72. non-neoplastic disorders of the hematopoietic system (anemia and polycythemia) Flashcards
72-1. Anemias of diminished erythropoiesis
types of anemia of diminished erythropoiesis (6)
Iron deficiency anemia Megaloblastic anemia Pernicious anemia Aplastic anemia Anemia of chronic disease Myelophthisic anemia
72-1. Anemias of diminished erythropoiesis
cause of iron deficiency anemia (4)
dietary lack
blood loss (peptic ulcer, colon cancer, menorrhagia)
malabsorption
gastrectomy
72-1. Anemias of diminished erythropoiesis
Cellular appearance of iron deficiency anemia
microcytic anemia
72-1. Anemias of diminished erythropoiesis
syndrome related to iron deficiency anemia
Plummer-Vinson syndrome : esophageal web & atrophic glossitis with dysphagia
72-1. Anemias of diminished erythropoiesis
cause of megaloblastic anemia (2)
folate deficiency
vitamin B12 deficiency
72-1. Anemias of diminished erythropoiesis
mechanism of folate & vitamin B12 deficiency
folate & vitamin B12 = DNA synthesis precursor
so deficiency cause impaired division and enlargement of RBC precursor (megaloblast)
72-1. Anemias of diminished erythropoiesis
morphology of megaloblastic anemia (5)
hypercellular bone marrow megaloblast (RBC precursor) giant metamyelocyte (granulocyte precursor) hypersegmented neutrophil large egg shaped RBC
72-1. Anemias of diminished erythropoiesis
cause of folate deficiency (3)
poor diet
increased metabolic need (pregnancy, chronic hemolytic anemia patient)
malabsorption disorder
72-1. Anemias of diminished erythropoiesis
cause of vitamin B12 deficiency (3)
pernicious anemia : autoimmune reaction against parietal cell and intrinsic factor
gastrectomy
strict vegan
72-1. Anemias of diminished erythropoiesis
clinical manifestation of pernicious anemia (2)
demyelinating disorder
increased risk of gastric carcinoma
72-1. Anemias of diminished erythropoiesis
cause of aplastic anemia (4)
damage to hematopoietic stem cell
- drug
- chemicals
- viral infection
- autoimmune damage
72-1. Anemias of diminished erythropoiesis
consequence of hematopoietic stem cell damage (4)
aplastic anemia
thrombocytopenia
leukopenia
low reticulocyte count
72-1. Anemias of diminished erythropoiesis
cause of anemia of chronic disease (3)
endocarditis
autoimmune disease
cancer
72-1. Anemias of diminished erythropoiesis
mechanism of anemia of chronic disease
production of acute phase reactant (hepcidin) : sequester iron in storage sites
72-1. Anemias of diminished erythropoiesis
cause of myelophthisic anemia
cancer metastasis to bone marrow
72-2. anemias related to increased loss of red blood cells
types of hemolytic anemia (6)
Hereditary spherocytosis Sickle cell anemia Thalassemia G6P dehydrogenase deficiency Immunolytic anemia Microangiopathic hemolytic anemia
72-2. anemias related to increased loss of red blood cells
cause of hereditary spherocytosis
inherited defect of RBC cytoskeleton-membrane tethering protein
72-2. anemias related to increased loss of red blood cells
clinicals of hereditary spherocytosis (3)
splenomegaly (spherocyte consumed by splenic macrophage)
jaundice
increased risk of gallstone
72-2. anemias related to increased loss of red blood cells
cause of sickle cell anemia
AR mutation of beta chain of hemoglobin, cause sickle hemoglobin
72-2. anemias related to increased loss of red blood cells
pathomechanism of sickle cell anemia (2)
cells continuously sicke and de-sickle while pass microcirculation, cause membrane damage
- extravascular hemolysis : reticuloenothelial system remove damaged RBC
- intravascular hemolysis :
72-2. anemias related to increased loss of red blood cells
consequences of sickle cell anemia (4)
compensatory hyperplasia of erythroid progenitor in bone marrow : skull change
jaundice
hemosiderosis
bilirubin gallstone
72-2. anemias related to increased loss of red blood cells
complications of sickle cell aneima (6)
due to vaso-occlusion (sicke cell occlude blood vessel)
- Dactylitis : swollen hand and feet
- Autosplenectomy
- Acute chest syndrome
- Pain crisis
- Renal papillary necrosis
72-2. anemias related to increased loss of red blood cells
cause of thalassemia
inherited mutation, decreased synthesis of the globin chains of hemoglobin
72-2. anemias related to increased loss of red blood cells
types of thalassemia (2)
alpha thalassemia
beta thalassemia
72-2. anemias related to increased loss of red blood cells
cause of alpha thalassemia
gene deletion (1 to 4, severity increases with number)
72-2. anemias related to increased loss of red blood cells
cause of beta thalassemia
gene mutation
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
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
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
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
72-2. anemias related to increased loss of red blood cells
characteristic morphology of G6P deficiency anemia
Heinz body (denatured hemoglobin)
72-2. anemias related to increased loss of red blood cells
cause of immune hemolytic anemia
antibody (IgG, IgM) mediated destruction of RBC
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)
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)
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)
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
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)
72-2. anemias related to increased loss of red blood cells
cause of microangiopathic hemolytic anemia
microthrombi in small vessel shears RBC
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
73-3. Polycythemia vera and essential thrombocythemia
definition of polycythemia vera
neoplastic proliferation of mature myeloid cell, especially RBC
73-3. Polycythemia vera and essential thrombocythemia
cause of polycythemia vera
JAK2 kinase mutation
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
73-3. Polycythemia vera and essential thrombocythemia
definition of essential thrombocytopenia
neoplastic proliferation mature myeloid cell, especially platelets
73-3. Polycythemia vera and essential thrombocythemia
cause of essential thrombocythemia
JAK2 kinase mutation
73-3. Polycythemia vera and essential thrombocythemia
symptoms of essential thrombocythemia (2)
increased risk of bleeding
increased risk of thrombosis
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)
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
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
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
74-4. non-neoplastic disorders of myeloid and lymphoid system
types of leukocytosis (5)
neutrophilic leukocytosis monocytosis eosinophilia basophilia lymphocytic leukocytosis
74-4. non-neoplastic disorders of myeloid and lymphoid system
cause of neutrophilic leukocytosis (3)
bacterial infection
tissue necrosis
high cortisol state
74-4. non-neoplastic disorders of myeloid and lymphoid system
cause of monocytosis (2)
chronic inflammatory state (autoimmune, infection)
malignancy
74-4. non-neoplastic disorders of myeloid and lymphoid system
cause of eosinophilia (3)
allergic reaction (type 1 hypersensitivity)
parasitic infection
Hodgkin lymphoma
74-4. non-neoplastic disorders of myeloid and lymphoid system
cause of basophilia
chronic myeloid leukemia
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
74-4. non-neoplastic disorders of myeloid and lymphoid system
cause of infectious mononucleosis (2)
EBV infection (kissing disease) CMV infection
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)
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
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