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AExam 3 Part 1
Alteration in erythrocyte, leukocyte, lymphoid, and hemostatic function, Alterations of hematologic function in children
58
Biology
Graduate
11/15/2012

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Term
Erythrocyte (RBC)
Definition
Most abundant cell in blood.
Biconcave shape allows narrowing for easy movement through capillaries.
Life span about 120d
Mature RBC's have no nucleus or organells.
Term
Reticulocyte
Definition
Immature RBC
About 1% total RBC count
# of reticulocytes in plasma is a good indicator of RBC production.
Term
Hemoglobin (Hg)
Definition
O2 carrying protein of RBC. Picks up O2 in lungs and deposits in tissues.
Term
Koilonychias
Definition
fingernails that are brittle and spoon shaped
Term
leukocytosis
Definition
an increase in the number of leukocytes in blood
Term
megaloblasts
Definition
unusually large stem cells in bone marrow
Term
leukopenia
Definition
a decrease in leukocytes in blood
Term
poikilocytosis
Definition
red blood cells in various shapes
Term
Macrocytic-normochromic anemia
Definition
- Large abnormally shaped erythrocytes but normal hemoglobin concentration.
Lower #RBC's b/c B12 or Folate deficiency causes DNA synthesis defect which makes RBC's not mature properly and die early.
Pernicious Anemia, Folate deficiency anemia
Term
Microcytic-hypochromic anemia
Definition
small abnormally shaped erythrocytes and reduced hemoglobin concentration.
Iron deficiency anemia, Sideroblastic anemia, Thalessemia, chronic bleeding
Term
Normocytic-normochromic anemia
Definition
normal size, normal hemoglobin concentration
Aplastic anemia, Posthemorrhagic anemia, Hemolytic anemia, Sickle cell anemia, Anemia of chronic disease
Term
Pernicious anemia
Definition
Most common form of B12 deficiency.
congenital, acquired, or autoimmune deficiency of intrinsic factor (IF)(needed for absorption of B12) due to destruction of parietal cells in stomach.
Lack of B12 leads to disorder of DNA synthesis in RBC = macrocytic leading to premature cell death.
Tyically effects older people, particularly women (autoimmune), alcoholics, people with poor nurtrition, or vegans.
Term
S/SX of Pernicious amenia
Definition
weakness, fatigue, paresthesias of feet and fingers, difficulty walking, loss of appetite, abd pain, weight loss. Tongue may become sore, smooth, and beefy red. Skin may become lemon yellow (sallow- combo of pale and jaundice), hepatomegaly, R sided heart failure.
Term
CBC for Pernicious anemia
Definition
dec RBC
MVC >100 (size of cells bigger than usual)
Hg wnl
dec B12
inc homocysteine
Term
Folate deficiency aneima
Definition
More common than B12 deficiency especially in alcoholics and poor nurtirion.
caused by dietary folate deficiency.
Term
S/SX Folate deficiency aneima
Definition
severe cheilosis (scales and fissures of the lips and corners of the mouth), stomatitis (inflammation of the mouth), painful ulcerations of buccal mucosa and tongue
S/SX similar to B12 def. except NO NEURO SYMPTOMS
Term
CBC for Folate deficiency aneima
Definition
dec RBC
dec serum folate
inc MCV
MC Hg wnl (Mean corpuscular Hg)
inc homocysteine
Term
Iron deficiency anemia
Definition
Most common microcytic hypochromic anemia.
caused by: chronic blood loss; dietary iron deficiency, and pregnancy.
Term
S/SX Iron deficiency anemia
Definition
sx begin gradually and usually are not severe enough for individual to seek medical attention until stage 3 (Hg 7-8).
Early sx: fatigue, weakness, SOB with exertion, pale earlobes, palms, and conjunctivae.
Later sx: Koilnoychias, tongue becomes red, sore, & painful, Angular stomatitis (dryness and soreness in corners of mouth).
