72. non-neoplastic disorders of the hematopoietic system (anemia and polycythemia) Flashcards

1
Q

72-1. Anemias of diminished erythropoiesis

types of anemia of diminished erythropoiesis (6)

A
Iron deficiency anemia
Megaloblastic anemia
Pernicious anemia
Aplastic anemia
Anemia of chronic disease
Myelophthisic anemia
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2
Q

72-1. Anemias of diminished erythropoiesis

cause of iron deficiency anemia (4)

A

dietary lack
blood loss (peptic ulcer, colon cancer, menorrhagia)
malabsorption
gastrectomy

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3
Q

72-1. Anemias of diminished erythropoiesis

Cellular appearance of iron deficiency anemia

A

microcytic anemia

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4
Q

72-1. Anemias of diminished erythropoiesis

syndrome related to iron deficiency anemia

A

Plummer-Vinson syndrome : esophageal web & atrophic glossitis with dysphagia

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5
Q

72-1. Anemias of diminished erythropoiesis

cause of megaloblastic anemia (2)

A

folate deficiency

vitamin B12 deficiency

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6
Q

72-1. Anemias of diminished erythropoiesis

mechanism of folate & vitamin B12 deficiency

A

folate & vitamin B12 = DNA synthesis precursor

so deficiency cause impaired division and enlargement of RBC precursor (megaloblast)

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7
Q

72-1. Anemias of diminished erythropoiesis

morphology of megaloblastic anemia (5)

A
hypercellular bone marrow
megaloblast (RBC precursor)
giant metamyelocyte (granulocyte precursor)
hypersegmented neutrophil
large egg shaped RBC
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8
Q

72-1. Anemias of diminished erythropoiesis

cause of folate deficiency (3)

A

poor diet
increased metabolic need (pregnancy, chronic hemolytic anemia patient)
malabsorption disorder

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9
Q

72-1. Anemias of diminished erythropoiesis

cause of vitamin B12 deficiency (3)

A

pernicious anemia : autoimmune reaction against parietal cell and intrinsic factor
gastrectomy
strict vegan

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10
Q

72-1. Anemias of diminished erythropoiesis

clinical manifestation of pernicious anemia (2)

A

demyelinating disorder

increased risk of gastric carcinoma

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11
Q

72-1. Anemias of diminished erythropoiesis

cause of aplastic anemia (4)

A

damage to hematopoietic stem cell

  • drug
  • chemicals
  • viral infection
  • autoimmune damage
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12
Q

72-1. Anemias of diminished erythropoiesis

consequence of hematopoietic stem cell damage (4)

A

aplastic anemia
thrombocytopenia
leukopenia
low reticulocyte count

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13
Q

72-1. Anemias of diminished erythropoiesis

cause of anemia of chronic disease (3)

A

endocarditis
autoimmune disease
cancer

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14
Q

72-1. Anemias of diminished erythropoiesis

mechanism of anemia of chronic disease

A

production of acute phase reactant (hepcidin) : sequester iron in storage sites

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15
Q

72-1. Anemias of diminished erythropoiesis

cause of myelophthisic anemia

A

cancer metastasis to bone marrow

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16
Q

72-2. anemias related to increased loss of red blood cells

types of hemolytic anemia (6)

A
Hereditary spherocytosis
Sickle cell anemia
Thalassemia
G6P dehydrogenase deficiency
Immunolytic anemia
Microangiopathic hemolytic anemia
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17
Q

72-2. anemias related to increased loss of red blood cells

cause of hereditary spherocytosis

A

inherited defect of RBC cytoskeleton-membrane tethering protein

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18
Q

72-2. anemias related to increased loss of red blood cells

clinicals of hereditary spherocytosis (3)

A

splenomegaly (spherocyte consumed by splenic macrophage)
jaundice
increased risk of gallstone

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19
Q

72-2. anemias related to increased loss of red blood cells

cause of sickle cell anemia

A

AR mutation of beta chain of hemoglobin, cause sickle hemoglobin

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20
Q

72-2. anemias related to increased loss of red blood cells

pathomechanism of sickle cell anemia (2)

