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49 Cards in this Set

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What does the peripheral smear in iron deficient anemia look like?
Hypochromic microcytic RBCs
Later: anisocytosis, poikilocytosis
In what type of anemia do you see a low MCV?
Iron deficiency, Thalassemia, Anemia of chronic dx (normal or slightly low)
First line of tx for iron deficient anemia? How would you tx refractory?
Ferrous Sulfate PO. Tx refractory with perenteral iron
When do you see a low retic count?
Iron deficient anemia, B12 deficiency
What test could be helpful in distinguishing between iron deficiency anemia and Thalassemia?
RDW increased in early stages of iron deficiency
What are some causes of B12 deficiency?
Inadequate dietary intake - rare, malabsorption (Crohn's, surgical), decreased production of intrinsic factor (Pernicious anemia), atrophic gastritis
What does the peripheral smear look like in B12deficiencies?
MAcrocytic magaloblastic anemia, anisocytosis, poikilcytosis, hypersegmented neutrophils
What test is more sensitive than serum for detecting a B12 deficiency?
Methylmalonic
What anemias have elevated MCVs?
B12, folic acid
Is what type of anemia do you see neurological symptoms like paresthesias, ataxia, decreased position sense, paranoid ideations "megaloblastic madness" ?
B12
What is glossitis and beefy red tongue a sign of?
B12
What is the epi of folic acid deficiency?
Elderly, pregnant, alcoholics, chronic disease states (CKD)
What drugs can induce a folic acid deficiency?
phenytoin, sulfasalazine, TMP-SMX, methotraxate
How can you distinguish between B12 and folic acid?
Pts will not exhibit the neurological abnormalities seen in B12. Serum B 12 will be normal Methylmalonic acid will be normal. RBC folate level low.
Eti of Anemia of chronic Dz?
Chronic Kidney Dz (reduced erythropoietin), Cancer, liver Dz, chronic inflammation or infection, clinical features are consistent with causative Dz
How to distinguish iron deficient anemia from anemia of chronic dz?
Ferritin will be normal or increased in anemia of chronic Dz.

Tsat is also extremely low, which isn't mentioned in iron deficiency
Tx for anemia of chronic Dz?
Purified recombinant erythropoietin IM or IV(Procrit, epogen, aranesp)

as well as iron, folate and B12 supplements

Try to maintain hemoglobin ~11-12
What are the intrinsic types of hemolytic anemias?
hereditary spherocytosis, G6PD deficiency, sickle cell anemia, thalassemia
What are extrinsic hemolytic anemias?
immune or microangiopathic hemolytic anemias
What labs are common in all types of hemolytic anemia?
Decreased haptoglobin,
Decreased hemoglobin,
Increased indirect/unconjugated bilirubin, hallmark is elevated retic count in the presence of stable or falling HgB/Hct
What is elevated retic count in the presence of stable or falling Hgb/Hct hallmark of?
Hemolytic anemias
What is the cause of hereditary spherocytosis?
autosomal dominant dz. Causes abnormal amount of specific protein. Spectrin, actin and other RBC proteins provide the scaffolding for the RBC membrane. This results in spherically shaped RBC that is less deformable than normal. Hemolysis occurs due to trapped RBCs within the spleen.
What is the MC type of hereditary hemolytic anemia?
Hereditary Spherocytosis
What is the CP of hereditary spherocytosis?
+/- S/S of anemia, palpable spleen, icterus, gallstones
What do these labs indicate: anemia of varying severity, reticulocytosis, MICROCYTOSIS, INCREASED MCHC, increased indirect bilirubin, increased RDW, (-) coombs test and osmotic fragility testing is done, but ektacytometry is more reliable?
Hereditary spherocytosis
Tx for hereditary spherocytosis?
Folic acid 1mg/d, splenectomy - only eliminates site of hemolysis
What causes episodic hemolytic anemia?
G6Pd deficiency. This hereditary enzyme defect characterized by decreased ability of RBC to deal with oxidative stress.
What is the epi of G6PD?
X linked recessive disorder
AA males
What triggers G6PD deficiency?
Most its are asymptomatic. Hemolysis triggered by: drugs, infection, diabetic ketoacidosis, exposure to fava beans
What are drugs to avoid if you have aG6Pd deficiency?
Aspirin, dapsone, antimalarials (quinine, primaquine, dapsone), quinidine, procainamide, sulfa drugs, nitrofurantoin, fava beans, red wine, soy
Lab findings of G6PD deficiency?
BEtween hemolytic episodes blood appears normal. During episodes: increased reticulocyte count, increased indirect bilirubin, peripheral smear shows, "BITE" or "BLISTER" cells
Tx of G6PD deficiency?
Avoidance oxidant drugs and substances
What causes sickle cell anemia?
autosomal recessive disorder of Hgb synthesis leading to chronic hemolytic anemia. Due to structural change, Hgb S is unstable and polymerizes in response to various stressors (hypoxia, acidosis)
What are the two types of sickle cell crisis?
Vascular - occlusion of sickled cells in small arteries and veins. Leads to pain, ischemia and infarction of tissue supplied. Spontaneous or provoked by infection, dehydration or hypoxia.

