HEME/ONC Flashcards
Mother’s is blood type O And baby is a, B, or AB
ABO incompatibility Is the incompatibility between the ABO blood group of the fetus and the mother
Symptoms of ABO incompatibility
Mild cases asymptomatic
Can have jaundice of the skin, sclera, gums or mouth
Janice usually occurring within the first 24 hours a wife up to one week; may have Hepatosplenomegaly
Diagnostic test for ABO incompatibility
Coombes test this is a direct anti-globulin test or DAT
-Positive and 13% of cases
Hemoglobin moderately low
Elevated indirect Bili level
Presence of antibodies an infant and maternal serum
Management of ABO incompatibility
Monitor indirect bilirubin levels
Phototherapy if indicated
If anemia severe may require exchange transfusion
Mother’s is RH negative and baby is Rh positive
Rh incompatibility the incompatibility between Rh boy group of the mother in the fetus
Relatively uncommon; becomes more severe with each pregnancy if untreated
Causes hemolysis, resulting in anemia and high bilirubinemia; severe cases causes fetal death, jaundice, hepatosplenomegaly
Signs and symptoms of RH incompatibility
Diagnostic test for RH Incompatibility
Blood type Mom RH negative, baby Rh positive
direct Coombes test—-positive
Hemoglobin low, hemodialysis often continues up to three months
Serum indirect BiliRubin —-Elevated
When should administration of Rh immunoglobulin be given?
After any invasive procedure during pregnancy, after termination of each pregnancy (including miscarriage/or abortion)
When is an RH Isoimmunization screen performed?
First prenatal visit
If mom is RH negative, test father; if father is Rh positive, pregnancy is at risk
Risk for problems increase with each pregnancy as antibody levels rise
Treatment for Rh incompatibility
Anti-natal treatment once diagnosis has been established, transfusion a fetus with Rh negative blood
Postpartum treatment
Photo therapy with exchange transfusion if needed
Transfusion of PRBC‘s if indicated by hemoglobin
Studies show efficacy of gamma globulin but no recommendation at this time
Microcytic, normocytic, macrocytic so what type of RBC indices ? And what are their values?
Mean corpuscular volume MCV “CYTIC”
Determine size of the RBC
Microcytic: ~<80
Normocytic:~ 80 to 100
Macrocytic: ~100
Determine the size of the RBC
Mean corpuscular volume
Determines color of the RBC
Mean corpuscular hemoglobin concentration MCHC
Determines weight of the RBC
MCH
What is the normal MCH of an RBC ? What does MCH measure?
Measures weight
Normal 26 to 34
Hypochromic
Less than 32
MCHC color
Normochromic
32 to 36 MCHC color
RDW Is helpful how
Red cell distribution with
Differentiates between IDA, thalassemia, ACD
RDW in IDA is _____.
Increased
RDW in ACD is ____.
Normal
RDW and thalassemia is _____.
Normal or slightly increased
What is the reticulocyte count
Number of new, young RBCs in circulation
Index of bone marrow health in response to anemia
Bone marrow failure send new baby RBCs increases retic count
Increase in hemorrhage or hemo- lysis
Normal reticulocyte count is ____ to ___%.
1 to 2%
Microcytic/hypochromic anemia’s are?
IDA, thalassemia, PB poisoning
Normocytic/normochromic anemia‘s include
ACD, acute blood loss, early IDA
Microcytic, hypo chromatic anemia due to an overall deficiency of iron
Iron deficiency anemia IDA
Anemia caused by decreased iron intake, increase needs, or slow gastrointestinal blood loss
Iron deficiency anemia
In infancy a micro hemorrhage from the gut due to an early Intake of whole milk before the age of nine months can cause what
Iron deficiency anemia
Because does not have the proteins a breakdown cows milk
In toddlers iron deficiency is often due to what
Increase reliance on whole milk at the expense of solid foods
And adolescents Iron deficiency is due to what?
Diet practice is contributing to an adequate intake of iron, specially in girls after monarchy
Easily fatigued, palpitations, lethargy, headaches, pica, delayed motor development, pale dry skin, brittle hair, tachypnea, tachycardia, flat brittle or spoon shaped nails
IDA symptoms
In IDA hemoglobin and hematocrit are_____. MCV is ____. MCHC is ____.