A

cells continuously sicke and de-sickle while pass microcirculation, cause membrane damage

  • extravascular hemolysis : reticuloenothelial system remove damaged RBC
  • intravascular hemolysis :
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21
Q

72-2. anemias related to increased loss of red blood cells

consequences of sickle cell anemia (4)

A

compensatory hyperplasia of erythroid progenitor in bone marrow : skull change
jaundice
hemosiderosis
bilirubin gallstone

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22
Q

72-2. anemias related to increased loss of red blood cells

complications of sickle cell aneima (6)

A

due to vaso-occlusion (sicke cell occlude blood vessel)

  • Dactylitis : swollen hand and feet
  • Autosplenectomy
  • Acute chest syndrome
  • Pain crisis
  • Renal papillary necrosis
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23
Q

72-2. anemias related to increased loss of red blood cells

cause of thalassemia

A

inherited mutation, decreased synthesis of the globin chains of hemoglobin

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24
Q

72-2. anemias related to increased loss of red blood cells

types of thalassemia (2)

A

alpha thalassemia

beta thalassemia

25
Q

72-2. anemias related to increased loss of red blood cells

cause of alpha thalassemia

A

gene deletion (1 to 4, severity increases with number)

26
Q

72-2. anemias related to increased loss of red blood cells

cause of beta thalassemia

A

gene mutation

27
Q

72-2. anemias related to increased loss of red blood cells

types of beta thalassemia (2)

A

beta thalassemia minor : mild, asymptomatic

beta thalassemia major : severe anemia

28
Q

72-2. anemias related to increased loss of red blood cells

Clinical manifestation of beta thalassemia major (4)

A

extravascular hemolysis
massive erythroid hyperplasia : hematopoiesis into skull and facial bone
extramedullary hematopoiesis : hepatosplenomegaly
risk of aplastic crisis

29
Q

72-2. anemias related to increased loss of red blood cells

cause of G6P dehydrogenase deficiency

A

X linked recessive disorder, cells susceptible to oxidative stress

30
Q

72-2. anemias related to increased loss of red blood cells

pathomechanism of G6P dehydrogenase deficiency

A

glutathione neutralize H2O2
glutathione regenerated by NADPH, byproduct of G6P dehydrogenase
↓ G6P cause ↓ NADPH, lead to ↓ glutathione, ↑ oxidative stress to RBC

31
Q

72-2. anemias related to increased loss of red blood cells

characteristic morphology of G6P deficiency anemia

A

Heinz body (denatured hemoglobin)

32
Q

72-2. anemias related to increased loss of red blood cells

cause of immune hemolytic anemia

A

antibody (IgG, IgM) mediated destruction of RBC

33
Q

72-2. anemias related to increased loss of red blood cells

pathomechanism of IgG mediated immune hemolytic anemia

A

IgG bind to RBC, consumed by splenic macrophage (extravascular hemolysis)

34
Q

72-2. anemias related to increased loss of red blood cells

disease related to IgG mediated immune hemolytic anemia (3)

A

SLE
Chronic lymphocytic leukemia
penicillin (drug binding or auto-antibody production)

35
Q

72-2. anemias related to increased loss of red blood cells

pathomechanism of IgM mediated immune hemolytic anemia

A

IgM bind to RBC, fix complement (intravascular hemolysis)

36
Q

72-2. anemias related to increased loss of red blood cells

disease related to IgM mediated immune hemolytic anemia (2)

A

mycoplasma pneumoniae infection

infectious mononucleosis by EBV

37
Q

72-2. anemias related to increased loss of red blood cells

types of immune hemolytic anemia (2)

A

IgG mediated immune hemolytic anemia (warm type : body center)
IgM mediated immune hemolytic anemia (cold type : extremities)

38
Q

72-2. anemias related to increased loss of red blood cells

cause of microangiopathic hemolytic anemia

A

microthrombi in small vessel shears RBC

39
Q

72-2. anemias related to increased loss of red blood cells

disease related to microangiopathic hemolytic anemia (2)

A

thrombotic thrombocytopenic purpura (TTP) : decreased ADAMTS13
hemolytic uremic syndrome (HUS) : E. coli verotoxin

