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Types of Anemia
Dr. Arya - October 18, 2011 Semester 3, Mini 2
Terms in this set (66)
What are some conditions where RBCs are broken down in the spleen?
Abnormal shape (HbS, spherocytes)
RBCs coated by IgG
What are some conditions where RBCs are broken down in the vasculature?
Enzyme deficiency (G6PD)
Complement destruction (via IgM)
What would appear on general examination of a hemolytic anemia patient?
(pigmented gall stones)
Why is serum haptoglobin levels low in intravascular hemolytic anemia?
Haptoglobin binds the free heme before it is lost in the urine. The Hb-Hp complex is then taken up by the reticuloendothelial system. thus lowering the serum haptogloben levels.
Is serum haptogloben levels low in extravascular hemolytic anemia?
The RBCs are primarily broken down in the spleen and splenic macrophages gobble up the Hb directly.
What are the four possible causes of microcytic, hypochromic anemia?
Anemia of Chronic Disease
What are the possible causes of normocytic, normochromic anemia?
Sickle Cell Anemia
Paroxysmal Nocturnal Hemoglobinuria (PNH)
Micro-Angiopathic Hemolytic Anemia
What is the cause and presenting symptoms of Hereditary Spherocytosis? Tx?
Mutation in ankyrin or spectrin gene
S/S: Jaundice, pigmented gallstones, splenomegaly
PBS and CBC shows low Hb, spherocytes, ↑ reticulocyte count, ↑ indirect bilirubun. What other tests could you order to help dx Hereditary Spherocytosis?
Osmotic fragility test = ↑
Coombs test = negative
Bone marrow = Erythroid hyperplasia
What is a severe complication of Hereditary Spherocytosis?
Aplastic crisis w/ parvovirus B19
(Spherocytes, sudden ↓ Hb, ↓ Reticulocytes)
What is the MCC of hemoglobinopathy in black Americans?
Sickle cell anemia
Why and when do RBCs sickle?
Why: HbS molecules aggregate and polymerize into long needle-like fibers
When: Conc.of HbS>60%
What factors increase sickling?
Why are sickle cell patients susceptible to recurrent infections w/ encapsulated bacteria?
What is the mutation that causes Sickle Cell Anemia?
Point mutation at codon 6
→ Glu to Val substitution
What lab tests would you order to further investigate sickle cell anemia? What would positive results look like?
Labs: Sickling test and Hb electrophoresis
(look for HbS)
PBS: Sickle cells, Howell-Jolly Bodies
CBC: ↑ reticulocyte count
What are the acute complications you can expect in a patient with sickle cell anemia?
What is suggestive of a sickle cell crisis?
Abdominal and bone/joint pain
Poor eyesight, blindness
When does sickle cell anemia start showing signs and symptoms?
about 6-9 months
(depletion of remnant HbF)
What does a sickle cell patient's spleen look like when they are young/old?
What happens if you have a sickle cell trait?
Hb electrophoresis: HbA 60%, HbS 40%
watch out for high altitudes though
What is the tx of sickle cell anemia?
What are the signs and symptoms of β Thalassemia major?
Microcytic, hypochromic target cells
Growth retardation, delayed sexual maturation
Skeletal changes (ex. enlarged maxilla)
Iron increased in BM exam
What are the signs and symptoms of α Thalassemia minor (trait)?
(Microcytic hypochromic; target cells)
What is a confirmatory tests for β thalassemia major?
How would you treat a β thalassemia major patient?
Bood transfusion → iron overload
→ Iron chelator (Deferoxamine)
A heme analysis of your patient shows mild microcytic anemia and 8% Hb Bart.
What type of anemia do you suspect they have?
How many α-chains are deleted in Hydrops fetalis and how long would this person live?
4 α-chain deletions
Lethal in utero (stillborn)
What types of disease show increased extra-medullary hematopoiesis?
Chronic hemolytic anemia
(thalassemia, sickle cell)
What PBS findings would you expect to see in Paroxysmal Nocturnal Hemoglobinuria?
normocytic RBCs w/ Pancytopenia
What is the mutation responsible for Paroxysmal Nocturnal Hemoglobinuria and what does it cause?