Hemolytic- due to splenic sequestration of RBCs
In this disorder, in children you see short stature, delayed puberty. Bony abnormalities (necrosis of metacarpals and metatarsals, poorly healing ulcers over the lower tibia. Hepatomegaly, jaundice, gallstone, retinal vessel obstruction, CVA, Tachycardia, acute chest syndrome (fever, chest pain, increasing WBC, pulmonary infiltrates), infection
sickle cell
How do you dx sickle cell?
Hgb electrophoresis - indicated for evaluation for sickle cell, thalassemia
Tx for sickle cell?
-Folic Acid, prevent crisis, transfusions, pneumococcal vaccine, pen rophylaxis, stem cell
What does the peripheral smear look like for sickle cell?
target, sickle and howell jolly bodies
What is the path of aplastic anemia?
Bone marrow failure that arises from injury to or abnormal expression of meatopietic stem cell. Bone marrow becomes hypo plastic and pancytopenia
What is the MC cause of aplastic anemia?
Autoimmune, radiation chemo
Pe reveals, pallor, purport, petechiae, NO hepatosplenomegaly, lymphadenopathy or bone tenderness
aplastic anemia
Pancytopenia is hallmark of what disease? Always associated with reticulocytopenia. Hypocellular in bone marrow.
Aplastic anemia
Tx of aplastic anemia?
RBC and platelet transfusions as necessary if mild, immunosuppression, allogenic hematopoietic stem cell transplant

fatal if untreated
What is thalassemia caused by?
Hereditary condition that affects the synthesis of adult hgb tetramer
What is the most clinically severe form of thalassemia?
Thalassemia Major, deficiency of B chain synthesis results in accumulation of a chain, aggregates and forms insoluble inclusions in bone marrow erythroid precursors causing destruction of 80% of erythroblasts
Epi of alpha thalassemia? types?
Asians
Silent carrier: 1 gene deleted
Minor/trait_ 2 genes deleted - mild microcytic anemia
Major- 3 genes deleted, chronic anemia, pallow, splenogamaegly

Hydrops fetalis: 4 genes deleted - stillborn, abnormal accumulation of fluid in 2 or more fetal compartments (ascites, pleural effusion)
Epi B thalassemia? types
mediterranean

Minor-asymp
Intermediate- chronic hemolytic anemia, hepatosplenomegaly, bone deformities

Major: at 6moa, severe anemia, growth failure, bony deformities, hepatosplenomegaly, jaundice
Lab finidings in thalassemia? Hgb? MCV? RBC? Peripheral? Bone marrow? What else?
low hub, low MCV, Norm or high RBC, peripheral smear (hypo chromic microcytic anemia, target cells, acanthocytes), hyper cellular with erythroblastic hyperplasia, Hgb electrophoresis
Tx thalassemia?
mild forms - just ID, a thalassemia major or hgb H - folic acid, avoid iron supplements and oxidative drugs

Thalassemia Major - allogenic bone marrow transplant is tx of choice, regular bone transfusions (chelation therapy, folic acid), splectomy