Increased RBC production and Normal/decreased RBC production
What is the key test for normocytic anemia?
Reticulocyte count
What are the 2 types of normocytic anemia associated with reticulocytosis?
Hemolysis and Acute blood loss
What are the 2 types classifications of normocytic anemia associated with no reticulocytosis?
Marrow pathology and no marrow pathology
If reticulocyte count doesn't increase with anemia, what must be the case?
RBC production must be abnormal
What are the classifications of hemolytic anemia?
Acquired/hereditary or intrinsic/extrinsic RBC defect
True or false: hemolytic anemias may be mildly macrocytic because of increased reticulocytes
True
What are the classifications of hereditary hemolytic anemias?
Membranopathies: disorders of RBC cytoskeleton
Hemoglobinopathies: disorders in quantity or quality of Hb
Enzymopathies: disorders of the glycolytic enzyme pathways in RBCs
What are examples of hereditary membanopathies (which lead to hemolytic anemia)?
Hereditary spherocytosis and elliptocytosis
What are key findings in RBC membranopathies?
Abnormal RBC shape, reticulocytosis, splenomegaly. You may expect a family history
What are the 2 major groups of hemoglobinopathies?
Quantitative defects: thalassemias
Qualitative defects: Sickle cell disease most common
True or false: There are many reliable prognostic variables identified for sickle cell disease
False - there are none
What are some of the systemic consequences of sickle cell disease?
Sickle crises (CNS, lungs, abdomen), organ infarction, severe hemolysis, pigment gallstones, chronic nephropathy, aplasia, shortened lifespan
How do you manage sickle cell disease?
Mainly supportive (transfusions, fluids, analgesia), Hydroxyurea to increase HbF content; bone marrow transplants are the only cure
What are the most common causes of RBC enzymopathies, which lead to hemolytic anemia?
G6PD and pyruvate kinase deficiency
How is G6PD deficiency inherited?
Autosomal recessive
What populations have higher incidence of G6PD deficiency?
Mediterranean and African populations
What are known inducers of G6PD deficiency-associated hemolysis?
Fava beans, drugs (sulfas, antimalarials, napthalene) that cause cells to lyse with oxidative stress
True or false: you expect to see Heinz body formation in G6PD deficiency anemia.
True
What are the 3 causes of non-heredtiary hemolytic anemias?
Autoimmune hemolytic anemias
Mechanical hemolysis
Infections
What are the causes of Autoimmune hemolytic anemias?
Usually idiopathic but sometimes connected to medications: quinidine, penicllin, methyl-dopa, or 2' infections. Autoimmune disease may be present such as lupus. Lymphoid malignancies may also cause it in older individuals (ie. luekemia or lymphoma).
What are some signs of autoimmune hemolytic anemias?
Effects leukopenia: recurrent or persistent infection
True or false: you expect splenomegaly in patients with aplastic anemia
False
How may a bone marrow biopsy look in a patient with Aplastic anemia?
Fat cells may have taken up a lot of the space
How do you treat aplastic anemia?
Blood products, antibiotics for neutropenic fever, immunosuppression with anti-thymocyte globulin (ATG), cyclosporin, corticosteroids, bone marrow transplant if eligible
What is the type of anemia associated with no reticulocytosis and no bone marrow pathology?
Anemia of chronic inflammation, which is a heterogenous group of disorders resulting in decreased RBC production