Blood Disorders pt 1 Flashcards
hemoglobinopathies
a group of disorders in which there is abnormal production or structure of the hemoglobin molecule.
how does sickle cell disease occur
a Glu is replaced with Val in the beta chain of hemoglobin
what is a megaloblast
abnormally large erythroid precursors
what is hemolytic anemia
RBCs destroyed faster than they can be made
what is aplastic anemia characterized by (3 things)
reduction of hematopoietic tissue
fatty marrow replacement
pancytopenia
what are some clinical manifestations of pernicious anemia
megaloblastic madness
paresthesia of the feet and hands - proprioception disturbances
what are the symptoms for moderate to severe anemia
hypotension
tachynea/dyspnea
tachycardia, transient murmurs, angina, HF
claudication, night cramps
headaches/lightheaded
tinnitus
what are some common causes of anemia
blood loss
impaired RBC production
increased RBC destruction
Describe the RBC indices for thalassemia
MCV: low
MCH: low
MCHC: norm/dec
Platelets: norm
RBC: inc
WBC: inc
Hb: low
Hct: low
Standard lab values for RBC count
M: 4.2-5.4x10^6/mL
F: 3.6-5.0x10^6/mL
Standard lab values for hemoglobin test
M: 14-16.5g/dL
F:12-15g/dL
what are some causes of b12 deficiency (3)
damage/atrophy of parietal cells in stomach
resectioning of stomach or SI
chronic malabsorption (chrons, aids)
Standard lab values for MCH
27-34 pg/cell
what are globin chains
the alpha and beta chain aspect of hemoglobin
Petechia
small blood vessels leak under skin (under 4mm)
how can we treat aplastic anemia
manage symptoms via transfusion
bone marrow transplant
immunosuppression therapy
what is hereditary spherocytosis
a disorder of the RBC membrane
Describe the RBC indices for chronic kidney failure
MCV: normal/slight dec
MCH: norm/slight dec
MCHC: normal/slight dec
Platelets: decreases as the kidney failure gets worse
RBC: low
WBC: normal/slight inc
Hb: low
Hct: low
genetic aplastic anemia - causes pancytopenia due to defects in DNA repair
Fanconi anemia
what are the symptoms for mild to moderate anemia
fatigue
weakness
loss of stamina
tachycardia
exertional dyspnea
what causes occlusions with SCD
sickled RBCs are not able to pass through membranes and get blocked up
What are some causes of folate deficiency
dietary
alcoholism
cirrhosis
pregnancy
malabsorption
what is the treatment for anemia of chronic kidney failure
epo therapy
What does the reticulocyte test tell us
Percent of immature RBCs
Describe the RBC indices for Sickle Cell
lots of different shapes and sizes
Platelets: norm
WBC: norm
what genes is thalassemia associated with
mutant genes that suppress the rate of globin chain synthesis
What does the RBC count tell us
number of RBCs per mL of blood
B12 and folate are required for what
nuclear maturation and DNA synthesis
how do you diagnose aplastic anemia
bone marrow biopsy
what are the 2 compensatory mechanisms for hypoxia from anemia
increase BF (HR, CO)
restore tissue oxygenation
what hormone stimulates RBC production in response to low pO2
erythropoietin
Describe the RBC indices for aplastic anemia
MCV: normal
MCH: norm/slight dec
MCHC: normal
Platelets: low
RBC: low
WBC: low
Hb: low
what are two common cardiovascular complications due ot sickle cell disease
chamber enlargement
myocardial infarction
What are the terms for a normal and low MCHC
normochromic
hypochromic
Purpura
small blood vessels leak under skin (4-10mm)
what are 3 clinical manifestations of folate deficiency
demeanor change - depression, irritability, sleep deprivation
memory impairment
perversions of smell and taste
Standard lab values for Reticulocyte test
1-1.5% of total RBC
what is aplastic anemia caused by
toxic, radiant or immunological injury to bone marrow stem cells
Anemia
reduction in the total number of erythrocytes/decrease in quality/quantity of hemoglobin
Relative anemia
normal total red cell mass with disturbances in regulation of plasma volume
Absolute anemia
actual decrease in numbers of RBCs
what causes RBCs to sickle with SCD
low O2 levels
what causes extrinsic hemolysis
drugs, toxins, antibdies, trauma, mechanical factors
What does the Mean corpuscular volume test tell us
volume of the average RBC
what two things do you use to classify hemolytic anemias
whether the hemolysis cause is intrinsic or extrinsic
whether hemolysis is inside or outside the vascular compartment
what is intravascular hemolysis caused by
complement fixation in transfusion reaction
mechanical injuires
toxic factors
describe the lab results for someone with thalassemia
low Hb
low MCV
what is pancytopenia
low RBC, WBC and platelets
Standard lab values for MCV
85-100 fL/RBC
why are those with aplastic anemia susceptible to infections
neutropenia
why are those with aplastic anemia more susceptible to bleeding
low levels of platelets
what is fanconi anemia
