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132 Cards in this Set

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What are the different classifications of anemias?
Microcytic
Normocytic
Macrocytic
Microcytic
Normocytic
Macrocytic
What is the definition of a microcytic anemia?
MCV < 80 fL
What is the definition of a normocytic anemia?
MCV<80-100 fL
What is the definition of a macrocytic anemia?
MCV>100
What are the different microcytic anemias?
Iron deficiency
ACD
Thalassemias
Lead poisoning
Sideroblastic anemia
Iron deficiency
ACD
Thalassemias
Lead poisoning
Sideroblastic anemia
What are the different classes of normocytic anemias?
Hemolytic
Nonhemolytic
Hemolytic
Nonhemolytic
What are the different classes of hemolytic anemias?
Intrinsic
Extrinsic
Intrinsic
Extrinsic
What are the different nonhemolytic normocytic anemias?
ACD
Aplastic anemia
Chronic kidney disease
What are the intrinsic hemolytic anemias?
RBC membrane defects: hereditary spherocytosis
RBC enzyme deficiency
HbC
Sickle cell anemia
Paroxysmal nocturnal hemoglobinuria
RBC membrane defects: hereditary spherocytosis
RBC enzyme deficiency
HbC
Sickle cell anemia
Paroxysmal nocturnal hemoglobinuria
What are the extrinsic hemolytic anemias?
Autoimmune
Microangiopathic
Macroangiopathic
Infections
Autoimmune
Microangiopathic
Macroangiopathic
Infections
What are the different calsses of macrocytic anemia?
Megaloblastic
Non-megaloblastic
What are the megaloblastic, macrocytic anemias?
Folate deficiency
B12 deficiency
Orotic aciduria
Folate deficiency
B12 deficiency
Orotic aciduria
What are the non-megaloblastic, macrocytic anemias?
Liver disease
Alcoholism
Reticulocytosis
Liver disease
Alcoholism
Reticulocytosis
What's the cause of iron deficiency anemia?
Decreased iron due to chronic bleeding: (GI loss; menorrhagia)
Malnutrition/absorption disorders
Increased demands
What is the mechanism of iron deficiency anemia?
No reagents for the final step of heme synthesis.
What are the findings in iron deficiency anemia?
Decreased iron
increase in TIBC
Decreased ferritin
What are the histologic findings in iron deficiency anemia?
Microcytosis
Hypochromia
What are the symptoms of Plummer Vinson syndrome?
Anemia
Esophageal webs
Atrophic glossitis
Anemia
Esophageal webs
Atrophic glossitis
What is defective in alpha-thalassemia?
Defect in the alpha globin gene: lack of synthesis
What kind of deletion is present in the Asian alpha thallasemias?
Cis deletion
What kind of deletion is present in the African alpha thallasemias?
Trans deletion
What are the different kinds of the alpha-thallasemias?
4 gene deletion: hydrops fetalis
3 gene deletion: HbH disease
1-2 genes: no anemia
What kinds of hemoglobin form if you have a 4 gene deletion of the alpha genes?
Gamma4 (Hb Barts)
What problem underlies the beta thallasemias?
Point mutations in the splice sits and promoter sequences of the beta-globin gene, leading to decreased synthesis.
What populations have a high incidence of thalassemias?
Mediterranean population
What is the genotype in beta-thallasemia minor?
Heterozygote
What are the symptoms of beta-thalassemia minor?
Asymptomatic
How do you diagnose the thalassemias?
Hb electrophoresis
What indicates a diagnosis of beta-thalassemia minor on Hb electrophoresis?
Increased HbA2 (>3.5%)
What is the phenotype of beta thalassemia major?
Homozygote
What is the therapy for beta-thalassemia major?
Chronic blood transfusion
What is the appearance of beta-thalassemia major on an xray?
Marrow expation-->deformities

"Chipmunk facies"
What types of hemoglobin are found in beta-thalassemia major?
Increased HbF (alpha2, gamma2)
What type of beta-thalassemia is a hybrid disease?
HbS/Beta-thalassemia heterozygote
What is the cause of toxicity with lead poisoning?
Lead inhibits the ferrochelatase and ALA dehydratase-->decreased heme synthesis