RDW is _____. RBCs are _____.
Serum ferritin is _____ ug/L, because it is the iron tagged for storage.
In IDA hemoglobin and hematocrit are low.
MCV is low, size of RBCs.
MCHC color of RBCs is low.
RBCs are low.
Increased red cell width >17.
Increased total arm binding capacity (TIPC) I can take all the iron you can give me.
Low serum ferritin <30 , iron that is tag for storage, FIRST VALUE TO FALL
What increases the absorption of iron
Orange juice
How long do RBCs live
120 days (three months)
Management of iron deficiency anemia
Maintain breast-feeding for the six months use supplemental iron drops or iron fortified cereal by 4 to 5 months
Use iron fortified formula until one year if NOT BF
Elemental iron 3 to 6 mg/KG/day in 1 to 3 doses until hemoglobin normalizes
Then,
Replace iron stores: 2 to 3 mg/kg/day x 4 months Time until RBCs can replace stores
What is the relationship of iron and hemoglobin
Hemoglobin binds to 4 02— transporter to all the tissues in the body
Iron binds to O2 helps carry oxygen to the tissues
Autosomal recessive genetic disorder the second most common microcytic anemia
Thalassemia
Group of hereditary disorders characterized by abnormal synthesis of alpha and beta genes globulin chains
Thalassemia
Alpha 4 genes and beta 2 genes
One or more of each gene can be missing; type is determined by which ones are missing alpha versus beta; severity depends on Number of genes affected
Pale or bronze color to skin, tachycardia, tachypnea, Weakness,Hepatosplenomegaly Characteristic faces (expose your frontal teeth, frontal bossing)
Symptoms of thalassemia
Thalassemia diagnostics
CBC shows low hemoglobin/low MCV/hypochromic RBCs/increased reticulocyte count
Increased total Bilirubin
Increase ferritin
Refer to hematologist
At what age do children be screened for anemia
Nine and 12 months, additionally if a child is at risk
Awesome recessive genetic disorder characterized by hemoglobin S variant
Sickle cell disease
Hemoglobin S and a single base pair of the beta globular gene; resulting in distorting RBCs into classic crescent shape when deoxygenated
Sickle cell pain crisis symptoms
Vasoinclusive acute event
Most often bones but can occur in any part of the body; children less than two years usually in hands or feet dactylitis
Swelling sometimes seen outside of pain, low-grade fever
Symptoms of sickle cell infection
Fever, malaise, anorexia, poor feeding
Symptoms of sickle cell splenic sequestration
Weakness, irritability, unusual sleepiness, Pilnäs, in large spleen, fast heart rate, pain in left side of abdomen
A plastic crisis is associated with what
Parvovirus B 19
Prenatal diagnosis of sickle cell disease
DNA through chorionic villus sampling
9-11 weeks
Or amniocentesis 11-17 weeks
Diagnostics for sickle cell
Hemoglobin electrophoresis and hematologist results, evidence of splenic sequestration (hemoglobin low, platelets low);A plastic crisis (hemoglobin below baseline, reticulocyte count <1.0%)
Management of sickle cell disease, should be directed towards_____\of complications again preventing ____.
Management focuses on prevention of complications in crisis precipitating factors:
administering all immunizations, additional pneumococcal vaccine every 5 to 10 years,
Prophylactic penicillin from 2 months to 5years of age
Increased risk for infection with encapsulated bacteria due to functional asplenia, therefore any fever greater than 101.5°F needs eval!!! Blood culture, broad abx
Cumulation of toxic amounts of lead blood in the body
Lead poisoning; a whole blood lead level (BLL) >5ug/Dallas
Sources of lead exposure
Lead based paint an older homes built prior to 1978 and those especially built before 1950
Lead contaminated soil and dust from automobile emission of gasoline
Lead contaminated drinking water related to pipes
Lead-based paint on important items like toys, certain folk remedies(Asian/Hispanic) cosmetics and other items
Children between what ages are at highest risk for lead poisoning
One to three years
Symptoms of lead poisoning
Low exposure may be asymptomatic
Mild—— gastroenteritis (anorexia, N/V), constipation, diarrhea, abdominal pain, sleep disturbance, metallic taste in mouse, limp pain, headache
Severe—-lethargy, difficulty walking, cognitive impairment, personality change
90% children will have pica
Bradycardia, papilledema, ataxia, neuropathy
Burtonian lines
Blueish discoloration of the gingival border scene in lead poisoning
Screening for lead poisoning is done when
Those deemed at risk living homes built before 1978; universal screening for Medicaid
Poor
Living in innner city
A lead level of____ is considered lead poisoning
> 5 ug/dL ; <5 is not blood poisoning per CDC
Lead level of ____ is a medical emergency
> 70 ug/dL
CLASS 5!