genetic aplastic anemia - causes pancytopenia due to defects in DNA repair
what ages are most affected by aplastic anemia
young 15-25
or old >60
what causes extravascular hemolysis
RBCs become deformed and cannot transverse capillaries
abnormal RBCs killed by macrophages in the spleen
15% of aplastic anemia cases are due to what
exposure of high doses of radiation, chemicals and toxins
what are the symptoms for mild anemia
typically nothing
what are the two morphological classifications of anemia
according to the MCV and MCHC
what does the mean corpuscular hemoglobin tell us
Red cell mass
what signs does aplastic anemia show
petechia and purpura due to low platelet function
aplastic anemia is rare, but is the most common form of what kind of anemia? (MCV and MCHC)
normocytic
normochromic
what are 4 inherited genetic defects causing anemia
abnormalities of hb synthesis
inc RBC hemolysis and destruction
structural abnormalities
inherited enzyme deficiencies
what are some symptoms are thalassemia minor
mild to moderate anemia
what is intravascular hemolysis characterized by (4 things)
hemoglobinemia
hemoglobinuria
jaundice
hemosiderinuria
what would you find in the bone marrow of vitamin deficiency anemia
megaloblastic dysplasia
What are the terms for a high, normal and low MCV
macrocytic
normocytic
microcytic
are intrinsic causes of hemolysis hereditary or aquired
hereditary
what causes intrinsic hemolysis
defects of RBC membranes and hemoglobinopathies
RBC indices for hereditary spherocytosis
MCV: normal/slight dec
MCH: norm/slight dec
MCHC: increased
RBC: norm/dec
WBC: normal
Hb: low
Hct: low
what is erythropoietin
hormone that stimulates RBC production in response to low pO2
what is the treatment for IDA
oral iron therapy
iron sulfate - for about 3-6 months
is the reticulocyte count for SCD high or low
high (immature RBCs)
what are some symptoms of major thalassemia
bone deformities
hepato/splenomegaly
cardiac failure
hypogonadism (due to excessive intestinal iron absorption)
what is the most common and most severe form of the hemolytic anemias
sickle cell disease
in IDA, RBCs are ____chromic and ______cytic
hypochromic
microcytic
Describe the RBC indices for B12 or folate related anemia
MCV: inc**
MCH: inc
MCHC: normal/slight dec
Platelets: low
RBC: low
WBC: low
what are the two groups of RBC disorders
Amenia
Polycythemia
Standard lab values for MCHC
31-35g/dL
What does the hematocrit test tell us
percent of total blood volume occupied by RBCs
what is the difference in the shape and qualities of a RBC in hereditary spherocytosis and normal RBCs
spherocytosis:
spherical
rigid
fragile
smaller
what are 4 major symptoms of sickle cell disease
severe anemia
vessel occlusion
pulm/cardio complications
splenomegaly, hepatomegaly
what kind of anemia is sickle cell disease
hemolytic
are extrinsic causes of hemolysis hereditary or aquired
acquired
hemolytic anemais are accompanied by what and why
jaundice
due to high levels of bilirubin
Koilonychia
concave, ridged and brittle nails
what kind of anemia is thalassemia
hemolytic
what does an increase in 2,3-DPG do with regards to anemia
allows RBCs to release oxygen easier - allows for better oxygenation without increasing CO
how is thalassemia classified
by the polypeptide chains with deficient synthessis
without b12 and folate, what happens
DNA synthesis of blast cells is disrupted leading to megaloblasts
what is the most common micronutrient deficiency worldwide
iron
what are 4 specific Iron deficiency anemia symptoms
Pallor
Koilonychia
Glossitis
Pica
What does the Hemoglobin test tell us
number of grams of Hb per liter of blood
glossitis
bald, fissured tongue appearance
what does it mean for something to be hypersegmented
have more than the normal number of lobes
75% of aplastic anemia cases are due to what
autoimmune disease against hematopoietic stem cells
what lab results for MCV, MCH, MCHC would you find for vitamin deficiency related anemia
increased MCV, MCH
normal MCHC
how does chronic kidney failure lead to anemia
it leads to impaired EPO production
4 anemias related to decreased RBC production
aplastic anemia
anemia of chronic renal failiure
vitamin b12 or folate deficiency
iron deficiency anemia
Describe the RBC indices for Iron Deficiency Anemia
MCV: low
MCH: low
MCHC: low
Platelets: varies
RBC: norm/slight dec
WBC: norm/slight inc
Hb: low
Hct: low
what is neutropenia
low levels of neutrophils in blood
what are the two kinds of aplastic anemia
acquired
Familial
how can hemolytic anemias be caused
congenitally
acquired (immune response)
Standard lab values for hematocrit test
M:40-50%
F:37-47%
pernicious anemia
autoimmune disease - antibodies against parietal cells and IF
What does the mean corpuscular hemoglobin concentration test tell us
grams of hemoglobin per 10mL of RBCs