096 Hemolytic Anemias Flashcards
Main sign of hemolysis (H20 C96 P709)
Jaundice
Telltale sign of intravascular hemolysis (H20 C96 P709)
Hemoglobinuria
LDH is increased by up to how many times normal in intravascular hemolysis? (H20 C96 P709)
10x
The increased number of reticulocytes is associated with increase in this RBC index
MCV
If bone marrow aspiration is done in hemolytic anemia, it will show: (H20 C96 P709)
Erythroid hyperplasia
The essential pathophysiologic process common to all hemolytic anemias (H20 C96 P710)
Increased red cell turnover
Gold standard for proving that the lifespan of red cells is reduced
Red cell survival study
Viral precipitant of aplastic crisis in hemolytic conditions (H20 C96 P710)
Parvovirus B19
Most abundant red cell membrane proteins [2] (H20 C96 P710)
glycophorins, band 3
Main red cell cytoskeletal protein (H20 C96 P711)
Spectrin
Mutations of this gene may account for majority of hereditary spherocytosis (H20 C96 P711)
ANK1
This finding on an ordinary blood count should raise the suspicion of hereditary spherocytosis because HS is almost the only condition in which this abnormality occurs (H20 C96 P712)
MCHC >34
In severe cases of hereditary spherocytosis, it is recommended that splenectomy is done at what age? (H20 C96 P712)
4-6 years
Hemolytic anemia characterized by mutation in SLC4A1 gene. This disorder is also protective against Plasmodium falciparum. (H20 C96 P713)
Southeast Asia ovalocytosis
Glycolytic enzymopathy that can cause severe mental retardation (H20 C96 P714)
Triose phosphate isomerase deficiency
G6PD Vianchan and G6PD Mahidol are widespread G6PD variants in this region of the world (H20 C96 P714)
Southeast Asia
G6PD variant that is distributed worldwide (H20 C96 P714)
G6PD Union
The most serious threat of acute hemolytic anemia in adults is the development of: (H20 C96 P716)
Acute renal failure
Commonest clinical manifestations of G6PD deficiency [2] (H20 C96 P716)
Neonatal jaundice, favism
Aspirin dose that is a possible risk for clinical hemolysis in G6PD deficiency (H20 C96 P716)
> 3 g/day
This hemolytic anemia occuring in the first month of life may be associated with deficiency of glutathione peroxidase (GSHPX) due to a transient nutritional deficiency of selenium. (H20 C96 P717)
Infantile poikilocytosis
Most frequent infectious cause of hemolytic anemias in areas not endemic with malaria (H20 C96 P718)
Shiga toxin-producing E. coli O157:H7
Main mechanism of septicemia causing intravascular hemolysis is exotoxin produced by: (H20 C96 P718)
Clostridium perfringens
Syndrome wherein AIHA is accompanied by autoimmune thrombocytopenia (H20 C96 P719)
Evans syndrome
Evans syndrome may be a manifestation of this primary immunodeficiency (H20 C96 P719)
Common variable immunodeficiency
This drug is increasingly being used together with prednisone as first-line treatment for warm AIHA (H20 C96 P719)
Rituximab
Acquired hemolytic anemia characterized by the involvement of Donath-Lansteiner antibody (H20 C96 P719)
Paroxysmal cold hemoglobinuria (PCH)
Hemolytic anemias characterized by basophilic stippling [2] (H20 C96)
Pyrimidine 5’-nucleotidae (P5N) deficiency, lead poisoning
Best known example of drug-independent drug-induced immune hemolytic anemia (H20 C96 P719)
Methyldopa
This group is unique in that hemolysis results from an inherited defect external to red cells (H20 C96 P717)
Familial/Atypical HUS
In the absence of liver disease, a full-fledged Budd-Chiari syndrome ought to raise the suspicion of: (H20 C96 P720)
Paroxysmal nocturnal hemoglobinuria
Most common cause of death in patients with PNH (H20 C96 P720)
Venous thrombosis (followed by infection and hemorrhage)
PNH may terminate into this malignancy (H20 C96 P721)
Acute myeloid leukemia
Gold standard diagnostic test for PNH (H20 C96 P721)
Flow cytometry
A somatic mutation in this X-linked gene results in PNH (H20 C96 P721)
PIGA
Eculizumab binds to this complement component (H20 C96 P721)
C5
Eculizumab has to be given intravenously at what frequency because of its short half-life (H20 C96 P721)
14 days
Minimum dose of folic acid supplements in PNH (H20 C96 P721)
At least 3 mg/day