Lecture 9: Hematology/Oncology Flashcards
Difference b/t ALL and AML (acute leukemia)
- prevalaence + peak ages
ALL
- MC (ALL kids have ALL)
- peak 2-4 yrs
AML
- less common
- peak: < 2 yrs
What d/o incr your risk of acute leukemia
- imunodefic syndromes
- DNA repair/Repair Syn
- Down Syndrome
Lung, skin, and GI organ s/s of Acute Leukemia
Lung –> SOB
Skin –> easy bleeding/bruising, petechiae
GI organs enlarged (liver/spleen)
3 Main things seen on labs a/w Acute Leuknemia
What are these things d/t?
Which 2 most commonly seen on labs
- Anemia (Normocytic, normochromic)
- Neutropenia
- Thrombocytopenia
- most have 2+3
blast cell proliferation in bone marrow –> decr production og RBCs, WBCs, Plts
What may be seen on PBS that is a/w Acute Leuknemia?
Seen on Tumor Lysis labs?
Seen on XR?
+/- circulating blast cells
incr K, Ca, P, LDH, Uric acid, Cr (d/t breakdown of leukemia cells)
+/- pleural effusions, mediastinal mass
What is used to determine type of leukemia/Tx? how?
Flow Cytometry determine type of leukemia/Tx by biological markers
Difference b/t Tx of ALL and AML (intensity, bone marrow transplant)
Which type is remission more common in? relapse?
ALL
- less intensive induction
- b. marrow transplant = rare
- remission more common
AML
- more intensive + toxic
- b. marrow transplant = common
- RELAPSE more common
- GH defic
- HL
- Heart damage
- 2nd CAs
- Abn bone/muscle growth
- infertility
- Cognitive defects
- Psych issues
- Low thyroid fxn
- Reduced lung fxn
- Obesity/metabolic Syn
Late effects of childhood CA
What type of lymphoma is MC?
Which is curable in most?
MC = NHL
Hodgkin’s Lymphoma = curable in most most
Difference in presentations b/t HL and NHL?
HL
- central/mediastinal LNs
- Reed Sternburg cells
NHL
- peripheral LNs
- Starry Sky histology
What is the MC S/s for brain tumors?
combo of what 2 Sxs together = more reliable for Brain tumor
Other s/s:
- N/V
- Visual Field defecit
- Endocrine dysfx
- seizure, gait abn
HA
HA + Neuro Sxs = more reliable for Brain tumor
Main imaging modality of choice for brain tumors
What would suggest marrow infiltration
Tx = surg, radiation, chemo
Do infants have good or poor prognosis?
MRI = best for brain tumors
bone pain or abn CBC suggests marrow infiltration
infancy = poor prognosis w/brain tumors
Where does neuroblastoma arise from
primitive neuroblasts in neural crest tissue
How does presentation of neuroblastoma differ before and after age 1?
< 1
- tumors above diaphragm/localized
- better prognosis
> 1
- MC = tumor in abd
- most has widespread dz
What can be seen on plain films w/neuroblastoma?
What labs are usu elevated in most pts?
Stripped calcifications on XR
Elevated urinary catecholamines in most pts
Tx of neuroblastoma:
- low risk
- intermed
- high
- low risk –> surgery
- intermed –> surgery + chemo
- high –> multimodal
Where is Wilm’s tumor located? MC age range? more commonly unilateral or bilateral?
tumor in kidneys
MC b/t 1-5 y/o
More commonly unilateral
MC presentation of Wilm’s tumor
Others:
- HTN
- Gross hematuria
- Fever
Asx (not painful) abd mass
describe constipation in Wilm’s tumor and whats seen on XR
Common lab finding?
Tx = multimodal
how long to f/u? why?
constipation
- doesnt resolve w/tx
- on XR = “shifting bowel”
Anemia
F/u for 8 yrs –> look for spread to other kidney
Where is osteosarcoma MC common? more specific?
age group most common in?
MC Sxs
Labs?
What is required for Dx?
MC at metaphysis of long bones (area of bone growth)
- distal femur = MC
MC in adolescence
Sx = Pain
HIGH AlkP + LDH
Need Bx for Dx
Imaging of Osetosarcoma:
- what is characteristically see on XR
- what imaging is used for looking for METs? MC sites?
XR –> sunburst rxn
MRI –>look for METS
- MC in lungs, other bones
Why is radation therapy not used for osteosarcoma?
What are the 2 surgical Tx options?
radiation wont work b/c osteosarcoma tumors are radioresistant
Surgery
- amputation
- limb sparing w/prosthesis
What is cause of Retinoblastoma? result?
More commonly uni or bilateral?
MC before what age
Heritable mutation/deletion of RB1 tumor suppressor gene –> uncontrolled tissue growth in eye
More commonly bilateral
MC before age 2
Main Sign of Retinoblastoma?
- meaning of sign?
3 other signs?
Main sign = leukocoria
- absent red reflex
- Strabismus
- Proptosis
- Neuro Sxs
3 Tx option for Retinoblastoma?
- Radiation
- Chemo
- *Enucleation
When is retic count high?
