Anemia/ RBC disorders - Exam 3 Flashcards
Low RBC or hemoglobin content
Anemia
What is low hemoglobin in men and in women
<13 men
<12 women
Polycythemia / erythrocytosis
increased RBC
(primary - neoplasm of BM)
(secondary - increased altitude, sleep apnea, cyanotic heart disease)
Anemia occurs due to these 3 things
Low erythropoiesis
Increased RBC loss / Decreased RBC like span
Dilutional anemia
Tissue hypoxia is a symptom of anemia - what is a response that occurs due to this
Cardiovascular-pulmonary compensatory responses
A hematocrit at 30%
<30%
<20 %
asymptomatic
moderate anemia (weakness, headache, tinnitus, vertigo)
Severe anemia (loss of period, libido, GI complaints, heart failure / shock)
Production defect due to heme/ globin synthesis alterations
Microcytic-hypochromic RBC
(Iron def. or malabsorption)
Hypoplastic or hypoproliferative mechanism
Normochromic-normocytic RB
(Leukemia or metastatic cancer)
Defect in DNA synthesis, defect in Vitamin B12, folate or folic acid
Macrocytic (megaloblastic) RBC
Anemias due to reduced or ineffective erythropoiesis (4)
Iron deficiency anemia
Anemia of chronic disease
Vitamin B12 deficiency / pernicious anemia
Folate (folic acid) deficiency anemia
Most common cause of anemia
Iron deficiency anemia
Second most common cause of anemia - most common type in hospitalized patients
Anemia of chronic disease
Blood loss
Poor iron intake
poor iron absorption
Iron deficiency anemia
Koilonychia
Pica
Dysphagia
Atrophic glossitis
Glossodynia
Iron deficiency anemia
Anemia of chronic Disease
Iron deficiency anemia + dysphagia + esophageal webs
Plummer-Vinson syndrome
Results in microcytic hypochromic anemia
Iron deficiency anemia
Associated with underlying diseases that have sustained inflammation
Anemia of chronic disease
Anemia of chronic disease :
Inflammatory cytokines release __ that blocks iron channels so iron is stuck in macrophages =
hepcidin
low erythropoietin production
Normocytic, normochromic, mild to moderate anemia
anemia of chronic disease
Decreased reticulocyte count
anemia of chronic disease
Autoimmune disease and H. pylori infection
Vitamin B12 deficiency / pernicious anemia
Hereditary in N. european ancestry : Scandinavia
Vitamin B12 deficiency / pernicious anemia
Autoimmune-mediated destruction of gastric mucosa that decreases production of intrinsic factor
Vitamin B12 deficiency / pernicious anemia
(intrinsic factor helps absorb B12)
Parietal cell antibodies present (70%)
Intrinsic factor antibodies present (50%)
Vitamin B12 deficiency / pernicious anemia
Prevalence in black women
Vitamin B12 deficiency / pernicious anemia
Atrophic gastritis
Vitamin B12 deficiency / pernicious anemia
(inflammation of stomach lining)
Folate (folic acid) deficiency Anemia
Achlorhydria
Vitamin B12 deficiency / pernicious anemia
(stomach doesn’t produce hydrochloric acid)
Folate (folic acid) deficiency Anemia
Atrophy of gastric glands histologically (achlorhydria)
GI symptoms
Vitamin B12 deficiency / pernicious anemia
Folate (folic acid) deficiency Anemia
Mild jaundice
Vitamin B12 deficiency / pernicious anemia
Folate (folic acid) deficiency Anemia
Complex neurologic syndrome secondary to methionine deficiency
(Decreased proprioception, bad balance/ hard to walk, impaired memory)
Vitamin B12 deficiency / pernicious anemia only!
Glossitis (as well as lips, buccal mucosa, other mucosal sites)
Vitamin B12 deficiency / pernicious anemia
Oral mucosal erythema and atrophy (redness)
Vitamin B12 deficiency / pernicious anemia
Do NOT use nitrous oxide sedation
Vitamin B12 deficiency / pernicious anemia
Megaloblastic macrocytic anemia
Vitamin B12 deficiency / pernicious anemia
(macrocytosis is absent in >30% of patients)
Can have non-hematologic signs for months before they develop a detectable anemia (anemia is absent in >20% of patients)
Vitamin B12 deficiency / pernicious anemia
(Vit. B12 def does not = anemia)
Comes from green veggies and some fruits
Folic acid
If cellular stores of folate fall –> __ is blocked and this leads to __
DNA replication
megaloblastic anemia
Does Folate (folic acid) deficiency Anemia involve neurologic abnormalities
NO
Megaloblastic, macrocytic anemia
Folate (folic acid) deficiency Anemia
CBC and smear are indistinguishable from Vit. B12 deficiency
Folate (folic acid) deficiency Anemia
How is Folate (folic acid) deficiency Anemia distinguished from Vit. B12 deficiency
Measuring serum and red cell folate and Vit. B12 levels
CBC shows increased platelet count
Iron deficiency anemia
CBC shows increased RDW
Iron deficiency anemia
CBC shows decreased
MCV
MCHC
Iron deficiency anemia
Blood smear shows microcytic hypochromic RBCs
Iron deficiency anemia
Blood smear shows poikilocytosis and anisocytosis (abnormal variation in size and abnormal variation in shape)
Iron deficiency anemia
Vitamin B12 deficiency / pernicious anemia
Decreased Ferritin and serum iron
