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Hematology/Oncology FirstAid > Anemias > Flashcards

Anemias Flashcards

1
Q

Iron deficiency

A

Microcytic anemia (MCV incr final step in heme synthesis.

  • Decr iron, incr TIBC, decr ferritin. Fatigue, conjunctival pallor.
  • Microcytosis and hypochromia. May manifest as Plummer-Vinson syndrome (triad of iron deficiency anemia, esophageal webs, and atrophic glossitis).
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2
Q

α-thalassemia

A

Microcytic anemia (MCV decr α-globin synthesis.

  • cis deletion prevalent in Asian populations; trans deletion prevalent in African populations.
  • 4 allele deletion: No α-globin. Excess γ-globin forms γ4 (Hb Barts). Incompatible with life (causes hydrops fetalis).
  • 3 allele deletion: HbH disease. Very little α-globin. Excess β-globin forms β4 (HbH).
  • 1–2 allele deletion: no clinically significant anemia.
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3
Q

β-thalassemia

A

Microcytic anemia (MCV decr β-globin synthesis. Prevalent in Mediterranean populations.

HbS/β-thalassemia heterozygote: mild to moderate sickle cell disease depending on amount of β-globin production.

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4
Q

β-thalassemia minor

A

Microcytic anemia (MCV 3.5%) on electrophoresis.

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5
Q

β-thalassemia major

A

Microcytic anemia (MCV severe anemia requiring blood transfusion (2° hemochromatosis).

  • Marrow expansion (“crew cut” on skull x-ray) –> skeletal deformities. “Chipmunk” facies.
  • Extramedullary hematopoiesis (leads to hepatosplenomegaly). Incr risk of parvovirus B19- induced aplastic crisis.
  • Major –> incr HbF (α2γ2). HbF is protective in the infant and disease only becomes symptomatic after 6 months.
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6
Q

Lead poisoning

A

Microcytic anemia (MCV decr heme synthesis and incr RBC protoporphyrin.

  • Also inhibits rRNA degradation, causing RBCs to retain aggregates of rRNA (basophilic stippling).
  • High risk in old houses with chipped paint

LEAD:

  • Lead Lines on gingivae (Burton lines) and on metaphyses of long bones D on x-ray.
  • Encephalopathy and Erythrocyte basophilic stippling.
  • Abdominal colic and sideroblastic Anemia.
  • Drops—wrist and foot drop. Dimercaprol and EDTA are 1st line of treatment.
  • Succimer used for chelation for kids (It “sucks” to be a kid who eats lead).
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7
Q

Sideroblastic anemia

A

Microcytic anemia (MCV

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8
Q

Megaloblastic anemia

A 
Macrocytic anemia (MCV > 100 fL).
Impaired DNA synthesis--> maturation of nucleus of precursor cells in bone marrow delayed relative to maturation of cytoplasm. 
Abnormal cell division--> pancytopenia.
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9
Q

Folate deficiency

A 
Macrocytic anemia (MCV > 100 fL).
Causes: malnutrition (e.g., alcoholics), malabsorption, antifolates (e.g., methotrexate, trimethoprim, phenytoin), incr requirement (e.g., hemolytic anemia, pregnancy). Hypersegmented neutrophils, glossitis, decr folate, incr homocysteine but normal methylmalonic acid. No neurologic symptoms (distinguishes from B12 deficiency).
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10
Q

B12 deficiency (cobalamin)

A

Macrocytic anemia (MCV > 100 fL)
Causes: insufficient intake (e.g., strict vegans), malabsorption (e.g., Crohn disease), pernicious anemia, Diphyllobothrium latum (fish tapeworm), proton pump inhibitors.
Hypersegmented neutrophils, glossitis, decr B12, incr homocysteine, incr methylmalonic acid.
Neurologic symptoms: subacute combined degeneration (due to involvement of B12 in fatty acid pathways and myelin synthesis):
-Peripheral neuropathy with sensorimotor dysfunction
-Dorsal columns (vibration/proprioception)
-Lateral corticospinal (spasticity)
-Dementia

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11
Q

Orotic aciduria

A 
Macrocytic anemia (MCV > 100 fL).
Inability to convert orotic acid to UMP (de novo pyrimidine synthesis pathway) because of defect in UMP synthase. Autosomal recessive. Presents in children as megaloblastic anemia that cannot be cured by folate or B12 with
failure to thrive. No hyperammonemia (vs. ornithine transcarbamylase deficiency— incr orotic acid with hyperammonemia).

Hypersegmented neutrophils, glossitis, orotic acid in urine.
Treatment: uridine monophosphate to bypass mutated enzyme.

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12
Q

sadf

A

Macrocytic anemia (MCV > 100 fL).