Inhibits rRNA degradation
What is the impact on lead inhibiting rRNA breakdown in blood cells?
Basophilic strippling
Basophilic strippling
What are the findings of lead poisoning?
Lead Lines on gingivae (and metaphyses of long bones on xray)
Encephalopathy
Abdominal colic
Drops (wrist and foot)
What are the histologic findings of lead poisoning?
Erythrocyte basophilic strippling
Sideroblastic anemia
What's the treatment for lead poisoning?
Dimercaprol
EDTA

Succimer for chelation
What is the purpose of succimer?
Chelate lead that a person has ingested.
Generally, what is the cause of sideroblastic anemia?
Defects in heme synthesis
What's a hereditary cause of sideroblastic anemia?
X-linked defect in the delta-ALA synthase gene
What are some reversible causes of sideroblastic anemia?
Alcohol
Lead
Isoniazid
What are histologic findings in sideroblastic anemia?
Ringed sideroblasts (with iron laden macrophages)
What are the lab findings in sideroblastic anemia?
Increased iron
Normal TIBC
Increased ferritin
What's the treatment for sideroblastic anemia?
Pyridoxine (B6; a cofactor for ALA synthetase)
Generally, what is the cause of megaloblastic anemia?
Impaired DNA synthesis leads to a delay in the maturation of the nucleus relative to the maturation of the cytoplasm
What are causes of folate deficiency?
Malnutrition
Malabsorption
Antifolates
Increased requirements
What are some causes that place an increased folated burden on the body?
Pregnancy
Hemolytic anemia
What are the findigns in folate deficiency?
Hypersegmented neutrophils
Glossitis
Decreased folate
Increased homocystine
Normal methylmalonic acid
What are the causes of B12 deficiency?
Insufficient intake (vegans!)
Malabsorption
Pernicious anemia
Diphyllobohrium latum
PPIs
What are the findings in B12 deficiency anemia?
Hypersegmented neutrophils
Glossitis
Decreased B12 levels
Increased homocystine
Increased methylmalonic acid
NEUROLOGIC SYMPTOMS
What are the neurologic symptoms in B12 deficiency?
Peripheral neuropathy with sensorimotor dysfunction
Posterior column problems (vibration/proprioception)
Lateral corticalspinal problems (spasticity)
Dementia
If there are problems with the posterior columns, what senses will be impaired?
Vibration
Proprioception
What is the cause of ortic aciduria?
Genetic mutation in the enzyme that synthesizes uridine from orotic acid
What's the presentation of orotic aciduria?
Megaloblastic anemia in kids that can't be cured with folate or B12
What are the findings in orotic aciduria?
Hypersegmented neutrophils
Glossitis
Orotic acid in the urine
What's the treatment for orotic aciduria?
Uridine monophasphate to bypass the mutated enzyme
What is the pathophysiology of the nonmegaloblastic macrocytic anemias?
Macrocytic anemia in which DNA synthesis is unimpaired
What are the causes of nonmegaloblastic macrocytic anemias?
Liver disease
Alcoholism
Regiculocytosis
Drugs
What are some drugs that can cause nonmegaloblastic macrocytic anemia?
5-FU
AZT
Hydroxyurea
What is the classification of the normocytic, normochromic anemias?
Hemolytic vs. nonhemolytic
How can you divide the hemolytic anemia/
Cause of the hemolysis: intrinsic vs. extrisic

Location of the hemolysis: intravascular vs. extravascular
What are some findings in intravascular hemolysis?
Decreased haptoglobin
Increased LDH
Hb in the urine
What are some findings in extravascular hemolysis?
Increased LDH
Increased UCB
Jaundice
What are the causes of nonhemolytic, normocytic anemia?
Anemia of chronic disease
Aplastic anemia
Chronic kidney disease
What is the mechanism of anemia development secondary to chronic disease?
1. Inflammation
2. Increased hepcidin release
3. Decreased release of iron from the macrophages
4. Anemia.
What is the action of hepcidin in the body?
Binds ferroportin on the intestinal mucosa-->decreased uptake