Iron deficiency anemia
(ferritin = iron storage)
Increased TIBIC
Iron deficiency anemia
(total iron binding capacity)
CBC shows Low reticulocyte count
Anemia of chronic disease
Blood smear shows normocytic normochromic OR microcytic hypochromic RBC
Anemia of chronic disease
CBC shows increased MCV and MCH
Vitamin B12 deficiency / pernicious anemia
Folate (folic acid) deficiency anemia
CBC shows a normal MCHC
Vitamin B12 deficiency / pernicious anemia
Folate (folic acid) deficiency anemia
CBC shows a decreased platelet count
Vitamin B12 deficiency / pernicious anemia
Folate (folic acid) deficiency anemia
CBC shows a low WBC count
Vitamin B12 deficiency / pernicious anemia
Folate (folic acid) deficiency anemia
Blood smear shows LARGE, macroovalocyte RBCs
Vitamin B12 deficiency / pernicious anemia
Folate (folic acid) deficiency anemia
Blood smear shows hyper-segmented neutrophils
Vitamin B12 deficiency / pernicious anemia
Folate (folic acid) deficiency anemia
Low serum vitamin B12
Vitamin B12 deficiency / pernicious anemia
Increased methylmalonic acid and homocysteine
Vitamin B12 deficiency / pernicious anemia
Low serum and red cell folate
Folate (folic acid) deficiency anemia
Increased homocysteine
Folate (folic acid) deficiency anemia
Normal methymalonic acid
Folate (folic acid) deficiency anemia
Anemias due to hemolysis (RBC destruction of reduced lifespan) can be congenital or acquired
Congenital = intrinsic RBC defect
Acquired = abnormal marrow or systemic disease (RBC itself is perfect)
Congenital hemolytic anemias can be a congenital defect it __, __, or in __
hemoglobin structure
RBC metabolism
hemoglobin synthesis
Hemolytic anemia caused by congenital, hereditary abnormality in the hemoglobin molecule
Hemoglobinopathies
2 anemias due to congenital hemolytic anemias
Sickle cell anemia
Thalassemia / Beta-thalassemia
Most common hereditary hemolytic anemia
Sickle cell anemia
Production of an abnormal hemoglobin molecule (hemoglobin S HbS)
Sickle cell anemia
Hemoglobin S HbS is due to a
single AA substitution of valine for glutamic acid
HbS is unstable and polymerizes when exposed to hypoxemia and acidosis =
RBDs deform into a sickle shape
Hemolysis in SCD is mainly __
extracellular (spleen)
Disease vs trait of SCD
Disease = 85-90% of normal Hbg is replaced
Trait = 30-45% per RBC = asymptomatic - carrier
Affects persons of african descent
Sickle cell anemia
Painful vaso-occlusive crises
Sickle cell anemia
Vaso-occlusive crises can be predicted by
hypoxia and dehydration
Splenic sequestration
Sickle cell anemia
Dactylitis
Sickle cell anemia
Leading cause of death with Sickle cell anemia
acute chest syndrome
Leading cause of death in children with Sickle cell anemia
Strep. pneumoniae infection
Renal failure (glomerulopathy)
Sickle cell anemia
Osteomyelitis
Sickle cell anemia
Jaundice in oral mucosal tissue
Sickle cell anemia
Erythropoietic activity observable on dental x-rays within the bone
Sickle cell anemia
Delayed dental eruption
Sickle cell anemia
Hypoplasia
Sickle cell anemia
Ischemic necrosis within the mandible and peripheral neuropathy from vaso-occlusive events
Sickle cell anemia
Occurs in patients from the mediterranean region
Thalassemia / beta-thalassemia
Caused by more than 200 point mutation that lead to defects in transcription, splicing, or translation of beta-globin mRNA
Thalassemia / beta-thalassemia
Heterozygous form of Thalassemia / beta-thalassemia
Beta-thalassemia minor
Cooleys anemia or mediterranean fever
Beta-thalassemia major (homozygous)
Blood transfusion dependence
Beta-thalassemia major
Severe anemia
Thalassemia / beta-thalassemia
Growth retardation
Beta-thalassemia major (homozygous)
Skeletal deformities
Beta-thalassemia major (homozygous)
Splenomegaly, hepatomegaly, and lymphadenopathy - gallstone and or cirrhosis
Beta-thalassemia major (homozygous)
Enlargement of the mandible and maxilla
Thalassemia / beta-thalassemia
Chipmunk faces - frontal bossing, maxillary hytrophy, depression of nasal bridge, class II malocclusion of teeth
Thalassemia / beta-thalassemia
CBC shows moderate to severe anemia with decreased RBC count, HCT and Hgb
Sickle cell disease
Blood smear shows reticulocytes, nucleated RBCs and target or bullseye cells
sickle cell disease
Major increase of lactic dehydrogenase (LDH)
sickle cell disease
CBC shows sever anemia, decreased MCV and MCHC
Thalassemia / beta-thalassemia major
CBC shows increased RDW
Thalassemia / beta-thalassemia major
Blood smear show deformed RBCs, microcytic and hypochromic
Thalassemia / beta-thalassemia major
Blood smear shows nucleated RBCs and target cells
Thalassemia / beta-thalassemia major
Blood smear shows anisocytosis, poikilocytosis, polychromatophilia
Thalassemia / beta-thalassemia major
Elevated indirect bilirubin and lactate dehyrdogenase (LDH)
Thalassemia / beta-thalassemia major
Decrease haptoglobin
Thalassemia / beta-thalassemia major