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what is a normal RBCs count?
4.5 to 5.5 million/mm3
what is the normal Hgb?
11.5 to 15.5 g/dL
what is normal Hct?
35% to 45%
what is he ANC?
the precent of neutrophils/bands times WBC count
what is normal ANC?
greater than 1000/mm3
what are general signs and symptoms associated with hematologic diseases?
tired/lethargy, abnormal breathing, pallor, increased rates of infections
what is occurring in microcytic anemia?
the RBCs are too small; they can not carry as much oxygen
what are examples of microcytic anemia?
iron deficiency, thalassemia
what is occurring in normocytic anemia?
RBCs are the correct size, but have a structural defect
what are examples of normocytic anemia?
sickle cell
what is occurring in macrocytic anemia?
RBCs are too large
what are examples of macrocytic anemias?
folate deficiency, B12 deficiency, aplastic
when does screening for iron deficiency anemia begin?
9-12 months
what are some complications of untreated iron deficiency anemia?
cognitive and motor delays
how is an infant tested for anemia?
fingerprick test
who is at risk for iron deficiency anemia?
premature infants, infants on formula that is not iron fortified, infants breastfeeding exclusively for more than 4 months, teenage girls
why are premature infants more likely to develop iron deficiency anemia?
most iron is obtained in the last few few weeks of pregnancy
what should you educate a patient on when they are taking oral iron supplementation?
drink through a straw to prevent teeth straining; take with citrus juice on an empty stomach to help with absorption
what lab values should be monitored while receiving iron supplementation?
hemoglobin and hematocrit
what suggestions could be made to increase iron in the diet of an individual who is vegetarian or vegan?
increase intake of dark leafy greens
a child on iron supplementation complains that their stomach hurts and says that their poop has been black and sticky. what do you tell this child?
this is normal when on iron
when should you recheck iron after starting iron supplementation?
6 weeks
how much iron should girls and boys 9-13 intake?
8 mg/day
how much iron should a boy aged 14-18 intake?
11 mg/day
how much iron should a man 18-21 intake?
8 mg/day
why to boys need an increase in iron between 14-18 years of age?
to support the myoglobin needed for the increased muscle mass
how much iron should a girl 14-18 get a day?
15mg/day
how much iron a day should a girl 19-21 receive a day?
18 mg/day
why do girls need more iron than boys starting at age 13?
mensuration increase risk for iron deficiency
a group of inherited blood disorders where Hgb synthesis is impaired -
thalassemia
what is the most common type of thalassemia?
beta
what group is thalassemia more common in?
Mediterranean descent
what is the issue with the RBCs in thalassemia?
they have a short life span - only about 10-20 days
what is a common complication of thalassemia?
hepatosplenomegaly (the shorter half life means they will have to break down more RBCs in a shorter time period)
a child presents with wide, thick cranial bones, pallor, and failure to thrive - what do you suspect?
thalassemia
what causes the bizarre shape of RBCs in beta thalassemia?
bone marrow makes a large amount of immature RBCs to compensate
when will a child start displaying symptoms of thalassemia?
1st year of life
what is the most common treatment for thalassemia?
blood transfusions every 2-3 weeks
what is the normal life expectancy of a child with thalassemia?
untreated die young; about 20 with treatment
who is more likely to have sickle cell disease?
African Americans (higher risk in those with west African descent)
what are some triggers that could cause a sickle cell crisis?
infection, stress, dehydration
what causes the pain in a sickle cell crisis?
the lack of oxygen reaching tissues
what are common manifestations of pain in a sickle cell crisis?
joint pain and headaches
what is the first intervention for an individual in a sickle cell crisis?
oxygen
what is a common complication associated with the splenic veins being clogged with cells?
functional asplenia
what is a normal hemoglobin result in a newborn screening?
A
what is a hemoglobin associated with sickle cell found in the newborn screening?
S
what is a hemoglobin associated with thalassemia found in a newborn screening?
