patho Flashcards
(37 cards)
▪ Any condition that causes the total number of RBCs, the amount of Hb, or the volume of packed RBCs to decrease.
▪ Caused hypoxia in tissues
▪ General symptoms include dyspnea on exertion, fatigue, dizziness, tinnitus,
headache
▪ Long-term -> pallor, tachycardia, systolic ejection murmur, orthostatic hypotension.
ANEMIAS
IRON DEFICIENCY ANEMIA
▪ Due to chronic blood loss, increased need for blood and oxygen (pregnancy), deficiency intake or poor absorption.
▪ Microcytic, hypochromic (smaller and less color RBCs) anemia
▪ Fatigue, lethargy, pale mucous membranes, low Hb, low RBC, low hematocrit,
small RBCs, low MCV, MCH and MCHC, low serum ferritin and iron.
▪ Long-term def -> in chronic fatigue, low energy, and depression.
▪ Severe def -> gastritis, glossitis, Plummer-Vinson syndrome (dysphagia, esophageal webs)
IRON DEFICIENCY ANEMIA
▪ Vitamin B12 or folic acid deficiency or malabsorption which affects DNA synthesis.
▪ Folic acid deficiency can be caused by alcoholism, pregnancy, intestinal
malabsorption, and folic acid antagonists
▪ Megaloblastic anemia – enlarged abnormal prescursor cells in the bone marrow.
▪ Sx: Fatigue, lethargy, low RBC, low Hb, low hematocrit, large cells (increased MCV, MCH, and normal MCHC), ferritin and iron levels normal, low serum B12, low serum folate, increased serum/urine methylmalonic acid (B12 deficiency), high serum homocysteine levels (folate deficiency), polysegmented neutrophils, pancytopenia
▪ Complications include dementia, depression, glossitis, stomatitis, paranoia, neuropathies, ataxia, hyperreflexia, death
MACROCYTIC ANEMIA
▪ An autoimmune disease that reduces the absorption of food-bound B12 by destroying
intrinsic factor (IF)
▪ Anti-parietal Ab and anti-intrinsic factor Ab
▪Chief cells are damaged resulting in achlorhydria and lack of pepsinogen secretion
▪ Autoimmune atrophic gastritis often results.
▪Complications include recurrent GI infection and the same issues as B12 deficiency
Pernicious anemia
▪ Severe anemia that results from a loss of the precursor cells of RBCs
▪ Results from toxic exposure (drugs, radiation, chemicals) or viral infection.
▪ Bone marrow is hypocellular (< than the normal # of cells) and all cell lines are decreased (peripheral pancytopenia)
APLASTIC ANEMIA
▪ Myeloproliferative disorder that result in the neoplastic clonal proliferation of myeloid stem cells within the marrow.
usually d/t aquired **JAK2 mutation **
▪ Results in an increased number of circulating RBCs, increased hemoglobin, and a moderate increase in circulating granulocytes and platelets.
▪ Splenomegaly and decrease erythropoietin results.
▪ Weakness, fatigue, vertigo, irritability, tinnitus, facial flushing,extremity pain/erythema, blue/black spots on the skin can occur.
POLYCYTHEMIA VERA
▪ Secondary to another condition that causes an increased need for RBCs
▪ High altitude exposure, right-to-left shunts, shock, abnormal Hb
▪ Can also be caused by inappropriate production of erythropoietin by tumors (renal cell carcinoma and hepatocellular carcinoma)
▪ Pheochromocytoma and Cushing’s syndrome can also cause this condition.
▪ High erythropoietin (EPO) result
SECONDARY POLYCYTHEMIA
DISEASES INVOLVING
PRODUCTION OF BLOOD
CELLS
▪ Anemias
▪ Iron deficiency anemia
▪ Macrocytic anemia
▪ Aplastic anemia
▪ Polycythemia vera
▪ Secondary polycythemia
what group of anemias:
▪ Sickle cell anemia
▪ Thalassemias
▪ Hereditary spherocytosis
▪ Glucose-6-phosphate dehydrogenase deficiency
HEMOLYTIC ANEMIAS
▪ Destruction of RBCs
▪ Can cause anemia
▪ Jaundice, hemosiderosis, and usual anemia symptoms
Causes include:
▪ Mechanical trauma to cells
▪ Heart valves, clots
▪ Complement-induced damage
▪ Extravascular hemolysis (within the spleen)
hemolysis
▪ Abnormal hemoglobin (HbS) is produced due to a genetic disease.
▪ People who are heterozygous for the gene are generally asymptomatic.
▪ Those who are homozygous for the defective gene results in deformed RBCs.
▪Can obstruct capillaries and are removed and destroyed by the spleen
▪ Severe hemolytic anemia, chronic leg ulcers, infarcts of the lungs and spleen, vaso-occlusive painful crisis (limbs, back, chest, abdomen), and aplastic crises
SICKLE CELL ANEMIA
THALASSEMIAS
▪ Genetic disorders characterized by deficient production of either of the two globin chains of hemoglobin.
