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74 Cards in this Set
- Front
- Back
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Normal Hgb at birth:
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13.5-24
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Normal MCV at birth:
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95-121
Drops to 78-98 by adulthood |
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Do teenage boys or girls have a higher Hgb?
Why? |
Teenage boys have a higher Hgb (13-16 vs 12-16)
This is because of the effect of testosterone, NOT due to bleeidng in anemia |
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What would you expect to happen to the RDW in iron deficiency anemia?
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Should increase
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Are there any major / common conditions where the RDW is decreased?
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Not really
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What would you expect to happen to the RDW in lead poisoning?
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Nothing
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Would you expect an increased or decreased reticulocyte count in folate deficiency / pernicious anemia?
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Decreased reticulocyte
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What is the key defect that underlies microcytic anemia?
(Can you guess what I'm thinking?) |
Abnormal hemoglobin synthesis
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What is the key defect that underlies macrocytic anemia?
(Can you guess what I'm thinking?) |
Abnormality in the maturity of RBCs
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What is physioligc anemia of infancy?
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Newborns usually have a very high Hgb. After birth this drops drammatically (down 11) as volume exands, and fetal blood RBCs.
This is completely normal! Just ensure baby gets essential nutrients |
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What is a target cell? What causes it?
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Concentric circles due to increase in membrane to Hgb ratio.
Seen in hemoglobinopathies, thalassemia |
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What is a dacrocyte? What causes it?
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Teardrop shaped RBCs due to extramedullary hematopoiesis
Can be seen in myelofibrosis |
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What is a schistocyte? What causes it?
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Cells with bitemarks. Happens with mechanical damage to RBCs
G6PD, DIC/HUS, vasculitis |
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What is a Heinz Body? What causes it?
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Round protuberance that deforms a cell caused by oxidized and denatured hemoglobin.
Can be caused by G6PD and oxidative chemicals |
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What is basophilic stippling? What causes it?
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Round, dark blue granules in the RBC caused by aggregation of ribosomes
Can be caused by sideroblatic anemia, MDS, lead poisoning |
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Erythroblastois of childhood is a disease whereby the body stops producing RBCs. It has been linked to infections.
If you encounter this, do you need to treat it? |
Depends on how bad the anemia. Typically production should resume in 30-60 days.
If not, must consider different diagnosis |
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A previously healthy 1 year old male with hx of cold 8 weeks ago shows up pale and cranky. H&H is 5/10, with 0% retics. Platelets are normal. What's the dx?
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Erythroblastosis of childhood
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What would you expect to happen to the reticulocyte count in iron deficiency anemia?
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Would expect to be low
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A 9 month old boy comes in with Hgb 7.5, and MCV 62. On further questioning the mom indicates that she is feeding the kid whole milk.
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Iron deficiency anemia
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Signs and symptoms of iron deficiency anemia:
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Usually sx of anemia (pallor, fatigue, irritability)
Angular stomatitis / glossitis Spoon nails Esophageal webs Splenomegaly |
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Would the ferritin be high or low in iron deficiency anemia?
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Low (<10)
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Would the TIBC be high or low in iron deficiency anemia?
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High
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What is the most common type of anemia in children?
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Iron deficiency anemia
Especially in children between 6 months and 3 years old |
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When do you screen for lead poisoning?
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Between 10 months and 2 years.
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What is the "antidote" to lead poisoning?
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Dimercaprol followed by EDTA or Succimer (DMSA)
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What would you expect to see in the blood smear of someone with lead poisoning?
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Microcytic hypochromic anemia with basophilic stippling
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Name 2 drugs that can lead to folate deficiency?
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Phenytoin, MTX
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Why is folic acid treatment contraindicated in someone with B12 deficiency.
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Because giving folic acid may "correct" the anemia, but the patient will still have neurological problems associated with B12 dficiency
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Signs and symptoms of B12 deficiency:
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Red/ beefy tongue, blue eyes, vitiligo, myxedema, weakness, iritability, anorexia, neurologic signs
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For B12 deficiency, do you use shots or PO?
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Shots (lucky children)
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What are the iron study findings you'd see in anemia of chronic disease?
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Low iron
Normal / low TIBC High ferritin |
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What is the key problem in hemolytic disease of the newborn?
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maternal sensitization to Rh / Kell / Duffy
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Will hemolytic anemia of the newborn lead to an increased conjugated bilirubin, unconjugated bilirubin, or both?
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Increased unconjugated bilirubin
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How could it be that hemolytic anemia of the newborn leads to increased bilirubin and subsequent jaundice only after delivery?
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Because the placenta can filter out a lot of the breakdown products. After delivery the placenta isn't there to protect baby
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Signs and symptoms of fetal hydrops (as seen in hemolytic disease of the newborn)
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Large placenta, jaundice, abduction of limbs with loss of flexion, scalp edema, cyanosis
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What's the difference between a direct and indirect Coomb's test?