40
Q

73-3. Polycythemia vera and essential thrombocythemia

definition of polycythemia vera

A

neoplastic proliferation of mature myeloid cell, especially RBC

41
Q

73-3. Polycythemia vera and essential thrombocythemia

cause of polycythemia vera

A

JAK2 kinase mutation

42
Q

73-3. Polycythemia vera and essential thrombocythemia

clinical symptoms of polycythemia vera (6)

A
hyperviscosity of blood
blurry vision
headache
increased risk of venous thrombosis
flushed face due to congestion
itching
43
Q

73-3. Polycythemia vera and essential thrombocythemia

definition of essential thrombocytopenia

A

neoplastic proliferation mature myeloid cell, especially platelets

44
Q

73-3. Polycythemia vera and essential thrombocythemia

cause of essential thrombocythemia

A

JAK2 kinase mutation

45
Q

73-3. Polycythemia vera and essential thrombocythemia

symptoms of essential thrombocythemia (2)

A

increased risk of bleeding

increased risk of thrombosis

46
Q

74-4. non-neoplastic disorders of myeloid and lymphoid system
types of non-neoplastic disorders of myeloid and lymphoid system (2)

A

leukopenia (deficiency)

leukocytosis (proliferation)

47
Q

74-4. non-neoplastic disorders of myeloid and lymphoid system
types of leukopenia (2)

A

neutropenia : decreased number of circulating neutrophil

lymphopenia : decreased nimber of circulating lymphocyte

48
Q

74-4. non-neoplastic disorders of myeloid and lymphoid system
cause of neutropenia (2)

A

drug toxicity : chemotherapy with alkylating agent

severe infection : increased movement of neutrophil into tissue

49
Q

74-4. non-neoplastic disorders of myeloid and lymphoid system
cause of lymphopenia (4)

A

immunodeficiency (DiGeroge syndrome, HIV)
high cortisol state (cushing syndrome) : induce apoptosis of lymphocyte
autoimmune destruction (SLE)
whole body radiation

50
Q

74-4. non-neoplastic disorders of myeloid and lymphoid system
types of leukocytosis (5)

A
neutrophilic leukocytosis
monocytosis
eosinophilia
basophilia
lymphocytic leukocytosis
51
Q

74-4. non-neoplastic disorders of myeloid and lymphoid system
cause of neutrophilic leukocytosis (3)

A

bacterial infection
tissue necrosis
high cortisol state

52
Q

74-4. non-neoplastic disorders of myeloid and lymphoid system
cause of monocytosis (2)

A

chronic inflammatory state (autoimmune, infection)

malignancy

53
Q

74-4. non-neoplastic disorders of myeloid and lymphoid system
cause of eosinophilia (3)

A

allergic reaction (type 1 hypersensitivity)
parasitic infection
Hodgkin lymphoma

54
Q

74-4. non-neoplastic disorders of myeloid and lymphoid system
cause of basophilia

A

chronic myeloid leukemia

55
Q

74-4. non-neoplastic disorders of myeloid and lymphoid system
cause of lymphocytic leukocytosis (2)

A

viral infection : cause T lymphocyte hyperplasia

Bordetella pertussis infection : bacteria produce lymphocytosis promoting factor

56
Q

74-4. non-neoplastic disorders of myeloid and lymphoid system
cause of infectious mononucleosis (2)

A
EBV infection (kissing disease)
CMV infection
57
Q

74-4. non-neoplastic disorders of myeloid and lymphoid system
pathomechanism of infectious mononucleosis

A

cause lymphocytic leukocytosis, produce reactive CD8 T cell (T cell hyperplasia)

58
Q

74-4. non-neoplastic disorders of myeloid and lymphoid system
organ manifestation of infectious mononucleosis (3)

A

lymphadenopathy
splenomegaly
high WBC count with reactive CD8 T cell in blood

59
Q

74-4. non-neoplastic disorders of myeloid and lymphoid system
diagnosis of infectious mononucleosis

A

monospot test : patient IgM with sheep or horse RBC, agglutination in case of IM