RBC disorder Flashcards
Major division
1. Vit B12 deficiency
2. folic acid deficiency
Megaloblastic anemia
decrease amount of cells in all cell lines
Pancytopenia
function: ileum
absorption
function: adenosylcobalamin
liver, storage
function: castle’s factor
intrinsic factor
function: methylcobalamin
plasma
function: transcobalamin
transporter
only vitamin synthesized by microorganism
cobalamin
caused by D. latum
vitamin B12 deficiency
associated with pernicious anemia
vitamin B12 deficiency
associated with hypochlorhydria
vitamin B12 deficiency
other name of pernicious anemia
addison’s anemia or atrophy gastritis
pernicious anemia produces a yellow lemon color of the skin and has been called as
megaloblastic madness
caused by failure of the gastric mucosa to secrete intrinsic factor
pernicious anemia
intrinsic factor is present or low at number to facilitate the absorption of cobalamin
pernicious anemia
intrinsic factor is present or low at number to facilitate the absorption of cobalamin
there is a problem in the absorption of cobalamin but the intrinsic factor is normal
Imerslund-Grasbeck Syndrome
Diagnosis of Cobalamin deficiency (4)
- schilling test
- serum cobalamin assay
- methylmalonic acid and homocysteine assays
- deoxyuridine suppression test
what is absorbed in the jejunum
folate
how is folate deficiency corrected
taking vitamin B12 supplementation
folate acid deficiency causes
- chronic alcoholics
- poor dietary
- pregnancy
- steatorrhea
vitamin B12 deficiency = __ B12, __ serum folate, __ red cell folate
↓ B12, NORMAL serum folate, ↓ red cell folate
Folic acid deficiency = __ B12, __ serum folate, __ red cell folate
NORMAL B12, ↓ serum folate, ↓ red cell folate
deficiency of both folic and vitamin b12 deficiency = ?
everything is low
manifest macro-ovalocytes and giant hypersegmented neutrophils
megaloblastic anemia
autosomally rcessive inherited defect
Imerslund-Grasbeck Syndrom
“shift reticulocyte”
non-megaloblastic anemia
(type of macrocyte)
seen in folic acid deficiency, vit b12 deficiency, pernicious anemia
oval macrocyte
(type of macrocyte)
seen in alcoholism, hypothyroidism, and liver disease
round hypochromic macrocyte
(type of macrocyte)
seen in neonate response to anemic stress, response to anemic stress
blue-tinged macrocyte
(type of macrocyte)
seen in neonate response to anemic stress, response to anemic stress
blue-tinged macrocyte
marrow replacement by involvement with abnormal cells or tissue components
myelophthisic anemia
associated with normochromic and normocytic, increased NRBC
myelophthisic anemia
bone marrow does not produce any blood cells
aplastic anemia
associated with pancytopenia, macrocytosis, increase RDW, chloramphenicol
aplastic anemia
medication that commonly cause aplastic anemia
chloramphenicol
inherited aplastic anemia, automal recessive, increased level of Hgb F and i antigen
Fanconi’s anemia
associated with parvo virus B19 infection, scattered GIANT pronormoblast in bone marrow, not aplastic anemia
Transient aplastic crises
not aplastic anemia, but pure red cell aplasia
Transient aplastic crises
congenital red cell aplasia
diamond blackfan anemia
there is pancytopenia wherein all the cell lines are affected
Fanconi’s anemia
only the red cells are affected
diamond blackfan anemia
blood lost over a short time
acute posthemorrhagic anemia
blood lost in small amounts over a extended t time
chronic posthemorrhagic anemia
increase red blood cell destruction
hemolytic anemia
- patient’s rbc would not survive when transfused to normal patient
- defect of rbc itself
- membrane, metabolic, and hemoglobin defects
intrinsic hemolytic anemia
- life span of rbc decrease, if transfused into the patient
- acquired
extrinsic hemolytic anemia
- hemoglobinemia
- methemalbuminemia
- hemoglobnuria
- hemosiderinuria
- ↑ LDH
INTRAVASCULAR
- hemoglobinemia
- hemoglobnuria
- ↑ urine/fecal urobilinogen
- ↑ blood carboxyhemoglobin level
- ↑ indirect-reacting serum
EXTRAVASCULAR
80-90% of most red cell destruction happens in
extravascular
site of destruction of erythrocyte (intravascular)
within blood cell
site of destruction of erythrocyte (extravascular)
spleen or liver
mechanism (intravascular)
activation of complement lgM of lgG
mechanism (extravascular)
cell-mediated phagocytosis of lgM or lgG-coated cells
laboratory findings (intravascular)
- hemoglobinuria direct antiglobulin test
- hemosidenuria
laboratory findings (extravascular)
- postive direct antiglobulin test
- erythrocytes
- most prevalent hereditary hemolytic anemia (northern european descent)
- deficiency of key membrane protein: SPECTRIN
- increased osmotic fragility
hereditary spherocytosis
hyperpermeable to sodium, increasing osmotic fragility
spherocytosis
- horizontal defect
- weakening of membrane skeleton
- southeast asian ovalocytosis
hereditary elliptocytosis
red cell fragment at 45°C to 46°C
hereditary pyropoikilocytosis
- well-defined BAND 3 molecular deletion
- increased resistance to malaria
- malenasian and malayan population
southeast asian ovalocytes
thinner variant of elliptocytes or ovalocytes
pencil cells/oat cells
red cell dehydration due to loss of cation, predominantly K+ and water
hereditary stomacytosis
- increased surface-to-volume ratio leading to moderate to severe anemia
- decrease osmotic fragility
xerocytosis
- increased surface-to-volume ratio leading to moderate to severe anemia
- decrease osmotic fragility
hereditary xerocytosis
- increased surface-to-volume ratio leading to moderate to severe anemia
- decrease osmotic fragility
