hematology

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