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

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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)

Study These Flashcards
A

alpha thalassemia

beta thalassemia

25
72-2. anemias related to increased loss of red blood cells | cause of alpha thalassemia
gene deletion (1 to 4, severity increases with number)
26
72-2. anemias related to increased loss of red blood cells | cause of beta thalassemia
gene mutation
27
72-2. anemias related to increased loss of red blood cells | types of beta thalassemia (2)
beta thalassemia minor : mild, asymptomatic | beta thalassemia major : severe anemia
28
72-2. anemias related to increased loss of red blood cells | Clinical manifestation of beta thalassemia major (4)
extravascular hemolysis massive erythroid hyperplasia : hematopoiesis into skull and facial bone extramedullary hematopoiesis : hepatosplenomegaly risk of aplastic crisis
29
72-2. anemias related to increased loss of red blood cells | cause of G6P dehydrogenase deficiency
X linked recessive disorder, cells susceptible to oxidative stress
30
72-2. anemias related to increased loss of red blood cells | pathomechanism of G6P dehydrogenase deficiency
glutathione neutralize H2O2 glutathione regenerated by NADPH, byproduct of G6P dehydrogenase ↓ G6P cause ↓ NADPH, lead to ↓ glutathione, ↑ oxidative stress to RBC
31
72-2. anemias related to increased loss of red blood cells | characteristic morphology of G6P deficiency anemia
Heinz body (denatured hemoglobin)
32
72-2. anemias related to increased loss of red blood cells | cause of immune hemolytic anemia
antibody (IgG, IgM) mediated destruction of RBC
33
72-2. anemias related to increased loss of red blood cells | pathomechanism of IgG mediated immune hemolytic anemia
IgG bind to RBC, consumed by splenic macrophage (extravascular hemolysis)
34
72-2. anemias related to increased loss of red blood cells | disease related to IgG mediated immune hemolytic anemia (3)
SLE Chronic lymphocytic leukemia penicillin (drug binding or auto-antibody production)
35
72-2. anemias related to increased loss of red blood cells | pathomechanism of IgM mediated immune hemolytic anemia
IgM bind to RBC, fix complement (intravascular hemolysis)
36
72-2. anemias related to increased loss of red blood cells | disease related to IgM mediated immune hemolytic anemia (2)
mycoplasma pneumoniae infection | infectious mononucleosis by EBV
37
72-2. anemias related to increased loss of red blood cells | types of immune hemolytic anemia (2)
IgG mediated immune hemolytic anemia (warm type : body center) IgM mediated immune hemolytic anemia (cold type : extremities)
38
72-2. anemias related to increased loss of red blood cells | cause of microangiopathic hemolytic anemia
microthrombi in small vessel shears RBC
39
72-2. anemias related to increased loss of red blood cells | disease related to microangiopathic hemolytic anemia (2)
thrombotic thrombocytopenic purpura (TTP) : decreased ADAMTS13 hemolytic uremic syndrome (HUS) : E. coli verotoxin
40
73-3. Polycythemia vera and essential thrombocythemia | definition of polycythemia vera
neoplastic proliferation of mature myeloid cell, especially RBC
41
73-3. Polycythemia vera and essential thrombocythemia | cause of polycythemia vera
JAK2 kinase mutation
42
73-3. Polycythemia vera and essential thrombocythemia | clinical symptoms of polycythemia vera (6)
``` hyperviscosity of blood blurry vision headache increased risk of venous thrombosis flushed face due to congestion itching ```
43
73-3. Polycythemia vera and essential thrombocythemia | definition of essential thrombocytopenia
neoplastic proliferation mature myeloid cell, especially platelets
44
73-3. Polycythemia vera and essential thrombocythemia | cause of essential thrombocythemia
JAK2 kinase mutation
45
73-3. Polycythemia vera and essential thrombocythemia | symptoms of essential thrombocythemia (2)
increased risk of bleeding | increased risk of thrombosis
46
74-4. non-neoplastic disorders of myeloid and lymphoid system types of non-neoplastic disorders of myeloid and lymphoid system (2)
leukopenia (deficiency) | leukocytosis (proliferation)
47
74-4. non-neoplastic disorders of myeloid and lymphoid system types of leukopenia (2)
neutropenia : decreased number of circulating neutrophil | lymphopenia : decreased nimber of circulating lymphocyte
48
74-4. non-neoplastic disorders of myeloid and lymphoid system cause of neutropenia (2)
drug toxicity : chemotherapy with alkylating agent | severe infection : increased movement of neutrophil into tissue
49
74-4. non-neoplastic disorders of myeloid and lymphoid system cause of lymphopenia (4)
immunodeficiency (DiGeroge syndrome, HIV) high cortisol state (cushing syndrome) : induce apoptosis of lymphocyte autoimmune destruction (SLE) whole body radiation
50
74-4. non-neoplastic disorders of myeloid and lymphoid system types of leukocytosis (5)
``` neutrophilic leukocytosis monocytosis eosinophilia basophilia lymphocytic leukocytosis ```
51
74-4. non-neoplastic disorders of myeloid and lymphoid system cause of neutrophilic leukocytosis (3)
bacterial infection tissue necrosis high cortisol state
52
74-4. non-neoplastic disorders of myeloid and lymphoid system cause of monocytosis (2)
chronic inflammatory state (autoimmune, infection) | malignancy
53
74-4. non-neoplastic disorders of myeloid and lymphoid system cause of eosinophilia (3)
allergic reaction (type 1 hypersensitivity) parasitic infection Hodgkin lymphoma
54
74-4. non-neoplastic disorders of myeloid and lymphoid system cause of basophilia
chronic myeloid leukemia
55
74-4. non-neoplastic disorders of myeloid and lymphoid system cause of lymphocytic leukocytosis (2)
viral infection : cause T lymphocyte hyperplasia | Bordetella pertussis infection : bacteria produce lymphocytosis promoting factor
56
74-4. non-neoplastic disorders of myeloid and lymphoid system cause of infectious mononucleosis (2)
``` EBV infection (kissing disease) CMV infection ```
57
74-4. non-neoplastic disorders of myeloid and lymphoid system pathomechanism of infectious mononucleosis
cause lymphocytic leukocytosis, produce reactive CD8 T cell (T cell hyperplasia)
58
74-4. non-neoplastic disorders of myeloid and lymphoid system organ manifestation of infectious mononucleosis (3)
lymphadenopathy splenomegaly high WBC count with reactive CD8 T cell in blood
59
74-4. non-neoplastic disorders of myeloid and lymphoid system diagnosis of infectious mononucleosis
monospot test : patient IgM with sheep or horse RBC, agglutination in case of IM

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