Term
CBC of Iron deficiency anemia
Definition
dec RBC
dec Hg
dec MCV
dec MC Hg
dec serum ferritin
dec serum transferrin
inc total iron binding capacity
Term
Siderblastic anemia
Definition
dysfunctional iron uptake by erythroblasts and defective heme synthesis.
Individuals demonstrate signs of iron overload known as erythropoietic hemochromatosis.
Term
S/SX Siderblastic anemia
Definition
Mild to moderate enlargementof the spleen and liver (liver function remains normal).
Increased tissure iron levels.
Occasionally abnormal skin pigmentation (bronze colored).
Heart rhythm disturbances & CHF are major life threatening complications related to cardiac iron overload.
Term
3 types of Siderblastic anemia
Definition
Acquired- idiopathic
Reversable- d/t ETOH abuse, drug RXN (ex: TB drugs), copper deficiency, hypothermia
Hereditary- Rare. In males d/t being X-linked recessive, present at birth but not seen until mid-life. heart failure d/t iron overload.
Term
Polycythemia vera (aka primary polycythemia)
Definition
abnormal regulation of the multipotent hematopoietic stem cells.
3 types: relative, absolute secondary, absolute primary.
Term
Relative Polycythemia vera
Definition
d/t dehydration- hemoconcentration. resolves with hydration.
Term
Absolute secondary polycythemia vera
Definition
physiologic response to hypoxia.
seen in people who live in high altitude, smoke a lot or COPD/CHF pts.
secondary to a condition causing hypoxia, the body inc RBC to inc O2
Term
Absolute primary polycythemia vera
Definition
Rare
abn proliferation of bone marrow stem cells with self destructive expansion of RBC's.
Mostly seen in white males of eastern european or jewish origin.
Seen between 55-80yo.
Term
S/SX Polycythemia vera
Definition
enlarged spleen, abd pain
inc blood thickness causes thrombosis and vessel occlusion which can lead to tissue and or organ ischemia or infarction and necrosis.
Plethora (ruddy, red color of the face, hands, feet, ears, and mucous membranes).
engorged retinal and cerebral vessels lead to inc risk of stroke.
Inc risk of HTN, MI, Reynauds.
Term
Sickle Cell Anemia
Definition
abnormal HgS (hemoglobin S) in RBC. HgS "sickles" with dec O2. Reversable unless large amts HgS in RBC's. HgS>30% may lead to permenant sickling.
Autosomal recessive disorder (both parents give recessive gene) where valene replaces glutamic acid.
May first be seen at 6-12 months of age as fetal Hg is replaced by HgS.
Infection is most common cause of death related to SCA. Mortality during first 5y = 25%.
Term
4 types of Sickle Cell Anemia crisis
Definition
Vaso-occlusive Crisis (thrombotic crisis)—sickling in the microcirculation. blood flow is obstructed by tangled masses of rigid, sickled cells, vasospasm occurs and a “logjam” effect brings all blood flow through the vessels to a halt. extremely painful and may last for days or even weeks, with an average duration of 4-6 days. It may develop spontaneously or be precipitated by infection, exposure to cold, dehydration, low PO2, acidosis (low pH), or localized hypoxemia.
Sequestration Crisis—large amounts of blood become acutely pooled in the liver and spleen. This type of crisis is seen only in a young child. Mortality rates up to 50% with death caused by cardiovascular collapse.
Aplastic Crisis—a transient cessation in RBC production resulting in acute anemia, occurs as a result of a viral infection, almost always with parvovirus B19. The virus causes temporary shutdown of RBC production in the bone marrow, or reticulocytosis. However, hemolysis continues. The outcome is a severe drop in hemoglobin with an extremely low reticulocyte count.
Hyperhemolytic Crisis—an accelerated rate of RBC destruction. Is very rare but may occur in association with certain drugs or infections. It is characterized by anemia, jaundice, and reticulocytosis.