F
along with normal anemia symptoms, what are some additional signs and symptoms of sickle cell anemia?
delayed growth, delayed puberty
tender/swollen hands and feet is a sign of sickle cell seen in infants and is referred to as?
hand-foot syndrome
some examples of vaso-occlusive crisis that can occur in an individual with sickle cell include -
stroke, heart attack, priapism, and acute chest syndrome
what is a sign of an individual with sickle cell developing acute chest syndrome?
respiratory distress
this occurs when there is excessive pooling of blood in the liver or spleen
sequestration crisis
what is a big risk for an individual with sickle cell who is in a sequestration crisis?
shock
what is an aplastic crisis?
lack of RBC production
what does an aplastic infection most commonly happen in an individual with sickle cell?
after infection
what infection are individuals with sickle cell at a high risk for?
strep
what are two medications commonly given to individuals with sickle cell?
penicillin and hydroxyurea
what extra immunizations are recommended for sickle cell patients?
pneumococcal and meningococcal
due to abnormal expression or injury to the bone marrow, they body is unable to produce enough blood cells
aplastic anemia
what other issues could cause the body to develop aplastic anemia?
leukemia and lymphoma
a child often has SOB, bruises easily, struggles with bruising easily, and is smaller than normal - what do you suspect?
aplastic anemia
what is done to diagnosis someone with aplastic anemia?
bone marrow aspiration
when treating an individual with aplastic anemia what is always your first intervention?
treat symptoms first (i.e. give oxygen for SOB)
what are common treatments for aplastic anemia?
platelets/PRBCs, bone marrow transplant, immunosuppressive therapy
bleeding disorder where there is a deficiency of a factor needed to coagulate blood -
hemophilia
what is the most common type of hemophilia?
von Willebrand’s disease
hematomas, hemarthrosis, hematuria, and epistaxis is commonly associated with -
hemophilia
what are some precautions for a small child with hemophilia?
no playing sports (especially contact), wear a helmet, good oral helmet
what should you educate a patient with hemophilia look out for?
blood in the stool (sign of GI bleeding)
that is the most common treatment for children with hemophilia?
replacing the clotting factor they are missing
what medication is given to help prevent bleeding in individuals with hemophilia?
desmopressin acetate
what should you educate a child with hemophilia on when they receive an injury?
RICE (rest, ice, compression, elevation)
in this type of anemia the factor that allow platelets to adhere to the injured blood vessel is missing -
von Willebrand’s disease
what is a common manifestation of hemophilia in women?
heavy menstrual cycles
what are the two treatments given for von Willebrand’s disease?
desmopressin and plasma-derived vWF/vWF concentrates
what is the most common cause of acute onset thrombocytopenia in children?
immune thrombocytopenic purpura (ITP)
when is ITP most common?
1-4 years
when is someone more likely to have issues with ITP?
late winter and spring
what most commonly triggers ITP?
virus
a child presents with a purpura and petechial rash, hemorrhagic blisters of the mucosal membranes, and ecchymoses - what do you suspect?
ITP
what do the labs of an individual with ITP look like?
platelets will be low (under 20,000) but all other labs are normal (Hgb, WBC, and differential)
what is the normal treatment for ITP?
mostly supportive (prevent injury, manage bleeding episodes) - disease will resolve within 6 months normally
what are some medications that can be given if ITP is severe enough?
prednisone or IV immunoglobin (IVIG)
what would indicate a need for a platelet transfer in an individual with ITP?
hemorrhage
when does DIC normally occur?
following another trauma
what is DIC?
excessive clotting and bleeding throughout the body
what occurs first in DIC?
excessive amounts of thrombin are produced leading to abnormal clotting in small vessels
what happens secondly in DIC?
all the clotting factors are used up and fibrin interferes with platelet aggregation, leading to excessive bleeding
what is a a common sign of DIC?
excessive bleeding from every orifice
what is given initially in to treat DIC?
heparin - interferes with clotting process
what is given during DIC to help prevent excessive bleeding?
platelets, clotting factors, and fibrinogen
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