Hospitalization
Lead level of _____will require Chelation therapy
> 45 ug/dL-69
Class 4
Requires chelation therapy
Lead level class
class I: <10 —— f/u 6 months-1 year Class IIA: 10-14 —— f/u 3 month Class IIB: 15-19 ——f/u 2 month Class III: 20-44 —— f/u 1 month Class IV: >45 Class V: >70
X-linked recessive bleeding disorder
Hemophilia A
Pleading disorder caused by congenital deficiency or absence of the Connie factor V I I I or IX
How does hemophilia affect males versus females
Only females are carriers; occurs in 1:7000 males
Male disorder
FEMALE CARRIER:
25% risk of having an infected son with each pregnancy
25% risk of having a carrier daughter
25% chance of having a non-carrier daughter or healthy son
Signs and symptoms of hemophilia
Easy bruising injection sites
Prolonged bleeding following circumcision
Mucosal bleeding
Prolonged bleeding in any part of the body
Bleeding in joint spaces
Swelling
Pain in joints
Diagnostics for hemophilia
Prenatal diagnosis can be done by Fetal blood sampling through the Periumbilical blood PUBS
@18-20 weeks gestation
Via fetoscopy; chorionic villus sample 9-11 weeks; amniocentesis 11-17weeks
Direct assay of plasma factor activity level for hemophilia a and B
Screening includes :
- activated partial thromboplastin time (APTT)—-prolonged
- PT ——normal
- Bleeding time ——normal
Management and treatment of hemophilia
Interdisciplinary approach
Hemophilia treatment center will receive factor replacement therapy:
Hemophilia A—— Factor VIII concentrate intravenously; B—-Factor IX
Regular prophylaxis of infusions given to prevent joint hemorrhage and bleeding episodes, desmopressin, PT, surgery
Glucose six phosphate Dehydrogenase (G-6-PD) deficiency
X linked or autosomal recessive genetic disorder in which activity of the reD cell enzyme G6PD is decreased or absent causing a hemolytic anemia
Babies May Experience hyperbilirubinemia —-neonatal jaundice
***Inability to deal with stress causing hemolysis!
Signs and symptoms of G6PD deficiency
Weakness, pale appearance, severe cases may have blood in urine/yellow discoloration of skin
Management of G6PD
Identification of appointments of food and drugs that caused hemolysis :
- Drugs: aspirin, sulfonamides, antimalarial‘s, nitrofurans
- Food: fava beans,
- Infection
Transfusion if needed
Diagnostics for G6PD
Fluorescence screen
RBC indices: Heinz bodies present, fragmented cells, Reticulocytosis, hemoglobin normal
Inherited hemorrhagic disorder characterized by a defective due to the quantitative or qualitative abnormalities Of VWF which helps form blood clots
Von Willebrand’s disease
Nose bleeds, bleeding gums, heavy menstrual cycles, prolonged losing from cuts, increased bleeding after trauma or surgery, bruising
Signs and symptoms of von Willebrand’s disease
Treatment of von Willebrand’s disease
Desmopressin as a Tate used to treat bleeding complications or as a preop
Anti-fibrinolytic agents —— helps continuous bleeding usually amicus membranes (nose, mouth, throat)
Autoimmune condition where the body produces its own antibodies to destroy its own thrombocytes and platelets—— resulting in small bleeding spots against the skin “purpura”
ITP idiopathic thrombocytopenia Purpura
Immune mediated disorder characterized by production of antiplatelet antibodies
Sometimes related to sensitization by viral infection
Primary ITP
Platelet count of 100,000/MM3 in the absence of other causes
Chronic ITP
Disease lasting for more than 12 months; approximately 10% of children with ITP
common in children >7 years; prevalent in females
Persistent ITP
Disease that continues between three and 12 months from diagnosis
Secondary ITP
All other forms of immune mediated thrombocytopenia except for primary ITP
Diagnostics for ITP
CBC generally they only require test
Hemoglobin normal or slightly reduced with prior bleeding
Platelet counts
- less than 20,000/mm3 DX acute ITP
- <100,000/mm3 for <12 months Dx chronic ITP
WBC normal
