of Chronic Disease, what is problematic about RBC's?Low Quantity (cells are NORMAL but their production is down regulated)
In Iron Deficiency Anemia, what is problematic about RBC's?Hypochromic & Microcytic (Low Hemoglobin content & Small)
In Iron Deficiency Anemia you don't make enough ____ and RBC's appear ____ & ____ under microscopeHemoglobin; Hypochromic (low hemoglobin) & Microcytic (small size)
In Anemia of Chronic Disease (cytokine causation) you don't make enough ____ and RBC's appear ____ under microscopeRBC's; Normal (but low quantity)
an example of a Microcytic AnemiaMegaloblastic Anemia (RBC's are too big)
These 2 essential nutrients are important for DNA synthesis, and their deficiency results in large, immature circulating RBC'sB12 & Folate
Liver product that regulates Iron absorptionHepcidin
Hepcidin is produced by the ____ and regulates ____ absorptionLiver; Iron
Approximately ___ mg of Iron is absorbed daily... or _________% of dietary intake1 mg; 10%
Normally, most ____ is lost through shedding surface cellsIron
T/F: Inorganic iron in non-animal food is absorbed inefficientlyTrue
T/F: Inorganic iron in non-animal food is absorbed efficientlyFalse
molecule responsible for transport of ironTransferrin
2 molecules responsible for storing ironFerritin & Hemosiderin
2 conditions that can lead to impaired Iron absorption in DuodenumChronic Diarrhea & Gastrectomy
3 conditions that increase demand for ironGrowth, Pregnancy, (physiologic regular) Blood loss
northern European women 30-50 yrs with anemia, glossitis, and dysphagia have what Syndrome and are at greater risk for what serious condition?Plummer-Vinson syndrome; Squamos Cell Carcinoma
cobalamin is akavit B12
vit B12 is akacobalamin
term meaning reduction in the number of red and white blood cells, as well as plateletsPancytopenia
blood count cutoff for Aplastic Anemia <___PMN's / microL, < _________ platelets / microL, & < ___ reticulocytes / microL500; 20,000; 10,000
Sickle cell anemia has similar features to Thalassemia... but have the added complication of what?Ischemic Necrosis (stressed conditions lead to sickling of cells which can form "stacks" or polymerize to block blood vessels leading to infarction)
What anemias are common in people of African descent?Sickle cell (8% heterozygous in US) & Thalassemia
What anemia is common in Mediterranean, African, Indian & SE Asian populations?Thalassemia
Extramedullary Hematopoiesis is associated with what condition?Thalassemia
Adult hemoglobin (HbA) is a tetramer composed of two ___ chains and two ___ chainsAlpha; Beta
Alpha Hb chains are encoded by ____(#) alpha-globin gene(s)two
Beta Hb chains are encoded by ___ (#) beta-globin gene(s)one
Most Beta Thalassemias are _____ base mutationssingle (deletions are very rare)
What specific type of anemia is associated with NO Beta-Globin production or severely diminishedThalassemia MAJOR
How (therapeutically) is Thalassemia Major managed?Blood Transfusions
Less Hemoglobin and precipitation of free-alpha globin chains can result from SEVEREBeta Thalassemia (Major)
Globin chains are not associated with ____ Thalassemia (Alpha/Beta)Alpha (this is b/c Beta globins do not polymerize in the same way that Alpha does when unpaired)
Hydrops fetalis is associated with what type of Thalassemia?Alpha Thalassemia (only when ALL alpha globin genes are defective)
Hemoglobin H disease is associated with what type of Thalassemia?Alpha Thalassemia (3/4 alpha globin genes defective)
Two tests to differentiate Thalassemia from Iron deficiency?Hb Electrophoresis & Genetic testing
if both parents have b Thalassemia trait, the child has a ___% chance of thalassemia major25%
Result of treating Thalassemia with oral Fe supplementsIron Overload
Sickle Cell patients that undergo hypoxia, infection, hypothermia, or dehydration can enter what state?Sickle Cell Crisis
A result of what Anemia can lead to Cor Pulmonale?Sickle Cell (following crisis)
Acute chest syndrome is associated with acute complications of what anemia?Sickle Cell
Hereditary Spherocytosis is treated with what surgical procedure?Splenectomy
Circular rather than bi-concave RBC's is what condition?Spherocytosis
2 types of cancer associated with Hemolytic Anemia symptoms and elevated levels of ErythropoietinRenal Cell Carcinoma & Hepatocellular Carcinoma
3 types of cancer that can produce excess RBC's; 2 secondary, 1 primaryRenal Cell Carcinoma & Hepatocellular Carcinoma (2nd); Polycythemia Vera (1)
Cause of "Relative Polycythemia"Dehydration