Which will be elevated in hemolytic disease of newborn? |
Direct coombs looks at antibodies on the RBCs themselves, while indirect looks at antibodies on the serum
Direct will be elevated in hemolytic disease of newborn |
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Which type of thalassemia is fatal without regular transfusions?
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Beta major (Homozygous)
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Would you expect LDH to be high or low in thalassemias?
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High due to ineffective erythropoiesis
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What is hemosiderosis? Who gets it? What organs does it most affect?
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Iron overload disorder. Most often happens when a person requires many many transfusions throughout their life (as in thalassemia)
Can affect heart (cardiomyopathy), Liver (cirrhosis), and Pancreas (Diabetes) |
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What is fanconi syndrome?
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Wasting of phosphate, glucose, amino acids and bicarbonate by the proximal renal tubules
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What is the most common cause of fanconi syndrome?
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Cystinosis (Defect of cystine metabilism)
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What are some secondary causes fo fanconi syndrome?
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Chemotherapeutic agents
Heavy metals Gentamicin Expired tetracycline |
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Symptoms of fanconi syndrome:
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Growth retardation
Rickets Polyuria Anorexia Vomiting |
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Key underlying defect in Hemophilia A.
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Factor VIII
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Key deficiency in Hemophilia B
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Factor IX deficiency
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Inheritance of Hemophilia A & B
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Both are X-Linked recessive
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Inheritance of von Willebrand's Disease
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Autosomal Dominant
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What will the bleeding time be in a person with Hemophilia compared to someone without hemophilia?
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Bleeding time should be unchanged
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How will prothrombin time change in hemophilia? How about vWD?
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Will be unchanged in both
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Is this Hemophilia or vWD?
Bleeding from mucous membranes, skin |
Von Willebrand's
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Is this Hemophilia or vWD?
Reduced platelet aggregation |
Trick question!
Platelet aggregation is normal in both cases |
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What is DDAVP? How does it work?
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Desmopressin acetate = Synthetic vasopressin analogue that releases factor VIII from tissue stores.
*Does not affect factor IX levels |
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What is the major worry-some side effect of DDAVP?
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Hyponatremia 2/2 water retention
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What is the most common cause of death of older patients with hemophilia?
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HIV from blood transfusions in the late 1970's and 1980's
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What is the goal of treatment for hemophilia?
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Prevent long-term crippling orthopedic injuries 2/2 hemarthroses
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What is the key lab for diagnosing hemophilia?
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PTT
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What is the normal function of von Willebrand factor?
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Connects subendothelial collagen to platelets once platelets are activated. It also binds factor VIII
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What is vWF:Ag?
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A lab test to measure if antibodies stick to vWF (hence "Ag"). Would expect it to be low in von Willebrand Disease
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What is the mainstay of treatment of von Willebrand disease?
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DDAVP,
Second line agents include virally attenuated vWF concentrate, or cryoprecipitate. |
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Which coagulation factors are dependent on Vitamin K?
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II, VII, IX, X, protein C, protein S
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Causes of vitamin K deficiency:
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Cystic Fibrosis
Antibiotic suppression Coumedin Phenytoin Most common cause is being a newborn (especially if breast fed) |
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What are the abnormal labs that you would expect in vitamin K deficiency?
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Increased PT / PTT with normal fibrinogen, and fibrinogen degradation products
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How would you differentiate DIC from vitamin K deficiency on labs?
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In addition to increased PT (which you would see in both DIC and VIT K deficiency) you also see decreased platelets, decreased fibrinogen, and decreased fibrinogen degradation productions
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This is an X-linked disorder characterized by hypogammaglobulinemia, eczema, and thrombocytopenia:
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Wiskott-Aldrich syndrome
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What is the treatment for Wiskott-Aldrich syndrome?
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Bone marow transplant
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What is the most common cause of platelet destruction?
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Immune mediated
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What is neonatal isoimmune thrombocytopenic purpura?
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When maternal antibodies recognize fetal platelets as foreign and send antibodies to kill them (IgG antibodies)
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What is the key problem in microangiopathic hemolytic anemias?
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Intravascular destruction of red cells leading to depleation of platelets and clotting factors
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List 3 major diseases that lead to Microangiopathic hemolytic anemia:
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DIC
HUS TTP |
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Why does E.Coli H7:0157 lead to thrombocytopenia
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These bugs produce a verotoxin that binds to endothelial cells and damages them. The body then consumes platelets to fix the damage.
Microangiopathic hemolytic anemia then occurs 2/2 mechanical injury to red cells as they pass through the damaged endothelium |
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What is the key problem in TTP?
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Body forms intravascular blood clots in small vessels. This leads to an increase in sheer stress of red blood cells leading to their destruction (schistocytes)
Platelets are used up in the process of creating clots, and the clots themselves lead to end organ damage. |
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What percentage of cases of acute ITP resolve spontaneously within 6 months?
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80%
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At what platelet count would you start treatment with steroids, IVIG and platelet transfusion in a patient with ITP?
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<20,000
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Treatment of chronic ITP (lasting 6-12 months)
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IVIG and splenectomy
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