EXAM 3 HEM/ONC Flashcards
RBC norm values and description
4.5-5.5
indirectly estimates Hgb and reflects function of bone marrow
Hgb norm values and description
11.5-15.5
Can tell if the RBCs are carrying a lot/a little Hgb if you compare it to RBC count.
Hct norm values and description
35-45%
Percentage of RBCs that make up whole blood. Generally is 3x Hgb content
WBC norm values and description
4.5-13.5
more important to know differentials so you can see what type of infection you’re fighting.
Ex:
Neutrophils: bacterial
Eosinophils: allergens, parasites, cancer
Basophils: Contain histamine, heparin, and serotonin to help win fight
Lymphocytes: Make antibodies
Monocytes: Infantry of immune system
Platelet norm values and description
150-400
Can tell if pt can clot well or not
Normal lifespan of RBCs
120 days
At what well-child check is it appropriate to check hemoglobin
1 year old
A Hgb below __ is considered too low for children
10-11g/dL
What are the 3 classifications of anemias
Decreased RBC production
Increased RBC loss
Increased RBC destruction
Examples of Decreased RBC production
Nutritional deficiency or bone marrow failure
examples of Increased RBC loss
epistaxis, hemophilia, hypersplenism
Examples of Increased RBC destruction
Intracorpuscular (sickle cell, thalassemia, membrane deficits)
Extra corpuscular (chemotherapy, infection, immunization)
Symptoms of Increased RBC destruction
yellow eyes, dark urine, big liver
Symptoms of Increased RBC loss
hypotension, decreased peripheral pulse
Symptoms of Decreased RBC production
pica, systolic murmur
Why do you check for iron deficiency at specifically 1 year of age?
They wean from fortified bottles/cereals that are fortified with iron. They usually start to drink too much cows milk which makes them iron deficient and fat
how should a child take iron supplements
2-3 divided doses between meals with Vit C
Sickle Cell Anemia is a …..
hemoglobinopathy
The cause of Sickle Cell Anemia is
a Autosomal recessive disorder
Describe pathology and symptoms of sickle cell disease
Sickle cells stick to each other and cause blockages at bifurcations.
Sx include stroke, paralysis, pneumonia, pulm hypertension, atelectasis, heart failure, anemia, priapism, osteomyelitis, big liver/spleen, gallstones, necrosis of tissue that lost flow d/t blockage, blindness
How do you manage sickle cell disease
Prevent sickling
Rest and minimize energy expenditure
Hydration
Electrolyte replacement
Analgesia
Blood replacement
Antibiotics
What causes a Sickle Cell Crisis
Anything that increases body’s need for oxygen or alters transport of oxygen
Trauma
Infection, fever
Physical and emotional stress
Increased blood viscosity caused by dehydration
Hypoxia
to treat:
Rest to minimize energy loss
Hydration through oral or IV therapy
Electrolyte replacement
Analgesia for pain
Blood replacement for anemia
Antibiotics for infection
What are the 5 types of Sickle Cell Crisis
vaso-occlusive, splenic-sequestration, aplastic crisis, CVA, and acute chest syndrome
Describe vaso-occlusive type of Sickle Cell Crisis
Most common crisis
Painful
Stasis of blood with clumping of cell 🡪 microcirculation 🡪 infarction
S/S; fever, pain, tissue engorgement
Describe splenic-sequestration type of Sickle Cell Crisis
Life threatening: death can occur within a few hours
Blood pools in spleen
S/S: profound anemia, hypovolemia, and shock
Describe aplastic crisis type of Sickle Cell Crisis
Diminished production and increased destruction of RBCs
Triggered by viral illness or depletion of folic acid
S/S: profound anemia, pallor
Describe CVA type of Sickle Cell Crisis
Severe, unrelieved HA
Severe vomiting
Seizures
Behavioral changes
Weakness or inability to move
Unsteady gait
Slurred speech
Vision changes
Describe acute chest syndrome type of Sickle Cell Crisis
Severe chest, back or abd pain
Fever
Very congested cough
Dyspnea, tachypnea
Retractions
Declining O2 sats
What is Thalassemia
Anemia from defective synthesis of Hgb, structural impairment of RBCs, and shortened life of RBCs
Dx by visualizing Overabundance of erythrocytes found in the bone marrow
Affects mainly descendants of people living near the Mediterranean sea. Infants become symptomatic at 6 weeks (anemia/growth failure) and have low hgb and hct.
