Hematology - Anemias Review Flashcards
What does it mean to increase and decrease the RPI?
Hight RPI (>3%) means a compensatory RBC production
Low RPI (<3%) indicates inadequate response to correct anemia
What does it mean to Mentzer index > 13 and < 13.
An index of > 13 suggest iron deficiency anemia
An index of < 13 suggest thalassemia
Which disease has low iron, high TIBC, low Ferritin and low transferrin saturation?
Iron deficiency
Which disease has low iron, low TIBC, high Ferritin and low transferrin saturation?
Chronic disease
Which disease has high iron, low TIBC, high ferritin and high transferrin saturation?
Hemochromatosis
What are the clinical signs of beta thalassemia major?
Severe microcytic anemia with target cells
Anisopoikilocytosis
Increase risk of hemocromatosis due increase requiring blood transfusions
Marrow expansion
Skeletal deformidades
Extramedullary hematopoiesis
What are the clinical signs of beta thalassemia major?
Severe microcytic anemia with target cells
Anisopoikilocytosis
Increase risk of hemocromatosis due increase requiring blood transfusions
Marrow expansion
Skeletal deformidades
Extramedullary hematopoiesis
Explain the sign “crew cut” on skull x-ray
It corresponds to the increase in extramedullary hematopoiesis, and can be seen in patients with beta thalassemia.
What is the enzyme affected in lead poisoning?
ALA dehydratase
What is the enzyme affected in sideroblastic anemia? What is your relationship with tuberculosi treatment?
ALA synthase
Decrease vitamin B6. Deficiency in this vitamin is commonly seen in patients taking isoniazid for the treatment of tuberculosis.
What are the neurologic structures affected in cobalamin deficiency?
Spinocerebellar tract, lateral corticospinal tract, dorsal columm dysfunction.
What are the causes of non megaloblastic anemia?
Chronic alcohol overuse, liver disease and Diamond Blackfan anemia
What is the problem with Diamond Blackfan anemia? What is the difference with Fanconi anemia?
Diamond Blackfan anemia is due to intrinsic defect in erythroid progenitor cells. This is a congenital form of pure red cell aplasia, different from Fanconi anemia, which causes pancytopenia.
Explain the decrease in iron in anemia of chronic disease.
Infammation increase IL-6 -> increase hepcidin-> decrease release of iron from macrophages and decrease iron absorption from gut.
What are the intrinsic hemolytic anemias?
Hereditary spherocytosis
Paroxysmal nocturnal hemoglobinuria
G6PD deficiency
Pyruvate kinase deficiency
Sickle cell anemia
HbC disease
What are the causes of extrinsic hemolytic anemias?
Autoimmune hemolytic anemia
Drug induced hemolytic anemia
Microangiopathic hemolytic anemia
Macroangiopathic hemolytic anemia
Infection
What are the findings in intravascular hemolysis?
Low haptoglobin, high schistocytes on blood smear, hemoglobinuria, hemosiderinuria and urobilinogeb in urine. Increase DHL.
What are the findings in extravascular hemolysis?
Splenomegaly, spherocytes in peripheral smear, NO hemoglobinuria/hemosiderinuria. Can present with urobilinogen in urine.
Classify the intrinsic hemolytic anemias in intravascular or extravascular hemolysis.
Hereditary spherocytosis -> extravascular hemolysis
Paroxysmal nocturnal hemoglobinuria -> intravascular hemolysis
G6PD deficiency -> extravascular and intravascular hemolysis
Pyruvate kinase deficiency -> extravascular hemolysis
Sickle cell anemia -> intravascular and extravascular hemolysis.
HbC disease extravascular hemolysis
Name examples of drugs that cause hemolytic anemia.
Antibiotics (penicillins, cephalosporins)
NSAIDs
Immunotherapy
Chemotherapy
There are two types of Autoimmune Hemolytic Anemia (AIHA), explain.
Warm AIHA - Chronic anemia in which primarily IgG causes extravascular hemolysis.
Cold AIHA - Acute anemia in which primarily IgM + complement cause RBC agglutination and extravascular hemolysis upon exposure to cold.
Explain the mechanism of micro and macroangiopathic hemolytic anemia.
Microangiopathic -> RBC are damage when passing through obstructed or narrowed vessels. Seen in DIC, TTP/HUS, SLE, HELLP syndrome, hypertensive emergency.
Macroangiopathic -> results from shear stress caused by turbulent blood flow or occurs in patients with prosthetic heart valves and aortic stenosis.