Binds ferroportin on macrophages --> decreased release

Overall, decreases body iron supply
Where is hepcidin released from?
Liver
What are the findings in anemia of chronic disease?
Deceased iron
Decreed TIBC
Increased ferritin
What are causes of aplastic anemia?
Radiation and drugs
Viruses
Fanconi's anemia
Idiopathic
What are some drugs that can cause aplastic anemia?
Benzen
Chloramphenicol
Alkylating agents
Antimetabolites
What are some viral agents that can cause aplastic anemia?
Parvovirus B19
EBV
HIV
HCV
What is defective in Fanconi's anemia?
DNA repair
What type of cell is destroyed during aplastic anemia?
Stem cells
What are the findings in aplastic anemia?
Pancytopenia
Hypocellular bone marrow wiith fatty infiltration
What are the symptoms of aplastic anemia?
Fatigue
Malaise
Pallor
Purpura
Mucosal bleeding
Petechiae
Infection
What's the treatment of aplastic anemia?
Withdraw the offending agent
Immunosuppression
Allogenic bone marrow transplation
RBC/platelet transfucsion
G-CSF
GM-CSF
What is the cause of nonmegaloblastic macrocytic anemia secondary to chronic kidney disease?
Decreased erythropoietin --> decreased hematopoiesis
What are the intrinsic hemolytic normocytic anemias?
Hereditary spherocytosis
G6PD deficiency
Pyruvate kinase deficiency
HbC sdefect
Paroxysmal nocturnal hemoglobinuria
Sickle cell anemia
Which of the intrinsic hemolytic normocytic anemias are intravascular?
G6PD deficiency
Paroxysmal nocturnal hemoglobinuria
Which of the intrinsic hemolytic normocytic anemias are extravascular?
Hereditary spherocytosis
G6PD deficiency
Pyruvate kinase deficiency
HbC defect
Sickle cell anemia
What proteins are defective in hereditary spherocytosis?
Defect in proteins interacting with the RBC membrane skeleton and the plasma membrane:
-Ankyrin
-Band 3
-Protein 4.2
-Spectrin
What is the appearance of RBCs in hereditary spherocytosis?
Small, round RBCs with no central pallor
Small, round RBCs with no central pallor
What occurs to spherocytes in the body?
Premature removal by the spleen
What are findings in hereditary spherocytosis?
Splenomegaly
Aplastic criss
What are the lab findings in hereditary spherocytosis?
Positive osmotic fragility tests
Normal to decreased MCV
Increased MCHC
What's the treatment for hereditary spherocytosis?
Splenectomy
What's the inheritance of G6PD deficiency?
X-linked
How does RBC damage occur in G6PD deficiency?
1. Defect in G6PD
2. Decreased glutathione
3. Increased RBC susceptibility to oxidant stress
What are some oxidant stresses that can exacerbate problems in G6PD deficiency?
Sulfa drugs
Infections
Fava beans
What are the labs in G6PD deficiency?
Smear:
Heinz bodies
Bite cells
Smear:
Heinz bodies
Bite cells
What's the inhteritance of pyruvate kinase deficinecy
Autosomal recessive
What is the effect of a lack of pyruvate kinase on RBCs?
Decreased ATP-->rigid RBCs
What is the presentation of pyruvate kinase deficiency?
Hemolytic anemia in a newborn
What is the cause of HbC defect?
Glutamic acid-->lysine mutation at position 6 in beta globin
What's the cause of paroxysmal nocturnal hemoglobinuria?
incrased complement mediated RBC lysis due to impaired synthesis of a GPI anchor that protects the RBC membrane from complement
What is the triad in PNH?
Hemolytic anemia
Pancytopenia
Venous thrombosis
What are the labs in paroxysmal nocturnal hemoglobinuria?
CD55/59 negative labs on flow cytometry
What is the treatment for paroxysmal nocturnal hemoglobinuria?
Eculizumab
What hemoglobin is mutated in sickle cell anemia?
Hemoglobin beta
What is the mutation that takes place in sickle cell anemia?
Substitution of a glutamic acid with valine at position 6 in the beta globin gene
How does pathology result from sickle cell disease?
Low O2 or dehydration causes sickling of the hemoglobin within the cells, leading to anemia and vaso-occlusive disease
What can cause HbS to sickle?
Deoxygenation
Dehydration
When does sickle cell disease present?
A couple of months after birth; due to the fact that there's still HbF around
Why does HbS stick around in the population?
Heterozygotes are resistant to malaria
What ethnicity has the Hbs trait?
African Americans: 8% carry the disease
What is the appearance of sickle cells?
Crescent shaped RBCs
Crescent shaped RBCs
What is a cause of an Xray like this? Why?
What is a cause of an Xray like this? Why?
Beta-thalassemia major
Sickle cell disease

