Heme/Onc - mostly anemia stuff Flashcards
Name 2 MICROcytic anemias.
iron deficiency
thalassemia
Name 4 MACROcytic anemias.
- folic acid deficiency
- B12 deficiency
- liver disease
- myelodysplasia
Name a normocytic anemia.
anemia of chronic disease
What is hemolytic anemia, in general?
The hemolytic anemias are a group of disorders in which red blood cell survival is reduced, either episodically or continuously. The bone marrow has the ability to increase erythroid production up to eightfold in response to reduced red cell survival, so anemia will be present only when the ability of the bone marrow to compensate is outstripped. This will occur when red cell survival is extremely short or when the ability of the bone marrow to compensate is impaired.
How is reticulocytosis related to hemolytic anemia?
Reticulocytosis is an important clue to the presence of hemolysis, since in most hemolytic disorders the bone marrow will respond with increased red blood cell production and earlier release of young red blood cells into the circulation.
Reticulocytosis does not necessarily imply hemolysis, since it also occurs during recovery from hypoproliferative anemia (replacement of a missing nutrient) or acute bleeding.
Hemolysis is correctly diagnosed (when acute bleeding is excluded) if the hematocrit is either falling or stable despite reticulocytosis.
What disorders are considered hemolytic anemias? (7)
- sickle cell anemia (Hb S disease)
- sickle cell trait
- G6PD deficiency
- cold agglutinin disease
- hemoglobin S-C disease
- hereditary spherocytosis
- autoimmune hemolytic anemia
What is the main hormone of iron regulation?
hepcidin
What is hemochromatosis?
an disease of iron overload
Hemochromatosis is an autosomal recessive disease caused in most cases by a mutation in the HFE gene on chromosome 6
When is hemochromatosis usually diagnosed?
The onset of clinical disease is usually after age 50 years—earlier in men than in women; however, because of widespread liver biochemical testing and iron screening, the diagnosis is usually made long before symptoms develop.
What are the s/s of hemochromatosis?
Early symptoms are nonspecific (eg, fatigue, arthralgia).
Later clinical manifestations include: arthropathy hepatomegaly evidence of hepatic dysfunction, skin pigmentation (combination of slate-gray due to iron and brown due to melanin, sometimes resulting in a bronze color) cardiac enlargement diabetes mellitus erectile dysfunction in men
What lab findings indicate hemochromatosis?
mildly abnormal liver tests
elevated plasma iron with > 45% transferrin saturation
elevated serum ferritin
What is the treatment of hemochromatosis?
PHLEBOTOMY
PPI
- avoid foods rich in iron and supplemental iron
- weekly phlebotomies of 1 or 2 units of blood is indicated in all symptomatic patients, those with a serum ferritin level of at least 1000 mcg/L, and those with an increased fasting iron saturation and should be continued for up to 2–3 years to achieve depletion of iron stores
- hematocrit and serum iron values should be monitored
- a proton pump inhibitor, which reduces intestinal iron absorption, decreases the phlebotomy requirement
- the chelating agent deferoxamine is indicated for patients with hemochromatosis and anemia or in those with secondary iron overload due to thalassemia who cannot tolerate phlebotomies
Name 3 diseases of bone marrow failure.
- aplastic anemia
- myelodysplasia
- pure red cell aplasia
In what ethnic groups is alpha-thalassemia primarily seen?
people of southeast Asia, China
In what ethnic groups is beta-thalassemia primarily seen?
people of Mediterranean origin