Bleeding time Test (unnecessary) always have normal if platelets < 50,000
Management and treatment of ITP
Controversial
- Acute ITP:
Treatment unnecessary platelet count >50,000
TX if <20,000/ bleeding / can’t have protective enviro
Pharm:
A.high dose corticosteroids/ prednisone 2-5 mg/kg/day 1-3 week
B.IVIG 1g/kg/day
C. Anti-D immunoglobulin 50 MCG/KG
- Chronic ITP—-refer to hematologist
Splenectomy, rituximab( monoclonal therapy) , low dose maintenance steroid, pharm
Patient and family education for ITP
Avoid all competitive contacts for second result in trauma or ruptured spleen
Avoid aspirin & NSAIDS
Monitor for signs of a occult bleeds
Seek medical care if signs of bleeding are noticed
Acute lymphocytic leukemia (a LL) has a peak incidence of ____years
4 years of age
Acute myelogenous leukemia (AML) occurs primarily in what age group?
Malignant hematological cancer occurs primarily an older children
Leukemia signs and symptoms
Anemia, Pale, listless, Irritable, chronically tired, frequent infections, bleeding, lymphadenopathy, hepatosplenomegaly, bone and joint pain
Leukemia diagnostics
CBC with differential—-presence of The blast cells peripheral blood smear highly suggestive
WBC, platelet, and reticulocyte count
Bone marrow aspiration/biopsy =required confirmed diagnosis
Leukemia management
Chemo 1 to 3 years
CNS prophylaxis (radiation, combine intrathecal chemo)
Patient family education
Relapse = bone marrow transplant recommendation
Hold all immunizations until 6 to 12 months after therapy
Any fever requires hospitalization for neutropenia
Fever in leukemia patient requires what
Hospitalization for neutropenia
How long do you hold immunizations for a child diagnosed with leukemia
6 to 12 months after therapy
Neuroblastoma
Neoplasm of the permanent cells from the sympathetic nervous system
Possible familial pre-deposition
Most common malignancy in infancy; 10:1mill births
Neuroblastoma symptoms
Poor feeding, pale, weight loss, abdominal pain, weakness, irritability, listlessness, lymph node enlargement, hepatomegaly, proptosis (raccoon eyes), scalp nodules
abdominal/flank mass
Diagnostics for neuroblastoma
CT
Or MRI to determine location and suspected mass
Tissue biopsy to confirm
Serum or urine catecholamine levels—increased
Mangement of Neuroblastoma
Surgery
Radiation therapy/chemo
Bone marrow transplant for high-risk
Immune mediated therapy/monoclonal antibody
Immunizations held while on therapy for 6 to 12 months after completion of therapy
Congenital malignant intraocular tumor
Retinoblastoma
Hereditary (germinal mutation in 40%); acquired somatic mutations
What are age range for children with retinoblastoma?
Dx usually before 5 years
Leukocoria
Yellow white pupillary reflex most common presentation of retinoblastoma
Diagnostics for retinoblastoma
Fundoscopic examination: creamy pink mask, white avascular tumor mass
CT to evaluate extent of tumor and optic nerve or bony structure involvement; MRI for optic nerve invasion; ultrasound
Treatment of retinoblastoma
Refer for surgery, radiation therapy/laser therapy, chemo
Anticipatory guidance for parents with children with cancer
Anticipatory guidance on developmental issues such as sleep, toileting, discipline, and childcare
Lymphomas
Malignant proliferation of cells; includes Hodgkins and non-Hodgkin’s lymphoma
Related to genetic predisposition, environment, Epstein bar virus
Peak Age for Hodgkin’s lymphoma
15 to 35 years; greater than 50 years
Peak Age for non-Hodgkin’s lymphoma
5-15 years
Hodgkin’s lymphoma symptoms
Painless, firm swelling of lymph nodes
Fatigue
Decreased appetite and attention weight loss 10%
Unexplained fever
Drenching night sweats
Non-Hodgkin’s lymphoma symptoms
Asymptomatic if not disseminated
Difficulty swallowing, breathing, or cough
Swelling in neck, face, upper extremity
Abdominal pain
Distended neck pain/respiratory stress due to superior vena cava syndrome
Which nodes in Lymphoma are often affected and how do they present?