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13
Q

Nonmegaloblastic macrocytic anemias

A 
Macrocytic anemia (MCV > 100 fL).
Macrocytic anemia in which DNA synthesis is unimpaired.
Causes: liver disease; alcoholism; reticulocytosis --> incr MCV; drugs (5-FU, zidovudine, hydroxyurea). 
Macrocytosis and bone marrow suppression can occur in the absence of folate/B12 deficiency.
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14
Q

Normocytic, normochromic anemia

A

Normocytic, normochromic anemias are classified as nonhemolytic or hemolytic. The hemolytic anemias are further classified according to the cause of the hemolysis (intrinsic vs. extrinsic to the RBC) and by the location of the hemolysis (intravascular vs. extravascular).

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15
Q

Intravascular hemolysis

A

Normocytic, normochromic anemia
Findings: decr haptoglobin, incr LDH, schistocytes and incr reticulocytes on peripheral blood smear; and urobilinogen in urine (e.g., paroxysmal nocturnal hemoglobinuria, mechanical destruction [aortic stenosis, prosthetic valve], microangiopathic hemolytic anemias).

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16
Q

Extravascular hemolysis

A

Normocytic, normochromic anemia
Findings: macrophage in spleen clears RBC. Spherocytes in peripheral smear, incr LDH plus incr unconjugated bilirubin, which causes jaundice (e.g., hereditary spherocytosis).

17
Q

Anemia of chronic disease

A 
Nonhemolytic, normocytic anemia
Inflammation --> incr hepcidin (released by liver, binds ferroportin on intestinal mucosal cells and macrophages, thus inhibiting iron transport) --> decr release of iron from
macrophages.
-decr iron, decr TIBC, incr ferritin.
-Can become microcytic, hypochromic
18
Q

Aplastic anemia

A

Nonhemolytic, normocytic anemia
Caused by failure or destruction of myeloid stem cells due to:
-Radiation and drugs (benzene, chloramphenicol, alkylating agents, antimetabolites)
-Viral agents (parvovirus B19, EBV, HIV, HCV)
-Fanconi anemia (DNA repair defect)
-Idiopathic (immune mediated, 1° stem cell defect); may follow acute hepatitis
-Pancytopenia characterized by severe anemia, leukopenia, and thrombocytopenia. Normal cell morphology, but hypo cellular bone marrow with fatty infiltration (dry bone marrow tap).
-Symptoms: fatigue, malaise, pallor, purpura, mucosal bleeding, petechiae, infection.
-Treatment: withdrawal of offending agent, immunosuppressive regimens (antithymocyte globulin, cyclosporine), allogeneic bone marrow transplantation, RBC and platelet transfusion, G-CSF, or GM-CSF.

19
Q

Chronic kidney disease

A

Nonhemolytic, normocytic anemia

decr EPO–> decr hematopoiesis.

20
Q

Hereditary spherocytosis (extravascular)

A
  • Intrinsic hemolytic normocytic anemia
  • Defect in proteins interacting with RBC membrane skeleton and plasma membrane (e.g., ankyrin, band 3, protein 4.2, spectrin).
  • Less membrane causes small and round RBCs with no central pallor (incr MCHC, incr red cell distribution width) –> premature removal of RBCs by spleen.
  • Splenomegaly, aplastic crisis (parvovirus B19 infection).
  • Labs: osmotic fragility test+. Eosin-5- maleimide binding test useful for screening. Normal to decr MCV with abundance of cells; masks microcytia.
  • Treatment: splenectomy.
21
Q

G6PD deficiency (extravascular/intravascular)

A
  • Intrinsic hemolytic normocytic anemia
  • Most common enzymatic disorder of RBCs.
  • X-linked recessive.
  • Defect in G6PD–> decr glutathione–> incr RBC susceptibility to oxidant stress. Hemolytic anemia following oxidant stress (classic causes: sulfa drugs, antimalarials, infections, fava beans).
  • Back pain, hemoglobinuria a few days after oxidant stress.
  • Labs: blood smear shows RBCs with Heinz bodies and bite cells.
  • “Stress makes me eat bites of fava beans with Heinz ketchup.”
22
Q

Pyruvate kinase deficiency (extravascular)

A
  • Intrinsic hemolytic normocytic anemia
  • Autosomal recessive.
  • Defect in pyruvate kinase–> decr ATP–> rigid RBCs.
  • Hemolytic anemia in a newborn.
23
Q

HbC defect (extravascular)

A
  • Intrinsic hemolytic normocytic anemia
  • Glutamic acid-to-lysine mutation at residue 6 in β-globin.
  • Patients with HbSC (1 of each mutant gene) have milder disease than have HbSS patients.
24
Q

Paroxysmal nocturnal hemoglobinuria (Intravascular)

A

-Intrinsic hemolytic normocytic anemia
-Incr complement-mediated RBC lysis (impaired synthesis of GPI anchor for decay-accelerating factor that protects RBC membrane from complement). Acquired mutation in a
hematopoietic stem cell. Increased incidence of acute leukemias.
-Triad: Coombs “ hemolytic anemia, pancytopenia, and venous thrombosis.
-Labs: CD55/59 “ RBCs on flow cytometry.
-Treatment: eculizumab.