X-linked condition where older RBC are susceptible to oxidative stress; hemolysis precipitated by infections, and some drugsGlucose-6-dehydrogenase deficiency
What common precursor do Neutrophils, Platelets, & Erythrocytes have in common (most recent)Common Myeloid Progenitor (all 3 come from this progenitor cell)
The ____ of hemoglobin can affect oxygen carrying capacity by affecting cell morphology and function of erythrocytesQUALITY
The ____ of hemoglobin can affect oxygen carrying capacity of hemoglobin and when there's a problem with this characteristic it shows via Hypochromic RBC'sQUANTITY
What are the underlying problems involved with iron deficiency/chronic blood loss?Insufficient Iron/Heme
Altered/defective hemoglobin or RBC's would lead to what mechanistic classification of anemias?Hemolytic (RBC's lyse or selected for destruction prematurely)
Malnutrition, Chronic bleeding, & Plummer's Vinson are what (mechanistic) types of anemias?Iron/Heme deficiency
G6PD deficiency would lead to what type (mechanistic) of anemia?Hemolytic
Gallstones are associated with what mechanistic class of anemias?Hemolytic
Anisocytosis and Poikilocytosis are associated with what mechanistic class of anemias?Hemolytic (congenital cause)
Jaundice has yellowing of skin but not sclera of eyes, that is associated with HypercarotinemiaFalse (vice versa)
Hypercaritenemia has yellowing of skin but not sclera of eyes, that is associated with JaundiceTrue
Elevated Erythropoietin is associated with what mechanistic class of anemias?Hemolytic
Iron overload or excess of HemosiderinHemosiderosis (assoc w/ Hemolytic anemias)
Pallor, fatigue, light-headedness, dyspnea, & tachycardia are associated with which mechanistic types of anemia?All 3 (but pallor is paired with jaundice in hemolytic)
serum Unconjugated bilirubin, methemoglobin, hemosiderin, & urobilin are elevated in what mechanistic type of anemia?Hemolytic
lacking (one of) what 3 things in serum would cause Decreased Production Anemia?Vitamins (B12 or Folate... DNA synthesis), Globin Peptides, & Erythropoietin
Normal Iron stores would be seen in ____ ____ Anemia, while excessive iron stores would be seen in _____ anemiaDecreased Production; Hemolytic
Plummer-Vinson syndrome has an increased risk for what type of neoplasia?Squamos Cell Carcinoma (5-50% greater risk)
autoimmune destruction of the gastric parietal and zymogenic cells inhibits intrinsic factor production --> B12 deficiencyPernicious Anemia
Pernicious Anemia has an _____ cause, affecting gastric parietal cellsAutoimmune
What essential nutrient is synthesized by microorganisms?B12 (cobalamin)
2 examples of Hemoglobinopathies that provide resistance to MalariaSickle Cell & Thalassemia
“hair-on-end” appearance of calvaria (radiograph) is associated with what anemias?Thalassemia & Sickle Cell (the malaria resistors)
Ischemic Necrosis is especially associated with what type of Anemia?Sickle Cell (ability to infarct vessels if patient enters sickle cell crisis)
Aplastic anemia is failure of the precursor to what 3 cell types?RBC's, Platelets, & Leukocytes (granulocytes)
2 examples of Intrinsic Hemolytic AnemiasThalassemia & Sickle Cell anemia
Hemolytic anemias that are due to problems with the quality of the RBC are referred to as _____ Hemolytic anemiasINTRINSIC
Hemolytic anemias that are due to problems "outside the RBC" are referred to as _____ Hemolytic anemiasEXTRINSIC (e.g. Autoimmune Hemolytic Anemia & Disseminated Intravascular Coagulation)
Accumulation of products of hemoglobin catabolism would be associated with what mechanistic class of anemias?Hemolytic
Marked increase in erythropoiesis would be associated with what mechanistic class of anemias?Hemolytic
term meaning enlargement of the spleenSplenomegaly
Splenomegaly associated with what mechanistic class of anemias?Hemolytic
Hemoglobin S is due to mutation in _______beta-globin (Sickle-cell anemia)
2 Hemoglobin types that can interfere with sickling of Hemoglobin SHbA & Fetal Hb
Is a patient with 40% HbS and 60% HbA symptomatic?No, (heterozygous and asymptomatic)
___% of African Americans in the US are heterozygous for Sickle Cell Anemia8%
How would CBC differ for Iron deficiency anemia?Less MCV (relating to smaller RBC's) aka less Hematocrit
memorize

Describe key mechanisms of common anemias

Question Answer
Due to a defect in the hematopoietic stem cellAplastic Anemia
Due to defective synthesis of hemoglobin thalassemia
Due to defective DNA synthesisMegaloblastic Anemia (B12 or Folate deficiency)
Autoimmune damage to Gastric cells leading to lack of intrinsic factorPernicious Anemia (causing Macrocytic/Megaloblastic anemia)
memorize