BLI 9 - Normocytric Anemia II (Siddiqui) Flashcards
Usually asymptomatic but may have difficulty concentrating urine
Hb SA (sickle cell trait)
In sickle cell disease, hemolysis is mostly _______
extravascular
Sickle cell disease point mutation
Glutamic acid to valine at 6th position
RIsk due to autosplenectomy
risk of infection from encapsulated organisms Spleen has fibrosed due to ischemia and infarction –> autosplenectomy
Spherocytosis w/ positive coombs test
AIHA
O157:H7
HUS Verotoxin causes endothelial cell damage –> platelet microthrombi –> schistocytes
Organism responsible for osteomyelitis in the a patient with sickle cell disease
Organism responsible for osteomyelitis in normal pt
Salmonella typhi S. aureus
Clinical manifestations for TTP/HUS
FAT RN
Fever
Anemia
Thrombocytopenia
Renal insufficiency – more HUS
Neurologic abnormalities – more TTP
Homozygous Hb S
Sickle cell disease
vaso-occlusive crisis
Sickle cell disease – sickled RBCs obstruct microvasculature
Schistocytes
MAHA
Hb C disease mutation
Glutamic acid –> Lysine at 6th position
Pancytopenia
Low RI
Paroxysmal Nocturnal Hemolysis
Aplastic Anemia
Hb SS vaccine
Pneumococcal
Heterozygous Hb S
Sickle cell trait
S. pneumo H. flu
Encapsulated organisms with increased risk of infection after autosplenectomy
Aplastic crisis
Stroke
Acute chest syndrome
Autosplenectomy
Aseptic necrosis
Skin infections/ulcers
Sickle cell disease
Detects antibody-coated RBCs
Direct Coombs test
Target cells Hb crystals
Hb C disease
Best screening test for DIC
D-dimer
IgM mediated AIHA: hemolysis
Intravascular hemolysis
Sepsis
Abruptio placentae
Trauma/burns
Acute promyelocytic leukemia
Etiologies of DIC
Sickle cell disease effect on bone marrow
Necrosis
Tx of TTP/HUS includes plasmaphoresis and steroids, but never ________
platelet transfusion
Hematopoietic stem cell defect
Aplastic Anemia
Like thalassemia, shows crewcut appearance on xray with chipmunk facies and hepatomegaly
Sickle cell disease
SLE, RA CLL PCN, quinidine, methyldopa
Warm agglutinin AIHA diseases
IgM (complement-fixation): Cold or warm Extravascular or intravascular
Cold Intravascular
Pulmonary risk of sickle cell disease
Acute chest syndrome
Initial presentation of sickle cell disease >6 mo
Dactylitits
Stroke/TIA sequelae Hypersplenism
Chronic complications of sickle cell disease
IgG mediated AIHA: hemolysis
Extravascular hemolysis
Sickle cell disease confirmatory test
Hemoglobin electrophoresis
Sickle cell disease inheritance pattern
Autosomal recessive
3 deoxygenated states that cause Hb S polymerization
Hypoxemia Acidosis Dehydration
Components of Microangioapthic Hemolytic Anemia (MAHA)
TTP/HUS & DIC
Mycoplasma pneumoniae Infectious Mononucleosis
Cold agglutinin AIHA diseases
ADAMTS13
TTP Decreased ADAMTS13 = failure to cleave large vWF multimers –> platelet microthrombi –> schistocytes
IgG (Opsonization): Cold or warm Extravascular or intravascular
Warm Extravascular
In sickle cell disease, this form of hemoglobin is protective against sickling
Hb F
Detects antibodies in serum
Indirect Coombs test
Hydroxyurea
Increased Hb F –> protective against sickling