Affected nodes ——often fixed, firm, nontender, discrete CERVICAL/Supraclavicular
Diagnostics for lymphoma
Chest radiograph examine airway patency
Pet scan
Biopsy—-Reed Sternberg cells
CBC with red cell indices; certain ferritin; ESR— increased
Primary malignant renal tumor
Wilms tumor (nephroblastoma)
Peak incidence for Wilms tumor
1 to 5 years
Abdominal mass usually non-painful, firm/confined to one side
malaise, fever, loss of appetite, vomiting, blood in urine
The symptoms are suspected of what?
Wilms tumor
Diagnostic test for Wilms tumor
CBC, urinalysis, CT of abdomen,
Don’t palpate ***ABD/pelvic US
Chest radiograph to determine meta-status to lung
Surgical removal, chemo, radiation treatments
Wilms tumor occurs 3x more in ____. It is more frequent in what gender____?
African Americans; males
Solid tumor in the bone which is malignant
Osteosarcoma
Peak age 15-19 with growth spurt
Localized pain, swelling, mass at the end of a long bone in decreased range of motion in affected extremity
Are signs and symptoms indicative of what?
Osteosarcoma
Osteosarcoma diagnostics
Radiograph of affected bone, MRI, biopsy, CT
Absence of T/B cell function leading to susceptibility to infection from any type of pathogen
Severe combined immuno deficiency (SCID)
Opportunistic infections and infections from any pathogen
Profound lymphopenia is always SCID until proven otherwise
Bone marrow transplant is a standard of care
One day Old child is evaluated in the newborn nursery for fever. A CBC reveals a white blood cell count of 18 with a differential of 82% PMNs, 4% lymphs 9% monos 3% do’s , and 2% with bands.
Which of the following will be the highest on your list for differential diagnosis?
Necrotizing enterocolitis
Maternally transmitted bacterial infection
SCID
TORCH infection
SCID related to profound lymphopenia (low b/t cells) he should be treated as though he has SCID until it is proven not
What test is required to diagnose leukemia?
CBC with diff
Bone aspiration/biopsy
CT
Biopsy of an enlarge lymph node
Bone marrow aspiration/biopsy
Which of the following is not include as a part of initial therapy for ALL?
Chemotherapy
Radiation
Bone marrow transplant
Intrathecal chemotherapy
Bone marrow transplant
Chemotherapy oral, IV, and intrathecal is first line with addition of radiation
Bone marrow transplant is rarely use because most people are cured with chemo alone
Which malignancy is associated with GU abnormalities?
Acute lymphocytic leukemia
Osteosarcoma
Chronic myelogenous leukemia
Wilms tumor
Wilms tumor
Live virus immunization may be resumed after____ months once chemotherapy is completed
12 months
Non-live virus musicians may be resumed @6months
Peak incidence of osteosarcoma age?
4-7 years
8-11 years
12-14 years
15-19 years
15-19 years of age
During growth spurt
Management of a patient with a splenectomy
Pneumococcal vaccine‘s at least two weeks prior to surgery
Prophylactic penicillin
Blood culture and paraenteral anabiotic’s for febrile illness is
Rocephin should be given if a patient appears toxic; febrile illnesses need to be immediately evaluated
Product of RBC breakdown by macrophages — turns to heme and globulin —goes to liver —-excreted via intestines
Bilirubin
Why NB higher risk for hyperbilirubinemia ?
- HCT is increased and RBC breakdown faster
- Liver not fully mature —- doesn’t make enzyme in uterus to turn unconjugated bili to conjugated to be excreted in intestines
Indirect bili
Unconjugated
Direct bili
Conjugated
Hyperbili peaks day 5-7
Physiologic jaundice
Resolves within 10 days
Risk: preterm
Day 7 hyperbilirubinemia , increased sleepiness, fussiness, feeding difficulty
Breast milk jaundice