Term
Thalassemias
Definition
are inherited autosomal recessive disorders that cause an impaired rate of synthesis of one of the two chains—α or β—of Hg A.
Beta-thalassemia, is prevalent among Greeks, Italians, some Arabs, Sephardic jews, and AA.
Alpha-thalassemia, is most common among Chinese, Vietnamese, Cambodians, and Laotians, and AA.
can be major or minor, depending on whether the defects are inherited homozygously (major) or heterozygously (minor).
Term
S/SX Thalassemias
Definition
Beta-thalassemia minor: causes mild to moderate microcytic-hypochromic anemia, mild splenomegaly, bronze coloring of the skin, and hyperplasia of the bone marrow. usually asymptomatic.
Beta-thalassemia major: may become quite ill. Anemia is severe and results in a significant cardiovascular burden, with high-output congestive heart failure. Liver and spleen enlargement. Spinal impairment that starts in infancy retard linear growth. Bone marrow hyperplasia causes a characteristic deformity of the facial bones, as the nasal bridge, mandible, and maxilla widen.
Alpha-thalassemia minor: usually are symptom free, having at most, mild microcytosis. Has clinical manifestations that are virtually identical to those of beta-thalassemia minor.
Alpha-thalassemia major: causes hydrops fetalis and fulminant intrauterine congestive heart failure. In addition to edema and massive ascites, the fetus has a grossly enlarged heart and liver.
Term
Hemolytic anemia
Definition
- caused by premature destruction (lysis) of mature erythrocytes in the circulation due to increased fragility of erythrocytes.
Usually autoimmune or medically induced.
Term
S/SX hemolytic anemia
Definition
jaundice d/t inc billi from destruction of RBC.
Jaundic in sclera, skin, frenulum of tongue, and hard palate.
Term
Anemia
Definition
as reduction in the total number erythrocytes in the circulating blood or a decrease in the quality or quantity of hemoglobin.
Term
Neutrophilia
Definition
Increas in neurtophils caused by inflammation and infection (ex: surgery, burns, strep, staph).
"shift to L" because more immature neurtaphils released.
Term
Neutropenia
Definition
Decrease in neutrophils caused by severe prolonged infection, particularly gram (-) infection, typhoid fever, severe viral infections, and protozoal infestations.
Term
Neurtophil
Definition
Most abundant WBC in body.
Term
Eosinophilia
Definition
Inc in eosinophils caused by allergy, parasitic infection, malignancy, dermatosis, drugs. (Examples: asthma, hay fever, parasites, dermatitis, digitalis, heparin, penicillins.)
Term
Eosinopenia
Definition
Decrease in eosiophils caused by a stress response or drugs. (Examples: trauma, burns, mental distress, steroids.)
Term
Basophilia
Definition
Increase in basophils caused by inflammation, hypersensitivity rxn, myeloproliferative disorder (hodgkins lymphoma).
Term
Basopenia
Definition
Decrease in basophils caused by a physiologic process, or endocrine problems. (Examples: pregnancy, ovulation, stress, Graves' disease.)
Term
Monocytosis
Definition
– caused by infection, hematologic disorders, physiologic processes. (Examples: bacterial endocarditis, TB)
Monocytosis in newborn is normal
Term
Monocytopenia
Definition
caused by a decrease in the number of circulating monocytes it is rare and not much is known about this condition. (Examples: hairy cell leukemia, and prednisone therapy.)
Term
Lymphocytosis
Definition
Increase in lymphocytes caused by physiologic process, infection,& malignancy.
EX: thyrotoxicosis, viral infection, leukemia.
Term
Lymphocytopenia
Definition
Decrease in lymphocytes caused by immunodeficiency syndrome, lymphocyte destruction. (Examples: AIDS, radiation, chemotherapy).
Term
Mononucleosis
Definition
aka "kissing disease", spread via saliva and close personal contact.
