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What are the 2 main groups of normocytic anemia?
Increased RBC production and Normal/decreased RBC production
What is the key test for normocytic anemia?
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
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?
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.
What are the 3 causes of non-heredtiary hemolytic anemias?
- Autoimmune hemolytic anemias
- Mechanical hemolysis
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?
Significant anemia, jaundice/dark urine, rarely splenomegaly
What do you expect to see on a blood film for autoimmune hemolytic anemia? Do you expect a Coomb's test to be positive or negative?
- Numerous spherocytes, polychromasia
- - expect positive coomb's test (presence of autoantibodies on RBC)
Which is more dangerous, warm or cold autoimmune hemolytic anemia?
Warm, as cold can often be treated by avoidance of cold temperatures.
How do you manage autoimmune hemolytic anemia?
- Oral prednisone, tapered over several months
- Splenectomy for steroid-refractory cases
- Immunosuppressives may help
- Transfusions are difficult (hard to cross-match, formation of new antibodies)
What do you expect to be a sign of mechanical hemolysis on a blood film?
What is the most dangerous type of mechanical hemolysis which may be fatal?
Micro-Angiopathic Hemolytic Anemia (MAHA)
What are some types of MAHA?
- TTP (thrombotic thrombocytopenic purpura), HUS (hemolyutic uremic syndrome), DIC (disseminated intravascular coagulation)
- - also includes pregnancy related complications, malignant hypertensions and septicemia
Which types of MAHA is plasmapheresis helpful for managing?
TTP and HUS
True or false: mechanical heart valves/malfunction may cause mechanical hemolysis
What do you do if there is no reticulocytosis present in a patient with normocytic anemia?
Check a bone marrow aspirate and biopsy for pathology
What are some of the possible causes of normocytic anemia when there is no reticulocytosis that may present with bone marrow pathology?
- RBC production may be failing (eg. aplastic or hypoplastic anemia)
- RBC precursors may be replaced (metastatic cancer, myeloma, leukemia, myelofibrosis)
- RBC precursors may be abnormal (dyserythropoietic anemias) - usually hereditary
True or false: splenomegaly can occur in myelofibrosis and dyserythropoiesis?
How do you treat patients with bone marrow pathology, no reticulocytosis, and presenting with normocytic anemia?
Treatment directed at primary diagnosis
What is Aplastic anemia? What are the categories of aplastic anemia?
- Failure to produce 1 or more types of blood cell(s)
- Primary: hereditary, ideopathic or
- Secondary: marrow toxins ie. chemicals, radiation, infections, drugs, immunologic
What are cellular effects of aplastic anemia?
- Effects anemia: pallor, weakness, dyspnea
- Effects thrombocytopenia: bruising, bleeding
- Effects leukopenia: recurrent or persistent infection
True or false: you expect splenomegaly in patients with aplastic anemia
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
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