Anemias Flashcards
What is the best indicator of red cell production?
reticulocyte count
For the kinetic classicfication of Anemia, what falls under inadequate production of RBC?
Hypoproliferative
* Impaired RBC production. Lower than expected numbers of RBC precursors in the marro
Ineffective erythropoiesis
* impaired RBC production despite increased bone marrow RBC precursors. Assembly line “rejects” via apoptosis defective RBC precursors
Can see with: Missing ingredients, low signal (EPO) some meds, infections, inflammation, autoimmunity, genetic, primary marrow disorder
For the kinetic classification of Anemia, what falls under increased RBC destruction or loss (RBC precursors & retics high)
- hemolysis : shortened lifespan of circulating RBC
- blood loos
MCV > 100
macrocytic anemia
MCV < 80
Microcytic anemia
MCV 80-100
Normocytic Anemia
Variation in RBC shape?
poikilocytosis
variation in RBC size
anisocytosis
cell with large, pale centers (less hemoglobin per cell)
hypochromia
what are some conditions that cause macrocytosis?
- recticulocytosis
- Folate deficiency
- B12 deficiency
- primary bone marrow problem (myelodysplasia, leukemia, multiple myeloma, MGUS
- Liver disease, EtOH
- thyroid disease
- medications (hydroxyurea)
- genetic conditions
Conditions that cause normocytosis?
- anemeia of kidney disease
- anemia of chronic disease
- mixed anemia
- early blood loss
conditions that cause microcytosis?
- Iron deficiency
- thalassemia
- anemia of chronic disease
- hereditary spherocytosis
- lead poisoning
If the bone marrow is responding to the anemia appropriately, what would you expect the reticulocytes to be?
High
Bone marrow is adequately trying to correct anemia
think blood loss or hemolysis
if the bone marrow is not responding to anemia appropriately, what you expect reticulocyte count to be?
Low
(bone marrow might be struggling
think ineffective erythropoiesis of hypoproliferation
what would be the lab findings in iron deficiency anemia
- Decreased ferritin, increased TIBC, Decreased serum iron
Lab findings
*hypochromic
*microcytic
*High RDW (anisocytosis)
symptoms of iron deficiency?
- fatigue
- pallor
- atrophic glossitis
- pica (eating dirt or ice)
- koilonychia (spooned nails)
- brittle nails
- restless legs
- cheilosis (cracking, crusting around the mouth)
what are some causes of iron deficiency?
- blood loss (GI bleed secondary to PUD or NSAID use or cancer)
- pregnancy and lactaton
- malabsorption (celiac, h. pylori, bariatric surgery, PPI
- poor nutritional intake
diminished or absen synthesis of globin chains (alpha or beta)
Thalassemia
what labs would you expect in thalassemia?
- hypochromia
- smear: target cells- could have basophilic stippling
- normal iron studies or high serum iron and ferritin
how do you diagnosise thalassemia?
how do you treat thalassemia?
Hgb electrophoresis
transfusions (RBC transfusion every 3-4 weeks, chelation of their iron)
stem cell transplant is curative, but morbid
due to deficient alpha chain synthesis (excess beta chains)
alpha thalassemia
Due to a deficiency of beta chains (excess of alpha chains)
beta thalassemia
what is beta thalassemia major? what are the symptoms
a severe transfusion dependent childhood disease, seen in the first year of life
sx: Pallor, jaundice and dark urine, hemolysis, irritability, anemia, abdominal swelling from hepatosplenomegaly
what is beta thalassemia minor?
mild anemia- but child is able to live full life
- microcytic
- hypochromic anemia
- ring sideroblasts are seen on prussion blue stained bone marrow aspirate smear
- can be congenital vs acquired
sideroblastic anemia
- normocytic anemia with marked reticulocytosi
- clinical sx: tachycardia, tachypnea, hypotension (decreased intravascular volume)
hemorrhage: acute blood loss
- seen in chronic immune activation/ chronic infection and malignancy
- sustained systemic inflammation alters iron utilization in the marrow, supresses hematopoiesis, and blunts the response of EPO to anemia
Anemia of chronic disease
what labs would you see in anemai of chronic disease?
- normocytic, normochromic
- may be microcytic in 1/3 cases
- normal or elevated ferritin
- serum iron usually low, TIBC low
- macrocytic
- megaloblastic anemia (hypersegmented neutrophils)
- sx: parasthesia, weakness, and unsteady gait; may have neurologic changes that +/- reversibility
B12 deficiency
diagnosis and treatment of B12 deficiency
Dx:
* Low B12, or Normal B12 w High MMA
tx:
* Parenteral B12 for absorptive issue, oral or parenteral for diet related
what is pernicous anemia? how can you diagnose it?