Mutation in PIGA that anchors GPI in WBCs, RBCs, platelets
CD59, CD55/DAF, can't be GPI-linked
→ less protection against complement system
A patient states that he has haemoglobinuria when he wakes up, but it clears up as the day goes on.
What do you think he has?
Paroxysmal Nocturnal Hemoglobinuria
→ respiratory acidosis enhances complement attachment
What is the leading cause of death in patients with Paroxysmal Nocturnal Hemoglobinuria?
due to the ↑ activation of platelets by complement
What test helps identify a CD55/CD59 deficiency?
(Acidified serum test)
A patient presents w/ sudden onset of back pain with hemoglobinuria 3 days after eating Favabeans. Current medication includes Dapsone. What do you expect to see on a PBS?
Heinz bodies (supravital stain)
A patient presents w/ sudden onset of back pain with jaundice and hemoglobinuria 2 days after eating Favabeans. Current medication includes Primaquine. What is your initial dx?
What tests would you do to confirm a G6PD deficiency?
RBC assay using fluorescence
and check G6PD protein level
What are the three types of immune hemolytic anemia?
What are some drugs that can cause hemolytic anemia?
Quinidine, Penicillin, Methyldopa
What would you find on a PBS w/ immune hemolytic anemia?
Does the warm or cold antibody fix complement?
Cold Agglutinin Type (IgM, 0°-4°C)
also Paroxysmal Cold Hemoglobinuria (IgG, <30°C)
A patient presents w/ jaundice, Raynaud phenomenon, and an ulcer on their finger tip. Coomb's test returns positive. What do you think they have?
Cold Agglutinin type hemolytic anemia
What are the two causes of spherocytes?
How would you differentiate them?
Hereditary spherocytosis and autoimmune
differentiate w/ Direct Coombs Test
What is the cause of macro-angio-pathic hemolytic anemia?
(cardiac valve prosthesis, aortic stenosis)
What is the cause of micro-angio-pathic hemolytic anemia
turbulent flow in veins
What can be the cause of schistocytes?
Why is PCV decreased in anemia due to acute blood loss?
shift of water from interstitial fluid compartment to intra-vascular compartment
A 5 year old patient presents w/ a fever and has a history of bleeding and fatigue. You do not find splenomegaly or hepatomegaly, but lab findings show a pancytopenia w/ low reticulocyte count. What disease would you suspect?
(sometimes called Fanconi anemia)
In what disease will you find tear drop cells?
What are some common causes of sideroblastic anemia?
35 y/o painter presents with abdominal colic w/ diarrhea, foot-drop. PBS shows microcytic, hypochromic RBCs w/ coarse basophilic stippling; Labs show increased ferritin, but decreased TIBC.
What is your dx?
5 year old patient presents with encephalopathy, growth retardation. X-ray reveals an area of intense radiopacity in the epiphysis of the femur and tibia.
What do you suspect?
→ sideroblastic anemia
What is another name for tear drop cells?
What is the only microcytic anemia that has a high TIBC?
Both sideroblastic anemia and thalassemia have high Fe, low TIBC, and high ferritin. How would you differentiate?
sideroblatic: basophilic stippling
thalassemia: target cells
What is the MCC of anemia in hospitalized patients in USA?
Anemia of chronic disease
What are some chronic diseases that can cause anemia?
TB, Lung abscess
What is the main pathogenesis in anemia of chronic disease?
Abnormality in transfer of iron out of storage
What are Howell-Jolly bodies are indicative of?
Splenectomy or splenic atrophy
(ex. Sickle cell anemia)
What are the two main causes of macrocytic anemia?
Folic Acid def.
What are some symptoms of B12 deficiency anemia?
↑ methyl-malonic acid
What is the Schilling Test for?
Test to determine if pernicious anemia is the cause of a patients B12 deficiency.
What type of anemia is Plummer Winson syndrome indicative of?
Koilonychia is indicative of what type of anemia?
Patient presents with chipmunk facies, jaundice, and splenomegaly. Before doing other tests, what disease do you suspect?
What are possible causes of hypocellular bone marrow?
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