Bone marrow transplantation is potential cure
What are the 4 types of Thalassemia
Thalassemia minor: asymptomatic silent carrier
Thalassemia trait: mild microcytic anemia
Thalassemia intermediate: moderate-to-severe anemia plus splenomegaly
Thalassemia major (also known as “Cooley’s anemia”): severe anemia necessitating transfusions to survive
What is Hemosiderosis
excessive iron deposits- esp lungs and kidneys- r/t chronic transfusions
s/s: chronic fatigue, severe exercise limitation, growth failure
Treat with iron-chelating drugs such as deferoxamine (Desferal). Drugs bind excess iron for excretion by kidney
Hemosiderosis is a common complication of
Therapeutic Management of Thalassemia through excessive blood transfusions
What are the 2 types of Aplastic Anemia
Pancytopenia: simultaneous depression of all formed elements of the blood
-Profound anemia
-Leukopenia
-Thrombocytopenia
Hypoplastic anemia: profound depression of RBCs but normal white blood cells (WBCs) and platelets
What is the difference between primary and secondary aplastic anemia
Primary (congenital)
Secondary (acquired)
How do you treat aplastic anemia
Immunosuppressive therapy
Bone marrow transplantation
What is Hemophilia
A group of hereditary bleeding disorders that result from deficiencies of specific clotting factors
Mom carries gene (x-linked recessive)
What is the difference between Hemophilia A and B
Hemophilia A
“Classic hemophilia”
Deficiency of factor VIII
Hemophilia B
Also known as “Christmas disease”
Caused by deficiency of factor IX
Von Willebrand disease
Deficiency of von Willebrand factor and factor VIII
How do you Dx Hemophilia
History of bleeding episodes
Overt prolonged bleeding
Hemarthrosis (bleed into joint)
Ecchymosis
X-linked inheritance
Laboratory findings
Low levels of factor VIII or IX, prolonged partial thromboplastin time
Normal: platelet count, parathormone level, and fibrinogen level
Therapeutic Management of Hemophilia
Replace missing clotting factors
Aggressive replacement therapy with factor concentrate
Home infusion
Desmopressin (DDAVP)
IV administration or nasal spray
Causes two to four times’ increase in factor VIII activity
Used for mild hemophilia
Aminocaproic acid
Prevents clot destruction
Care Management of Hemophilia
Prevent bleeding
Safe environment
Dental hygiene
Recognize and control bleeding
RICE
Prevent the crippling effects of bleeding
Support the family and home care
Genetic counseling
What is Immune Thrombocytopenia
An acquired hemorrhagic disorder characterized by:
Thrombocytopenia: excessive destruction of platelets
Purpura: discoloration caused by petechiae beneath the skin
Normal bone marrow with increased number of immature platelets or eosinophils
Usually acute but can become chronic if it lasts over 12 mo
Often follows upper respiratory or other infection
What are the clinical manifestations of Immune Thrombocytopenia
Easy Bruising
-petechiae
-ecchymoses
-most often over bony prominences
Bleeding from Mucous Membranes
-epistaxis
-bleeding gums
-internal hemorrhage evidenced by the following:
-hematuria
-hematemesis
-melena
-hemarthrosis
-menorrhagia
-hematomas over lower extremities
Management of Immune Thrombocytopenia
Primarily supportive:
IV immuno-globulin
Anti-D antibody
What can Recurrent or severe episodes of nosebleeds mean
Vascular abnormalities, leukemia, thrombocytopenia, clotting factor deficiency diseases (von Willebrand disease and hemophilia)
____ therapy prevents perinatal transmission of HIV
HAART
HIV Virus takes over ______ lymphocytes
CD4+T
Therapeutic management of HIV/AIDS
Antiretroviral drugs:
Slow virus growth
Prevent/treat opportunistic infections
Nutritional support
Symptomatic treatment
What are clinical manifestations of HIV
Malnutrition, short stature, cardiomyopathy
Name some acute reactions to blood transfusion
Hemolytic: the most severe, but rare
Sudden severe HA, chills, shaking, fever, N/V, red or black urine, tight chest
Febrile reactions: fever, chills
Allergic reaction: urticaria, pruritus, laryngeal edema
Air emboli: may occur when blood is transfused under pressure
Difficulty breathing, sharp pain in chest, apprehension
Hypothermia
Chills, decreased temp, irregular HR, possible cardiac arrest, Nausea diarrhea
Name some delayed reactions to blood transfusion
-transmission of infection (jaundice, etc)
-hepatitis
-HIV
-Malaria
-Syphilis
-Bacterial or viral infection
When both parents have sickle cell trait, what is the chance that each of their children will have sickle cell anemia?