Self-limiting acute viral infection on B-lymphocytes (b cells).
Caused Epstein Barr Virus or cytomegalo virus
Incubatoin 30-50d
widespread infection of the b lymphocytes, all of which possess the receptors for EBV. Uninfected b-cells produce IgM, IgG & IgA against virus. Cytotoxic T cells activate. B cells plus T cells plus removal of damaged WBC = lymphnode and spleen swelling.
Term
S/Sx of mononucleosis
Definition
flu-like symptoms such as a HA, malaise, fatigue, arthralgia, fever, chills, and dysphagia which may appear the first 3-5 days.
at time of dx pt has the classic triad of symptoms; pharyngitis (painful thick grey/green exuate), lymphaenopathy, and fever.
Term
Acute Idiopathic Thrombocytopenia Purpura
Definition
caused by immune response, usually to virus. leads to large amts of antigen in blood. Immune complexes bind to platelets causing destruction in the spleen.
resolves when antigens removed from blood.
Term
Chronic Idiopathic Thrombocytopenia Purpura
Definition
IgG antibodies against platelet antigens. More common in adults, espicall women, 20-40yo.
Can get progressively worse leading to spleenectomy.
Main sign = bleeding
Term
S/Sx Idiopathic Thrombocytopenia Purpura
Definition
bruising,purpura, and generalized petechial rash. Asymmetric bleeding is typical and is found on the legs and trunk. Hemorrhagic bullae of the gums, lips, and other mucous membranes may be prominent. Epistaxis may be severe and difficult to control. Intracranial hemorrhage (incidence of less than 1%) is the most serious complication.
Platelets <150K, increased risk hemorrhage
Platelets <15K = spontaneous bleeding
Term
Vitamin K
Definition
Responsible for necessary synthesis and regulation of prothrombin, procoagulant factors (VII, IX, and X) and anticoagulant regulators within in the liver.
Acute and chronic liver disease effects clotting hemostatis leading to increased risk of bleeding disorder.
Term
Disseminated Intravascular Coagulation (DIC)
Definition
Complex acquired clinical syndrome characterize by clotting and hemorrhage at sametime- extensive clotting consumes clotting factors centrally leading to peripherial bleeding.
Amount of activated thrombin exceeds bodys antithrombin and thrombin does not remain localized. widespread thromboses cause widespread ischemia, infarction, and ogan hypoprofusion.
Endothelial damage (inlamation/ septecemia...)is #1 initiator of DIC.
DIC reults from increased protease activity caused by in blood caused by unregulate thrombin followed by fibrin formation, and accelerated finbinolysis.
High mortality rate, treatment is to remove stimulus.
Term
DIC labs
Definition
dec platelets
inc clotting times (INR?)
presence of FDP (fibrin degredation products)and coagulation inhibitors.
Term
S/Sx DIC
Definition
Bleeding from venipuncture sites/arterial lines, purpura, petechiae, hematoma, symmetric cyanosis of fingers and toes.
Term
Hemolytic disease of the newborn (HDN)
Definition
aka:Erythroblastosis Fatalis
Mom Rh neg, partner Rh pos
If baby Rh+ mother's body builds antibodies (IgM/IgG) against Rh+ blood when her blood comes in contact with baby's blood at birth.
IgG antibodies will cross placenta and attack RBC's of subsequent Rh+ fetuses.
Term
Rhogam
Definition
Given at 28W (preventativly)and after birth (if baby is Rh+).
Immunizes mom against Rh+ RBCs.
Passive immunity
Only covers about 30cc of RBC or less
Works for about 14 weeks
Term
Iron deficiency anemia of the child
Definition
Most common cause of insufficient erythropoesis (making RBC).
1/3 of all infants with severe iron deficiency anemia have a chronic GI bleed from too much cows milk. Other cuases include parasitic infestation or hemorrhagic disease.
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