B12 deficiency due to lack of intrinsic factor (loss of parietal cells which make the intrinsic factor or lack of ileum which absorbs it)
- antibodies to intrinsic factor or parietal cells can diagnose pernicious anemia
- megaloblastic anemia
- macrocytic anemia
- hypersegmeneted neutrophils
- Serum B12 is normal, MMA is normal, homocysteine is elevated
Folate deficiency
What are symptoms of folate deficiency? what are some causes?
- fatigue, presyncope but no neuro defects
- dietary factors- no enough folate, alcoholism
- folate is needed in pregnancy to reduce the incidence of neural tube defects
compare and contrast the causes of folate and B12 deficiency
folate
* malnutrition
* pregnancy/lactation
* malabsorption
* drugs (e.g, TMP sulfa, methotrexate)
* alcohol
* chronic hemolytic anemai
B12
* malabsorption (pernicious anemia, gastrectomy, pancreatic insufficiency, small intestine resection, chrons
* inadequte intake (strict vegan)
if your patient had a decrease in plasma volume due to dehydration, what would you see on the CBC?
mildly elevated WBC, RBC, plt
what are the hereditary hemolytic anemias?
hereditary spherocytosis
G6PD deficiency
thalassemia
sickle cell
what are the “trauma” hemolytic anemias?
TTP, HUS, DIC
- an inherited abnormality of the RBC, defect in the structural membrane proteins
- the abnormal cells are sperichal and are removed by the spleen resulting in reduced red cell life
Herediatry spehreocytosis
symptoms, diagnosis and treatment of hereditary spherocytosis?
sx: jaundice, pallor, splenomegaly
dx: spherocytes on blood smear, pincer cell (mushroom shaped) negative direct antiglobulin test (DAT), elevated reticulocyte count
tx: supportive care to splenectomy in severe
- Autosomal recessive genetic defect
- abnormal production of beta-globin subunit of Hgb (qualitive defect)
- onset/presentation: often in the first year of life when HgbF falls
- splenomegaly is from increase sequestration and destruction of abnormally shaped cells
Sickle Cell Disease
- rare condition that occur after the bone marrow becomes damaged
- labs: complete absence of hematopoiesis (decreased RBC, WBC &Plt) decreased reticulocytes
aplastic anemia
how do you diagnose and treat aplastic anemia? what are some causes
dx: bone marrow biopsy
tx: depends on severity ( supportive transfusions/observation, transplant, horse ATG+cyclosporine)
causes: acute or chronic; exposure to agents like benzene, viral infection, pregnancy, idiopathic, certain antibiotics- sulfa, chloramphenicol
- one of the most common causes of aquired hemolytic anemia- autoantibody to your own RBCs
- can be caused by viral infection, SLE or other autoimmune conditions, lymphoproliferative disorder/cancer, drugs (PCN, methyldopa)
Autoimmune hemolytic anemia
what lab levels would you expect in autoimmune hemolytic anemia
- anemia
- MCHC increased (spherocytosis)
- reticulocytes elevated
- elevated indirect bilirubin, LDL, direct coombs test
differentiate between cold and warm hemolytic anemia. How are they treated?
Cold- IgM antibodies that react with RBC surface at temps below core temp of body
* direct coombs test positive (usually IG-, C3+)
Warm- IgG antibodies attack RBC
* direct coombs test positive (usually IG+, C3-)
TX: warm needs steroids and treatment of underlying issue, cold sometimes needs chemo
- most common enzyme deficiency. X-linked recessive disorder seen in black males and some mediterranean populations
- clinically patients are healthy until oxidative stress causes hemolysis.
- oxidative drugs and fava been intake, infection –> episodic hemolysis
- Enzyme catalyzes NADP to NADPH, without it, NADPH is unable to protect RBC from destruction from oxidative stress
G6PD deficiency
what labs would you expect to see in G6PD deficiency?
- increased reticulocytes
- indirect bilirubin (both increaed during hemolytic episode)
- G6PD will be low between hemolytic episodes
- smear: BITE CELLS & HEINZ BODIES
other labs to considerer in microcytic anemia?
ferritin, iron studies, stool guiacs, hemoglobin electrophoresis
other labs to consider in normocytic anemia?
creatinine, erythropoietin, ESR
other labs to consider in macrocytic anemia?
B12, folate, methlmalonic acid, homocysteine
other labs to consider with hemolysis?
hepatoglobin, bilirubin, LDH, direct antiglobulin test (coomb’s) smear