▪ Hypochromic, microcytic anemia
▪ Beta-thalassemias is the most common form in the Mediterranean and US
▪ Majorandminorforms
▪ Defectsinthebetachainofhemoglobin
▪ Major: anemia, splenomegaly, distortion of the facial and long bones, hemosiderosis ▪ Minor: generally asymptomatic
▪ Alpha-thalassemias is most common in Southeast Asia
▪ Resultsfromadefectofoneormoreof4alphahemoglobingenes
▪ The higher the number of deletion the more clinical abnormalities
▪ (Beta/alpha) -thalassemias is the most common form in the Mediterranean and US
▪ Majorandminorforms
Defects in the beta chain of hemoglobin
▪ Major: anemia, splenomegaly, distortion of the facial and long bones, hemosiderosis
▪ Minor: generally asymptomatic
Beta-thalassemias
(Betal/Alpha)-thalassemias is most common in Southeast Asia
▪ Results from a defect of one or more of 4 alpha hemoglobin genes
▪ The higher the number of deletion the more clinical abnormalities
Alpha-thalassemias
▪ Genetic disorders characterized by deficient production of either of the two globin chains of hemoglobin.
▪ Hypochromic, microcytic anemia
THALASSEMIAS
▪ X-linked disorder which causes hemolytic anemia during oxidative stress and is caused by a number of drugs
▪ G6PD is the rate-limiting enzyme for the HMP shunt
It maintains NADPH levels and reduced glutathione
Heinz bodes & bite cells on blood smear
GLUCOSE-6 PHOSPHATE
DEHYDROGENAGE
DEFICIENCY
▪ Genetic defect that causes spherical RBCs that get stuck in the spleen and destroyed
▪ Autosomal dominant
HEREDITARY SPHEROCYTOSIS
Occurs when the mother and fetus have different blood types
▪ Mother is Rh-but baby is Rh+ where the mother generates antibody against the Rhantigens
▪ ABO incompatibility
Hemolysis results in elevated bilirubin levels and jaundice.
▪ Risk of kernicterus, hepatosplenomegaly
No complications occur in the initial pregnancy.
▪ In subsequent pregnancies, maternal antibodies cross the placenta and lyse fetal RBCs which causes anemia, hypoalbuminemia, and possibly high-output heart failure, or fetal death.
Maternal sensitization and antibody production due to Rh incompatibility can be prevented by giving the woman Rh immune globulin.
ERYTHROBLASTOSIS FETALIS
▪ Disseminated intravascular coagulation (DIC)
▪ Hemophilia
▪ Thrombocytopenia
▪ Von Willebrand’s disease
▪ Vitamin K deficiency
CLOTTING ABNORMALITIES
▪ Common hemorrhagic syndrome that occurs after uncontrolled activation of clotting factors and fibrinolytic enzymes throughout small blood vessels.
Triggered by massive tissue damage, sepsis, pregnancy, cancer
▪ Caused by the release of tissue thromboplastin or activation of the intrinsic pathway of
coagulation
▪ Results in prolonged PT, APTT, bleeding and thrombin time
Acute DIC results in fibrin deposition everywhere and platelets and clotting factors are consumed
▪ Fibrin degradation products (D-dimers) inhibit fibrin polymerization and block platelet function.
▪ Outcome is lethal tissue necrosis and bleeding.
Chronic DIC occurs primarily as a result of certain cancers, retained dead fetuses, hemangiomas, and aneurysms.
DISSEMINATED
INTRAVASCULAR
COAGULATION
▪ Blood coagulation disorders (X-linked recessive) due to absence, deficiency, impaired activity of clotting factors which results in prolonged bleeding.
HEMOPHILIA
stages for which blood coagulation cdx:
Mild (5-40% of factor levels)
▪ Asymptomatic or experience prolonged bleeding only after serious injury
Moderate (1-5% normal factor levels)
▪ Prolonged bleeding after injuries and occasional spontaneous bleeding.
Severe (<1% normal factor levels)
▪ Prolonged bleeding even after minor injury, frequent spontaneous bleeding
Hemophilia
Hemophilia A - Factor (VII/VIII) deficiency
▪ Normal bleeding time, platelet count, prothrombin time but prolonged activated partial thromboplastin time
▪ Bleeding occurs in the muscles, subcutaneous tissues, and joints.
Hemophilia B - Factor (IX/X) deficiency
▪ Similar to Hemophilia A
▪ Hemophilia C (Autosomal Recessive) - Factor (XI/XII) deficiency
▪ Hemophilia A - Factor VIII deficiency
▪ Hemophilia B - Factor IX deficiency
▪ Hemophilia C (Autosomal Recessive) - Factor XI deficiency
▪ Reduction in the number of platelets which results from decreased production, sequestration, or increased destruction of platelets.
▪ Decreased production of platelet so ccurs with bone marrow damage, congenital abnormalities, or nutrition deficiencies (B12 and folate).
▪ Petechial cutaneous bleeding and prolonged bleeding time.
▪ Sequestration occurs in splenomegaly → causes platelets to accumulate in the spleen
▪ Increased destruction of platelets occurs in disseminated intravascular coagulation (DIC) or purpura.
THROMBOCYTOPENIA
▪ Platelets require vWF to adhere to the endothelial basement membranes in order to begin clot formation.
▪ vWF is also important in stabilizing factor VIII
▪ vWF is either absent or abnormal in this disease which causes increased
bleeding time (PTT).
▪ Autosomal dominant
▪ Acquired Von Willebrand disease can occur in some autoimmune diseases, aortic
stenosis, Wilm’ s tumor, and hypothyroidism.
▪ Results in impaired platelet adhesion and prolonged bleeding time.
▪ Results in mild prolonged bleeding to severe, hemophilia A-like spontaneously bleeding into joints
VON WILLEBRAND’S DISEASE
