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FOD > Anemias > Flashcards

Anemias Flashcards

1
Q

Silent carrier of alpha thalassemia

A

Three alpha genes, with RBCs of low normal size, but usually completely asymptomatic are called ____, 36% of African- Americans

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2
Q

Alpha thalassemia minor

A

Two alpha genes (one missing on opposite chromosomes or the same), with small RBCs, but otherwise normal

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3
Q

Alpha thalassemia major

A

1 in 1600 SE Asian children have no alpha genes, and usually die in utero of hydrops fetalis

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4
Q

Alpha thalassemia intermedia

A

Inherit only one alpha gene. Marked anemia and microcytosis. Make hemoglobin H

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5
Q

Hemoglobin H

A

Tetramer of beta globins. Unstable and slowly preciptates to form Heinz bodies

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6
Q

beta thalassemia minor

A

Loss of one full beta gene, either from partial loss of both or full loss of one, four alpha and

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7
Q

Beta thalassema major and intermedia

A

Present in infancy when HbF production stops and HbA production does not start- jaundice, growth retardation and skeletal deformations possible

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8
Q

Thalassemias

A

Normal globin chains produced in abnormal number. RBC have low Hb content and often are small when unpaired globin chains precipitate and are removed along with membrane

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9
Q

Hemoglobinopathies

A

Abnormal alleles of globin chains are produced (ex. sickle cell)

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10
Q

HbS

A

Sickle cell hemoglobin, evolved at least 5 times in Africa under the pressure of falciparal malaria

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11
Q

Glu to Val (GAG to GTG)

A

In sickle cell, 6 AA of beta globin gene is changed from ____

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12
Q

Deoxyhemoglobin-S

A

Strands assemble in groups to form fibers from two strands of molecules held together by beta6 valines - fibers form fascicles, which can grow and distort red cells as conc increases

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13
Q

Drive sickle cell formation

A

Intracellular dehydration (increased Hb conc), low pH (low O2 tension), decreased transit time through vascular beds (normally in spleen, bone marrow and inflammation)

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14
Q

Aplastic crisis

A

Marrow is not functioning, is wiped out

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15
Q

Parvovirus B-19

A

Causes aplastic crisis anytime there is a red cell mutation

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16
Q

Sickle cell problems

A

Intravascular hemolysis - chronic hemolytic anemia. RBCs only survive 10 days (free Hb injures endothelium and scavenges NO-> pulm hypertensions), high rates of marrow turnover leads to folate deficiency

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17
Q

Functional asplenia

A

Problem in sickle cell- deoxygenation of RBC in splenic sinusoids leads to sequestration and infarction. Loss makes patients more susceptible to bacterial infections (pneumococcal pneumonia and salmonella osteolyelitis)

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18
Q

Renal injury

A

Symptom of sickle cell- papillary infarction and sloughing can lead to obstruction and chronic blood less, loss leads to systemic hyperosmolarity and more frequent sickling episodes

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19
Q

Bone infarction

A

Symptom of sickle cell- rigid blood vessels in bone obstruct easily. Marrow infarcts cause painful episodes and loss of productive marrow. Femoral and humeral head and other large bone infarcts can lead to orthopedic disability

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20
Q

Pulmonary infarction

A

Most common cause of death in sickle cell patients. Pneumonia can lead to lung hypoxia and lung vessel infarction. Lung infarcts cause areas of necrotic lung which are more susceptible to infection -> vicous cycle of infection and infarction leading to shunting and global hypoxia

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21
Q

Brain infarction

A

Second most common cause of death and most important cause of disability in sickle cell. Blood vessel injury from sickle cells leads to blood vessel thickening and increased susceptibility to stroke

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22
Q

Hydroxyurea

A

only drug approved for sickle cell. Increases HbF levels (prevents polymers with S and produces stable tetramers with alpha), reduces frequency of painful episodes, and works in about 40% of HbSS patients

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23
Q

Transfusions

A

Helps to prevent stroke in sickle cell

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24
Q

HbAS (sickle cell trait)

A

Generally benign, causes hematuria, can cause splenic infarction, rare infarction in orthopedic surgery and rare deaths in military recruits

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25
Q

HbC

A

Glu to Lys in B6 globin. Milder disease

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26
Q

microcytic anemia

A

iron deficiency causes

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27
Q

Macrocytic and megaloblastic anemias

A

Folate and B12 deficiencies cause

28
Q

Iron deficiency anemia

A

Affects hemoglobin synthesis

29
Q

Vitamin B12 and folate deficiency

A

Affects DNA synthesis

30
Q

Transferrin

A

iron absorbed from the gut is bound to plasma ____ and transported to the marrow where it is incorporated into hemoglobin in developing RBCs. Iron is extracted when RBCs are ingested by macrophages and recycled back to this.
Synthesized in the liver

31
Q

Ferritin

A

Soluble iron protein complex for storing iron

32
Q

Hepcidin

A

Protein made in liver that blocks intestinal absorption and transport. Keeps track of much transferrin is bringing in iron and liver release this if there is too much iron
Release leads to downregulation of ferroportin so iron gets lost when duodenal epithelial cells are shred into the gut
Reduces transfer of iron from storage pool to developing erythroid precursors in bone marrow

33
Q

Ferroportin

A

Allows iron absorbed into gut to be transferred to plasma transferrin

34
Q

duodenum and prox jejunum

A

Iron absorbed in what part of gut

35
Q

Markers of iron deficiency

A

Decreased serum iron, increased serum transferrin (also TIBC), decreased transferrin saturation (%sat= Fe/TIBC), increased soluble transferrin receptor, decreased ferritin (reflects low storage iron)