25%
What are some early signs of cancer in children
Fever and pain common in childhood, leading to easily missed problem
Unexplained loss of energy
Sudden vision changes
Excessive, rapid weight loss
anemia, firm and enlarged lymph nodes
ecchymosis, petechiae, spontaneous bleeding
What Laboratory tests can help confirm the presence of cancer
CBC, LFTs, coagulation studies, urinalysis, Lumbar puncture, bone marrow aspiration, CT, MRI, PET, MIBG
What are some ways to get ahead of side effects of cancer treatment
monitor neutrophil count to make sure it doesn’t go lower than 500/mm3
Keep in mind that Usual signs of infection and inflammatory response are limited
Prevention of infection is a priority so use prophylactic antibiotics and use
When would you give a platelet transfusion to a cancer pt
Platelet transfusions generally reserved for active bleeding not responding to basic treatment measures
Repeated transfusions increase risk of sensitization and antigen formation
Teach to avoid activities that may cause injury, bike, skate boards, contact sports
Name some Side Effects of Cancer Treatment
Hemorrhage, Anemia, N/V, Altered nutrition, mucosal ulceration, neurological problem’s, Hemorrhagic cystitis
How do you decrease nausea and vomiting during cancer tx
Synthetic cannabinoids are being used in children
Administer antiemetic 30 minutes before chemotherapy begins and routinely for 24 hours after chemotherapy
How do you monitor nutritional status of cancer patients
Monitor height, weight routinely, monitor prealbumin, transferrin and albumin
What should a cancer patient try to eat
High protein, high calorie, full-fat rather than reduced fat options
How do you prevent Hemorrhagic cystitis
Prevented with at least 1 ½ times recommended daily fluid needs
Encourage frequent voiding
Watch for dysuria or hematuria
how long does it take hair to regrow after cancer treatment is complete
3 to 6 months
Steroid use during cancer treatment can result in
Cushingoid appearance (moon face) so Avoid salt intake to reduce fluid retention and wear Loose-fitting clothes
T/F: Cancer kids should still get their vaccines on time
F: Only the inactivated vaccines, not the Varicella/MMR
What is the most common form of childhood cancer
Acute lymphoid leukemia (ALL)
more often in hispanic boys. might begin prenatally
What is Leukemia
A broad group of malignant diseases of bone marrow and lymphatic system
Unrestricted proliferation of immature WBCs in the blood-forming tissues of the body
Liver and spleen are the most severely affected organs
Leukemia demonstrates the same neoplastic properties as solid tumors
Classifications are increasingly complex
Describe the onset of leukemia
Can be acute or insidious onset
Well child incidental, or illness that they don’t seem to recover from, or could be presenting like other conditions (RA)
Name the most common characteristic of leukemia
Although leukemia is an overproduction of WBCs, often acute form causes low leukocyte count
Greatly elevated immature cells, or blasts
Immature cells do not attack and destroy normal blood cells
Cellular destruction takes place by infiltration and subsequent competition for metabolic elements
Describe the cycle of leukemia
Bone marrow makes too many WBC blasts (babies) which never mature and become helpful but compete for the same resources other cells need. Eventually the baby WBCs take up so much nutrition that the bone marrow can’t make cells anymore d/t lack of nutrients necessary to manufacture. This results in:
-anemia (No RBCs)
-infection (no mature WBCs)
-bleeding (no platelets)
Then your bones weaken and start to fracture. This results in
-bone pain
-fever
-pallor
-fatigue
-hemorrhage
-joint pain
Name the sites of disturbance for leukemia pts.
Spleen, liver, and lymph glands demonstrate infiltration, enlargement, and fibrosis
CNS all messed up:
Cranial nerves
Spinal nerves
How do you definitively Dx Leukemia
Bone marrow aspiration or biopsy.
Once Dx, do a lumbar puncture to evaluate central nervous system (CNS) involvement
What places in the body are considered “sanctuaries” for leukemic cells?