25
Q

Sickle cell anemia (extravascular)

A

-Intrinsic hemolytic normocytic anemia
-HbS point mutation causes a single amino acid replacement in β chain (substitution of glutamic acid with valine) at position 6.
-Pathogenesis: low O2, dehydration, or acidosis precipitates sickling (deoxygenated HbS polymerizes), which results in anemia and vaso-occlusive disease.
-Newborns are initially asymptomatic because of
incr HbF and decr HbS. Heterozygotes (sickle cell trait) have resistance to malaria.
-8% of African Americans carry the HbS trait.

-Sickled cells are crescent-shaped RBCs. “Crew cut” on skull x-ray due to marrow expansion from incr erythropoiesis (also in thalassemias).
Complications in sickle cell disease (SS):
-Aplastic crisis (due to parvovirus B19).
-Autosplenectomy (Howell-Jolly bodies) –> incr risk of infection with encapsulated organisms; early splenic dysfunction occurs in childhood.
-Splenic sequestration crisis.
-Salmonella osteomyelitis.
-Painful crisis (vaso-occlusive): dactylics. (painful hand swelling), acute chest syndrome (most common cause of death in adults), avascular necrosis, stroke.
-Renal papillary necrosis (due to low O2 in papilla; also seen in heterozygotes) and microhematuria (medullary infarcts).
-Diagnosis: hemoglobin electrophoresis.
-Treatment: hydroxyurea (incr HbF) and bone marrow transplantation.

26
Q

Autoimmune hemolytic anemia

A
  • Extrinsic hemolytic normocytic anemia
  • Warm agglutinin (IgG)—chronic anemia seen in SLE, CLL, or with certain drugs (e.g., α-methyldopa) (“warm weather is GGGreat”).
  • Cold agglutinin (IgM)—acute anemia triggered by cold; seen in CLL, Mycoplasma pneumonia infections, or infectious mononucleosis (“cold ice cream—yuMMM”).
  • Many warm and cold AIHA are idiopathic in etiology.
  • Autoimmune hemolytic anemias are usually Coombs+.
  • Direct Coombs test—anti-Ig antibody (Coombs reagent) added to patient’s blood. RBCs agglutinate if RBCs are coated with Ig.
  • Indirect Coombs test—normal RBCs added to patient’s serum. If serum has anti-RBC surface Ig, RBCs agglutinate when anti-Ig antibodies (Coombs reagent) added.
27
Q

Microangiopathic anemia

A
  • Extrinsic hemolytic normocytic anemia
  • Pathogenesis: RBCs are damaged when passing through obstructed or narrowed vessel lumina.
  • Seen in DIC, TTP-HUS, SLE, and malignant hypertension.
  • Schistocytes (helmet cells) are seen on blood smear due to mechanical destruction of RBCs.
28
Q

Macroangiopathic anemia

A
  • Extrinsic hemolytic normocytic anemia
  • Prosthetic heart valves and aortic stenosis may also cause hemolytic anemia 2° to mechanical destruction.
  • Schistocytes on peripheral blood smear
29
Q

Infections

A
  • Extrinsic hemolytic normocytic anemia

- Incr destruction of RBCs (e.g., malaria, Babesia).

30
Q

Infections

A
  • Extrinsic hemolytic normocytic anemia

- Incr destruction of RBCs (e.g., malaria, Babesia).

31
Q

Lab values in anemia

A
  • Transferrin—transports iron in blood.
  • Ferritin—1° iron storage protein of body.
  • Evolutionary reasoning—pathogens use circulating iron to thrive. The body has adapted a system in which iron is stored within the cells of the body and prevents pathogens from acquiring circulating iron.
  • Transferrin production is incr in pregnancy and by OCPs.

Iron deficiency: decr iron, incr TIBC, decr ferritin
Chronic dis: decr iron, decr TIBC, incr ferritin
Hemo-chromatosis: incr iron, decr TIBC, incr ferritin
Pregnancy/OCP: XXiron, incr TIBC, XX ferritin

32
Q

Neutropenia

A

-Absolute neutrophil count

33
Q

Lymphopenia

A

-Absolute lymphocyte count

34
Q

Eosinopenia

A
  • Corticosteroids cause neutrophilia, but eosinopenia and lymphopenia. Corticosteroids decr activation of neutrophil adhesion molecules, impairing migration out of the vasculature to sites of inflammation.
  • In contrast, corticosteroids sequester eosinophils in lymph nodes and cause apoptosis of lymphocytes.

Hematology/Oncology FirstAid (6 decks)

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