Chapter 7 Normochromic Anemias Exam 4 Flashcards
The condition Southeastern Asian Ovalocytosis have the hematology indicators of
spoon-shaped RBCS with 2 bars across the center
The condition Aplastic Anemia causes the defects
antibody directed against antigen and T cells suppress stem cell proliferation
What are the hematologic indicators/symptoms of Aplastic anemia
caused by viruses, chemicals, or radiation
Pancytopenia
What are the defects of G6PD deficiency
It is a sex-linked disorder with an enzyme deficiency in the EMP that is unable to reduce Glutathione to protect RBCS
What are the hematologic indicators/symptoms of G6PD deficiency
decreased Hgb and Hct within 24-48 hours, heinz bodies and bite cells, and may be caused by Fava and Broad bean ingestion
What are the defects caused by Hereditary Pyropoikilocytosis
spectrin deficiency in alpha and beta regions
What are the hematologic indicators/symptoms of Hereditary Pyropoikilocytosis
increased sensitivity to heat; hemolyzes at 46 degrees C
RBCs-crenated, fragmented, and budding
What is the defects of Pyruvate Kinase deficiency
Enzyme deficiency and without PK, RBCS are unable to generate energy or ATP
What are the defects of Fanconi Anemia
a defect in DNA repair, Bone marrow failure
What are the hematologic factors/symptoms of Fanconi anemia
congenital deformities and increased Hgb F
What is the defect of Diamond-Blackfan Anemia
immature blood cells fail to develop normally
What are the hematologic factors/symptoms of Diamond-Blackfan anemia
congenital deformities and increased risk of developing AML
What are the defects of Hereditary Stomatocytosis
stomatin and protein band 7 deficiency, abnormal K and NA permeability
What is the hematologic factors/symptoms of hereditary stomatocytosis
stomatocytes
What are the defects of Paroxysmal Nocturnal Hemoglobinuria
the rbc membrane lacks the proteins CD 55 & CD 59
rbcs are susceptible to complement lysis due to x-linked (PIGA) mutation
CD 55 and CD 59 are affected by this mutation
What is the hematologic picture/symptoms of Paroxysmal Nocturnal Hemoglobinuria
hemolytic anemia, RBCS are destroyed at night
Hemoglobinuria present in morning
Heinz bodies
Sucrose hemolysis test AKA sugar water test
Acid hemolysis test AKA Ham’s test
Flow cytometry
FLAER
What are the defects caused by Hereditary Spherocytosis
deficiency of spectrin and ankyrin
What is the hematologic picture/symptoms of hereditary spherocytosis
spherocytes, polychromasia
Increased osmotic fragility
cells lyse at 0.65% NaCl
Retic counts 3% to 10%
MCHC >36%
cholelithiasis
What is the defects of hereditary eliptocytosis
spectrin deficiency in alpha and beta regions
What is the hematologic picture/symptoms of hereditary eliptocytosis
greater than 25% eliptocytes
What is the hematologic picture/symptoms of hereditary Xerocytosis
RBCs with hemoglobin puddling on the cell’s periphery
What are the defects of Paroxysmal Cold Hemoglobinuria
Anti- P attaches to RBCs at low temperatures
RBCs lyse at warm temperatures
What is the hematologic picture/symptoms of Paroxysmal Cold Hemoglobinuria
intravascular hemolysis and Donath-Landsteiner test
What are the defects of Cold Agglutinin syndrome
IgM autoantibody
Binds complement at 0-5 C
Lysis occurs at 25-30 C
What are the hematologic picture/symptoms of Cold Agglutinin syndrome
CBC samples incubated at 37 C and retested
Positive DAT
Mycoplasma pneumonia