Due to increased erythropoiesis
What are complication in sickle cell homozygotes?
Aplastic crisis
Autosplenectomy
Splenic sequestration crisis
Salmonella osteomyelitis
Pinful crisis!
Renal papillary necrosis
What kinds of infections are people with sickle cell more vulnerable to?
Encapsulated organisms:

S. pneumoniae
H. influenzae type B
N. meningitidis
What's the treatment for sickle-cell anemia?
hydroxyurea
Bone marrow transplantation
What are the extrinsic hemolytic normocytic anemias?
Autoimmune hemolytic anemia
Microangiopathic anemia
Macroangiopathic anemia
Infections
What are the different types of autoimmune hemolytic anemias?
Warm agglutinin
Cold agglutinin
What type of immune globulin is implicated in warm agglutinin hemolytic anemia?
IgG
What type of immune globulin is implicated in cold agglutinin hemolytic anemia?
igM
What diseases are associated with warm agglutinin anemia?
SLE
CLL
Drugs
What are conditions associated with cold agglutinin anemia?
CLL
Mycoplasma pneumonia
Infectious mononucleosis
What are the different tests that you can run in autoimmune hemolytic anemias?
Direct coombs test
Indirect Coombs test
What do you do in a direct coombs test?
Anti-Ig antibody is added to the patient's serum

RBCs agglutinate if RBCs are coated with Ig
What is performed in an indirect Coombs test?
Normal RBCs added to patient's serum agglutinate if serum has anti-RBC surface Ig
What is the pathogenesis of microangiopathic anemia?
RBCs are damaged when passing through obstructed or narrowed vessel lumina
What conditions cause a microangiopathic anemia?
DIC
TTP-HUS
SLE
Malignant hypertension
What are the findings in a microangiopathic anemia?
Schistocytes (helmet cells)
Schistocytes (helmet cells)
What are causes of macroangiopathic anemia?
Prosthetic heart valves
Aortic stenosis
What are findings in macroangiopathic anemia?
Schistocytes on a peripheral blood smear
Schistocytes on a peripheral blood smear
What types of infections can cause anemia?
Malaria
Babesia
What are the the following lab findings in iron deficiency:
Serum iron?
Transferrin?
Ferritin?
Transferrin saturation?
Serum iron: decreased
Transferrin: increased
Ferritin: decreased
Transferrin saturation: markedly decreased
What are the the following lab findings in chronic disease:
Serum iron?
Transferrin?
Ferritin?
Transferrin saturation?
Serum iron: decreased
Transferrin: decreased
Ferritin: increased
Transferrin saturation: unchanged
What are the the following lab findings in hemochromatosis:
Serum iron?
Transferrin?
Ferritin?
Transferrin saturation?
Serum iron: increased
Transferrin: decreased
Ferritin: increased
Transferrin saturation: markedly increased
What are the the following lab findings in pregnancy/OCP use:
Serum iron?
Transferrin?
Ferritin?
Transferrin saturation?
Serum iron: no change
Transferrin: increased
Ferritin:
Transferrin saturation: decrased
What's the effect of pregnancy on transferrin production?
Increased