36
Q

Microcytic

A

Iron deficiency causes this as anemia worsens. Decreasing MCV (mean corpuscular volume) - avg volume of a red cell

37
Q

Hypochromic

A

Decreasing MCHC (mean corpuscular hemoglobin concentration)- avg conc of Hb in red cell. Reduced Hb. Cells become this as iron def anemia worsens

38
Q

Anemia of chronic disease

A

Impaired red cell production associated with reduction in proliferation of erythroid progenitors and impaired iron utilization. Can be caused by infections (osteomyelitis), chronic immune disorders (rheumatoid arthritis) or malignancies
Have normal to increased amounts of bone marrow iron, but not released normally to blood- developing RBC starved for iron even though storage is present

39
Q

Anemia of chronic disease pathophys

A

Cytokine mediated induction of hepcidin (largely by interleukin-6) -> decreased iron absorption, decreased release of macrophage iron, not responsive to iron supplementation. Cytokine mediated inhibition of erythropoiesis

40
Q

Decreased iron release

A

Inflammation/anemia of chronic disease causes anemia because of ____

41
Q

Decreased globin synthesis

A

Thalassemia causes anemia because of _____

42
Q

Increased, reduced to normal

A

Transferrin is _____ in iron deficiency anemia and ______ in anemia of chronic disease

43
Q

Reduced, normal to increased

A

Ferritin is ______ in iron deficiency anemia and _____ in anemia of chronic disease

44
Q

Macrocytic anemias

A

Big cells, not all are megaloblastic. Folate and B23 deficiencies, reticulocytosis, liver disease, and other conditions cause

45
Q

Megaloblastic cells

A

Have a characteristic nuclear maturation defect. Normal cytoplasmic maturation

46
Q

Folate and B12

A

These are necessary for production of thymine. Deficiency causes a nuclear but not a cytoplasmic maturation defect

47
Q

Folate deficiency

A

Causes developmental neurologic injuries

48
Q

B12 deficiency

A

Causes an acquired central and long tract neurologic injury

49
Q

Folate

A

Accepts one carbon units from donor molecules and passes them on -> methionine, purines, pyrimidines

50
Q

Folate and B12

A

Needed to convert homocysteine to methionine

51
Q

B12 only

A

Needed to convert methyl malonyl CoA to succinyl CoA in nerve cells. Methyl malonate (MMA) is neurotoxin, converted to methyl malonyl CoA then succinyl coA. Failure of normal detox system leads to neuron death

52
Q

Megaloblastic changes in bone marrow

A

Large polychromatophilic normoblasts with poorly condensed chromatin. Nuclear-cytoplasmic dissynchrony

some cells fail to mature and undergo apoptosis, hematopoiesis becomes inefficient

53
Q

Neural tube defects, intestinal dysplasia, hypercoagulable state

A

Non-hematologic problems associated with low folic acid

54
Q

Folate deficiency

A

Anemia, usually macrocytic, pancytopenia (all cell lines of marrow affected), malabsorption (weight loss, diarrhea, ab pain, glossitis)
Alcohol has directly toxic effect (malabsorption, reduce tissue release, interruption of hepatoenteric circulation, increased urinary excretion)

55
Q

R-binders

A

Contained in saliva, biind to B12 when B12 is released from food by action of pepsin in stomach

56
Q

Gastric fundic parietal cells

A

Intrinsic factor is secreted by these cells. B12 is released from R-binder by pancreatic proteases and bound to IF

57
Q

Cubilin

A

IF-B12 complex absorbed in terminal ileum

58
Q

Transcobalamins I-III

A

carries B12 in the blood stream, II is required for transfer to tissues

59
Q

IF deficiency

A

key cause of B12 deficiency, congential deficieny, gastric resection or destruction by autoantibodies

60
Q

pernicious anemia

A

Intrinsic factor deficiency caused by destruction by autoantibodies
Type A gastritis- parietal cells destory
Type I anti-IF antibodies block IF binding, Type II anti-IF antibodies block absorption

61
Q

Causes of B12 deficiency

A

Dietary insufficiency (rare), IF deficiency, competitive utilization (bacterial overgrowth or fish tapeworm), ileal disease

62
Q

B12 deficiency symptoms

A

Anemia (pallor, dyspnea, palpitations, lassitude), GI (wt loss, diarrhea, pain), reproductive (infertility), neurological (loss of sensation), psych (depressed, confused), autoimmune diseases, behavorial (restricted diet)

63
Q

Vitiligo

A

skin blotches, Associated with pernicious anemia and other autoimmune diseases

64
Q

Neuro presentation of B12 deficiency

A

Peripheral neuropathy (paresthesias, hyporeflexia), spinal cord degeneration, memory loss, disorientation, depression

65
Q

Schilling test

A

Test B12 absorption. Oral radioactive B12 given after IV load, percentage of b12 estimated from 24 hour urine collection. If abnormal, repeat with IF supplement. If still abnormal, antibiotics to treat bacterial overgrowth. If still abnormal, pancreatic enzyme supplementation

66
Q

Tuberculous ileitis

A

Leads to malabsoprtion, causing B12 deficiency and brain atrophy. another cause of megaloblastic anemia

67
Q

Fish tapeworm (diphyllobothrium latum)

A

parasite absorbs 80% or more of host’s B12 intake and causes megaloblastic anemia

FOD (5 decks)

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