RBC Disorders (Part 1) Flashcards
Anemia of chronic disease, Chronic renal disease, and Hypothyroidism are caused by?
Decreased Erythropoietin
This results to Leukemia, Leukoerythroblastosis, Aplastic anemia, Lymphoma, Multiple myeloma, Myelofibrosis, and Pure red cell
Bone marrow Damage, infiltration Atrophy
Vitamin & Mineral Deficiency results to?
-Iron
-Vitamin B12 deficiency
-Folic acid deficiency
This is caused by defect in globin synthesis
Thalassemia
Iron overload results to?
-Sideroblastic anemia
-hemochromatosis
Congenital dyserythropoiesis is caused by?
Ineffective Erythropoiesis
Intrinsic defects in Hereditary (membrane) results to?
-Spherocytosis
-Elliptocytosis
-Acanthocytosis
-Stomatocytosis
-Rh null disease
Represent disordered hemoglobin synthesis and has an MCV of <80.
Microcytic Anemia
Characterized by low iron and ferritin with high TIBC
Iron deficiency anemia
Characterized by low iron and ferritin with low TIBC
Anemia of chronic disease
The most common cause of microcytic anemia
iron deficiency
Second most frequent type of microcytic anemia
Anemia of chronic disease
Characterized by an MCV of >100
Macrocytic anemia
Causes deficiencies of folic acid and vitamin B12 deficiency
Megaloblastic; Abnormal DNA synthesis
Usually with an MCV of 100 to 110, but typically without anemia
Alcoholism
Presence of young erythrocytes released early
from the marrow indicates?
anemia caused by hemorrhage or hemolysis
Bone marrow responds maximally by increasing red cell production and releasing young erythrocytes prematurely. It is also characterized with an MCV count of 80-100.
Normocytic Anemia
IDENTIFY: Erythrocytes are usually normocytic, reticulocyte index < 2, E:M ratio is <1:2 and Indirect bilirubin and LDH are normal
hypoproliferative anemias
IDENTIFY:
Retriculocyte index : > 3, E:M ratio is 1:1, Serum LDH & indirect bilirubin are characteristically elevated, Polychromatophilia is prominent
Hemolysis
IDENTIFY: Reticulocyte index is <2, E:M ration is > 1:1 with severe anemias, serum LDH and the indirect bilirubin are
elevated (except in iron deficiency), polychromasia is present
maturation defects
Causes of iron deficiency anemia
Increased need as in pregnancy, children during stages of rapid growth, intake is low, e.g in maturation, malabsorption, blood loss e.g bleeding, excessive donation, e.g in blood donors
Characterized by a progressive
loss of storage iron. RBC development is normal, however, because the body’s reserve of iron is sufficient to maintain
the transport and functional compartments through this
phase
Stage 1 of iron deficiency
Frank anemia. The hemoglobin concentration and hematocrit are low relative to the reference intervals. Depletion of storage iron and diminished levels of transport iron prevent normal development of RBC precursors.
Stage 3 of iron deficiency
Defined by the exhaustion of the storage pool of iron. Quickly the hemoglobin content of reticulocytes begins to decrease, which reflects the onset of iron deficient erythropoiesis, but because the bulk of the circulating RBCs were produced during the period of adequate iron availability, the overall hemoglobin measurement is still normal.
Stage 2 of iron deficiency
This would decrease in iron deficiency anemia
Hemoglobin A2
IDENTIFY: Absence (β0) or a marked decrease (β+) of β- chain production, an excess of α-chains and ineffective erythropoiesis
Thalassemia Major (Cooley’s Anemia)
IDENTIFY: Formed from tetrads of Beta chains, hemoglobin H inclusion, low Red cell count, very low MCV, Low RDW
Hemoglobin H Disease
Common in Southeast Asia, where it is found in about 50% of cases of Hb H disease(αCSα/−−). Normal CV, low RBC count
Hemoglobin Constant Spring (aCSa/)
An abnormal δβ-fusion chain is produced, a
result of chromosome crossing-over and fusion
of genetic material at the δβ-genes
δβ+-Thalassemia
Interferes with heme synthesis by blocking the enzymes
ALAS, ALA dehydratase, and heme synthase
Lead
Only clinical presentation might be a
refractory anemia of pregnancy
Heterozygous B-Thalassemia
Most common single-gene disorder in humans. Its distribution is largely limited to tropical and subtropical regions of Asia and Africa and the Mediterranean
α-Thalassemia
Caused by subacute or chronic infections, such as tuberculosis, lung abscess, and bacterial endocarditis. Other cases may be caused by
neoplasms, rheumatoid arthritis, rheumatic fever, systemic lupus erythematosus (SLE), uremia, or
chronic liver disease
Anemia of Chronic Inflammation
Promotes liver production of hepcidin, an acute phase reactant that impairs iron absorption in intestinal enterocytes and iron release from macrophages and hepatocyte
Interleukin-6 (IL-6)
Chronic infections (bacterial, viral, parasitic, or fungal),malignancy, or autoimmune dysregulation result in the release of inflammatory cytokines from activated macrophages and T lymphocyte
Mechanisms of anemia in chronic inflammatory conditions
In this type of anemia, the body has
adequate iron but is unable to incorporate it into hemoglobin synthesis
Sideroblastic Anemia
Most common acute and probably the most common inherited porphyria PBG deaminase deficiency. Elevated levels of ALA & PBG in urine during acute attacks
Acute Intermittent Porphyria (AIP)
Most common of the reversible sideroblastic anemia
Ethanol-induced anemia
Often associated with malnutrition
Primary pyridoxine anemia
Diagnosis includes: Decrease activity of the enzyme ALA synthase, increase serum iron, increase percent saturation, and decrease TIBC
Sideroblastic anemia
Most common of the porphyrias in the United States
Porphyria Cutanea Tarda (PCT)
One of the most common genetic disorders in persons with European ancestry
Hereditary hemochromatosis
Characterized as “bronze diabetes”
Hereditary Hemochromstosis
Characterized by increased serum iron, increased TIBC, increased % saturation, and increased ferritin
Hereditary Hemochromstosis
Characterized by increased serum iron, normal TIBC, normal % saturation, and normal ferritin
Thalassemia minor