When is the retic count low?
High retics w/incr RBC destruction
Low retics w/RBC production problem
MCV (mean cell/corpuscular volume) stands for?
How is MCV in newborns? why?
How to calculate norm MCV in young kids
MCV (mean cell/corpuscular volume) = avg size of the RBCs
MCV is high in newborns d/t fetal Hb –> decr over couple months
upper limit of norm MCV in kids = 70 + age
incr RBC destruction:
- Hemoglobinopathies
- RBC membrane defects
- Enzyme Defects
- immune mediated
examples
- ..
- RBC membrane defects= Speherocytosis, Elliptocytosis, Pyropoikilocytosis
- Enzyme Defects = G6PD or Pyruvate Kinase Defic
Pt has unconjugated hyperbilirubinemia, + DAT/coombs test and + maternal Ab screening and hemolysis on PBS (incr retics, macrocytosis, polychromasia) What is Dx?
Immune mediated Hemolytic Dz
If D antigen is present on surface of RBCs what does that tell you about the pt?
Pt is Rh+
in Anti-Rhesus (Rh) Dz what causes hemolytic anemia in the neonate/newborn
Maternal sensitization
What occurs during maternal sensitization
Why is not a problem in the pregnancy that the sensitization occurs in?
When is most maternal sensitization? Other time can occur?
small amts of FETAL blood transferred to MOTHER across placenta during pregnancy –> mother forms IgM Abs
- not a problem b/c IgM Abs dont cross placenta –> baby not infected
most in 3rd trimester
- also during delivery (incr amt of fetal blood exposed to mother)
Why are babies of subsequent pregnancies at risk for Rh-Dz? result?
IgG Abs produced by mother –> can cross placenta –> Hemolytic Dz of the Newborn
What is the fetal response to maternal sensitization in susequent pregnancies? (3)
- Fetal RBC’s get coated w/maternal IgG –> erythroblastosis –> destruction of fetal RBCs
- Anemia –> stim erythropoiesis
- Extramed erythropoiesis–> HSM
How to prevent Rh-Dz and immune mediated hemolytic dz of newborn?
GIVE ALL Rh (-) women Rhogam
- anti-D immunoglobulin
When is Rhogam given to Rh (-) women? (2 instances)
How does it work to prevent immune mediated hemolytic dz?
- at 28 and 34 wks gestation
- Postpartum if baby is Rh+
Fetal blood crosses placenta to mother–> Ab attaches to infant blood
- mother’s body destroys Ab-coated RBCs before she creates Abs to it
A women comes in who has had a previous maternal sensitization but not given rhogam during that pregnancy and now has Rh Abs. She is pregnant again, can you give her Rhogam? why y/n?
No
- Rhogam can only be given if mother doesnt have Abs
What is the predominant feature of Hemolytic Dz of newborn/ABO compatability….?
Hyperbilirubinemia in first 12
to 24 hours of life
How does ABO incompatibility differ from Rh-Dz?
No prior exposure is needed w/ABO incompatability
- body automatically makes Abs to what you dont have
Two ways to manage hemolytic Dz of the Newborn, what are they aimed at preventing?
- Antepartum therapy
- prevent Hydrops Fetalis
( > 2 abn fetal fluid collections) - Postpartum therapy
- prevent kernicterus
(acute bilirubin encephalopathy)
How to prevent Hydrops Fetalis w/ antepartum therapy (2)
- Intrauterine transfusion
2. Early delivery
Elevated lab findings in Iron Deficiency Anemia?
TIBC, transferrin
everything else is LOW
Ferritin, MCV, Hb, MCH, MCHC, retics, RBCs
MC cause of Fe defic in toddlers? what is this d/t?
inadequate dietary intake of iron
- d/t excessive cow’s milk intake
2 Tx options for Iron deficiency anemia?
Other edu/Tx?
How long to treat?
- Liquid preps - elemental iron
- Polysaccharides prep - Niferex, Nu-Iron
Edu –> NO MILK, incr dietary iron
Tx til iron labs normalize then 2-3 mo after to replete stores
3 main causes of bleeding d/o and their assoc tests?
- Vasoconstriction problems
- no testing for - Primary hemostasis problems
- plt count/aggreg, vWF studies - Secondary hemostasis problems
- PT/PTT, fibrinogen
4 common Sxs of Bleeding d/o?
Others:
- hematemesis
- melena
- hematuria
- hemoptysis
- Bruises
- Petechiae
- Bleeding
- Deep muscle + joint bleeding
Pt presents w/ bleeding from large vessels, subQ hematoma, Hemarthosis, Intramuscular hematoma. What type of bleeding is this?
Secondary Hemostasis bleeding
Presentation of Hemophilia in peds pts (5)?
- Bleeding from circumcision
- Prolonged bleeding w/heel stick
- multiple, raised palpable bruises
- Bleeding from IM inj
- Swollen tender jionts after minor injury
What is peds palliative care
interdisciplinary approach w/with curative, restorative and life prolonging Tx
What 3 things does peds palliatvie care focus
- relieve suffering
- slow dz progression
- Improve QoL