CNS and testes
What factors influence the prognosis of a child with leukemia
Age and WBC count at diagnosis, CNS involvement, testicular involvement, Down syndrome, sex, race and ethnicity, and nutritional status; cell characteristics; response to initial treatment
What are the Four types of standard treatment for leukemia
Chemotherapy, radiation therapy, chemotherapy w/stem cell transplant, and targeted therapy (TKIs, monoclonal antibodies, proteasome inhibitor therapy)
What are the Three phases of therapy
remission induction (4-5 weeks), consolidation/intensification, maintenance
What are the four standard treatments for cancer
chemotherapy, radiation therapy, chemo w/ stem cell transplant, and targeted therapy (tyrosine kinase inhibitors/TKI)
How do you determine complete remission
presence of less than 5% blast cells in the bone marrow and no detectable abnormal blood cells in the extramedullary sites
Describe CNS prophylactic therapy
Used in high risk children to reduce the risk of leukemic cells taking over the CNS
Describe Reinduction after relapse
Each relapse means poorer prognosis
Testes are resistant to chemotherapy and may lead to relapse
Relapse in testes leads to intensive chemotherapy and irradiation
Describe Bone marrow transplantation
Used in both ALL and AML
What are the two types of lymphomas
Hodgkin disease
More prevalent among children 15 to 19 years of age
Non-Hodgkin Lymphoma
More prevalent among children <14 years of age
Describe HODGKIN DISEASE
Affects LYMPH NODES more than lymph itself.
Pt is positive for STERNBERG-REED CELLS in lymph nodes
Sx: giant neck lymph nodes, recurrent cough
Not as common in children. Herpes (EBV) thought to play a part. Often metastasizes to spleen, liver, bone marrow, lungs, and other tissues. STAGING IS IMPORTANT
Describe NON-HODGKIN LYMPHOMA
Occurs more frequently in children than Hodgkin disease
Disease usually diffuse rather than nodular. Cell type undifferentiated or poorly differentiated. Occurs early, often, and rapidly
STAGING NOT AS IMPORTANT.
Sx more like leukemia
Use aggressive chemo to fix
What is the Most common solid tumor in children
brain tumor
Name some clinical manifestations of a brain tumor
Hydrocephalus
Headache
Vomiting
Nystagmus
Ataxia
Dysarthria
Diabetes insipidus
Growth failure
What 3 things do you need to asses after surgery to remove a brain tumor
assess pupils = ICP changes
Bleeding – assess dressing and mark
Colorless= CSF and needs to be reported immediately
What is the most common cancer diagnosed in infancy
NEUROBLASTOMA
Describe a NEUROBLASTOMA
Originate from embryonic neural crest cells
Primary site is in abdomen
(Firm, nontender, irregular mass in the abdomen)
BUT CAUSES
Neurological impairment
Respiratory obstruction
Paralysis
Periorbital edema
Exophthalmos
Supraorbital ecchymosis
To treat you remove as much as you can surgically, cover the cavity in radiation, and finish with chemo.
most common type of bone tumor
Osteosarcoma
Describe Osteosarcomas
Most are curable
Sx: localized bone pain, palpable mass on bone, limping/can’t hold heavy objects
Likely arises from bone-forming mesenchyme
Found mostly in lower extremities near growth plate
Usually remove affected area for biopsy, remove affected bone and replace with prosthetic, use chemo to reduce tumor size. If they can’t salvage an entire bone, they’ll amputate.
Describe a EWING SARCOMA
Arise in marrow spaces
Originates in shaft of long and trunk bones
Ex: Pelvis, femur, tibia, fibula, humerous, ulna, vertebrae, scapula, ribs, skull
Therapeutic management
Limb salvage or amputation depending on situation
Radiotherapy
Chemotherapy
If a limb is irradiated to treat bone cancer, you should…
Wear loose fitting clothes over irradiated area
Protect irradiated area from sunlight and sudden temperature changes
Most common kidney tumor of childhood
WILMS’ TUMOR
Painless swelling or mass in abdomen
Firm, nontender, confined to one side, deep within flank area
Often discovered during routine hygiene cares
Surgery usually occurs within 24 to 48 hours of diagnosis. DO NOT PALPATE THE TUMOR. Handle infants carefully. Manipulation may disseminate the cancer
If unfavorable histology, prognosis is poorer
Survival rates highest among all childhood cancers
Describe a RETINOBLASTOMA
Whitish “glow” in the pupil
Staging done with ophthalmoscope and then surgically removed AND/OR Surgical implantation of iodine-125 for radiation
Photocoagulation
Long-term concern is secondary tumors
Describe some long-term effects of cancer treatment
Radiotherapy can decrease growth in bones or reproductive organs
Treatments may cause hormonal dysfunction
Growth and Tanner stages are important to document
Assess all body areas for abnormalities based on treatment used
