Peds - Lecture 14 (Hematology/Oncology)

What is the normal lower limit of Hgb for children 6 mo to < 2 years?

11.0

What is the normal lower limit of Hgb for children 2-6 years?

11.0

What is the normal lower limit of Hgb for children 6-12 years?

11.2

What is the normal lower limit of Hgb for children 12-18 years?

F - 11.4

M - 12.4

What is the definition of anemia?

any hemoglobin or hematocrit value that is 2 standard deviations below the mean for age and sex

What are the clinical features of acute anemia in children?

poorly compensated state, more symptoms

What are the clinical features of anemia of chronic disease in children?

growth failure or poor weight gain,

What are the clinical features of chronic hemolytic anemia?

prominent cheek bones, frontal bossing, and dental malocclusion, splenomegaly and jaundice may be present,

What are the clinical features of anemia in children with infiltrative disease of the bone marrow?

LAD and hepatosplenomegaly

What may be present in patients with anemia?

flow murmur

What is the most common cause of anemia in children?

iron deficiency

What are the risk factors for iron deficiency anemia?

cow's milk - toddlers, infants

menstruating adolescent females

dietary - toddlers, low SES

What are the clinical manifestations of iron deficiency anemia?

CNS - irritability, poor concentration

cardiac - murmur (systolic), elevated HR

fatigue, pallor, poor exercise tolerance, HA, syncope

When do we screen for anemia?

12 mo !

What is the workup for iron deficiency anemia?

1: if Hgb low on capillary sample --> repeat with venipuncture

-if hx sugg of IDA --> limited eval (CBC +/- ferritin)

-if not --> CBC, reticulocyte count, ferritin, peripheral blood smear, and stool for occult blood

generally, when should you start w/u for anemia?

6 mo - < 5 y.o - Hgb < 11

5 - < 12 y.o - Hgb < 11.5

How do you dx iron deficiency anemia?

Hgb < 11 with low serum ferritin, confirmed by trial of iron therapy

How do you treat IDA?

therapeutic trial of oral iron (3 mg/kg/d of ferrous sulfate), recheck CBC w/in 4 weeks - if no improvement check for compliance and obtain full w/u and consider referral to hematology

if improved - continue and recheck at 3 mo

AND diet changes

When should you consider thalassemia in children?

if anemia NOT responding to iron supplementation

What is the w/u for thalassemia?

general w/u for anemia

AND hgb electrophoresis to confirm

refer to genetics and hematology

What is the most common inherited hemolytic anemia in the US?

hereditary spherocytosis

What is the w/u for hereditary spherocytosis?

CBC and reticulocyte count, spherocytes on blood smear, positive osmotic fragility test, coombs neg, genetic studies to confirm

refer to genetics and hematology

What is sickle cell anemia?

autosomal recessive inherited disorder, sickle point mutation in beta globin gene, Hgb SS

What is the clinical presentation of chronic sickle cell anemia?

jaundice, pallor, variable splenomegaly in infancy, a cardiac flow murmur, and delayed growth and sexual maturation

What is the clinical presentation of acute pain episodes in sickle cell anemia?

vaso-occlusive events, sickling phenomenon is exacerbated by hypoxia, acidosis, fever, hypothermia, and dehydration

What is the main clinical finding in children with sickle cell anemia?

dactylitis - hand-foot swelling in early infancy

How do you diagnose sickle cell anemia?

routine newborn screen --> confirmation by hgb electrophoresis

Who do you refer patients with sickle cell anemia to?

pediatric hematologist, ophthalmology starting at age 10, genetics at new dx and reproductive counseling

Who manages patients with sickle cell anemia?

Hematology:

-hydroxyurea is mainstay of tx

-transfusions, infection prevention, nutrition, transcranial US (yearly from age 2-16), pain management, transplant and gene therapy

What is sickle cell trait?

heterozygous for sickle gene, typically asx, very specific conditions can lead to sickling (high altitude and exertional rhabdomyolysis)

When do you screen for Lead poisoning?

12 AND 24 months

T/F: repetitive small ingestions are much worse than one large exposure in lead poisoning

TRUE

What is the clinical presentation of a child with lead poisoning?

vague sx - weakness, irritability, WL, vomiting, personality changes, ataxia, constipation, HA, colicky abdominal pain

pica - eating non-food items

late - cognitive impairment, convulsions, coma with elevated ICP

What is the workup for lead poisoning?

lead level - if elevated capillary level (≥3 mcg/dL) --> venipuncture to recheck

CBC and ferritin

What is the actionable lead level indicating tx?

≥3.5 mcg/dL

What is the treatment if lead level is <45 mcg/dL?

no chelation, notify public health, screen other children in home, follow-up screening

What is the treatment if lead level is ≥45 mcg/dL?

chelation therapy, refer to pediatric environmental health speciality unit or regional poison control center,

*hospitalize if >69 mcg/dL

How do you prevent lead poisoning?

screen! primary prevention is the only way to reduce neurocognitive effects of lead poisoning

What are the three most common cancers in children and adolescents?

1. leukemia

2. CNS tumors

3. lymphoma

What malignancies are almost exclusive to children?

neuroblastoma, Wilm's tumor, retinoblastoma, hepatoblastoma

What is the most common childhood cancer? When is typically seen?

Acute lymphoblastic leukemia (ALL), B cell or T cell

peaks age 2-4, M>F

What childhood cancer involves translocation involving chromosomes 9 and 22?

chronic myeloid leukemia (CML)

Which childhood cancer is seen in the neonatal period and adolescence and accounts for about 20% of leukemia in kids?

acute myeloid leukemia (AML)

What are the typical presenting symptoms of leukemia in children?

fever, pallor, petechiae +/or ecchymoses, lethargy, malaise, anorexia, and bone/joint pain

What is the seen in the w/u for leukemia?

CBC w/ smear - immature blasts

bone marrow aspiration/bx - immature blasts

How do you diagnose leukemia?

definitive dx requires evaluation of cell surface markers by flow cytometry, cytogenic studies

Who do you refer patients with leukemia to?

oncology

What is the treatment for leukemia?

ALL - chemo

AML - chemo, poss stem cell transplant

CML - tyrosine kinase inhibitor (TKIs), poss chemo

What is cancer of lymphoid tumors? What are the two main subtypes?

lymphoma, hodgkin lymphoma (HL) and non-hodgkin lymphoma (NHL)

What is the typical clinical presentation of lymphoma?

most often - PAINLESS, firm LAD

supraclavicular and cervical nodes, mediastinal LAD - cough, SOB, B-cell type NHL - fever, night sweats and WL

How do you diagnose lymphoma?

tissue or fluid sample

bx - reed sternberg cells

Who do you refer patients with lymphoma to?

oncology

How do you treat lymphoma?

HL - poss sx, chemo/radiation

NHL - less localized, chemo, poss sx, radiation, bone marrow transplant, metastases common

What is the leading cause of childhood cancer deaths?

CNS tumors

What is the clinical presentation of CNS tumors?

impingement on normal tissue - irritability, anorexia, poor school performance, and loss of dev milestones

increase in ICP (obstruction of CSF flow or direct mass effect) - HA, vomiting (in am), and lethargy

What is involved in the w/u of CNS tumors?

MRI - imaging of choice

LP - AFTER MRI

Who do you refer patients with CNS tumors to?

oncology

How do you treat CNS tumors?

depends on type of tumor, size, location and associated symptoms, dexamethasone, surgery, +/- chemo/radiation

What is the most common malignancy in infancy?

neuroblastoma

What is the clinical presentation of neuroblastomas?

if localized - asx

if mets - systemic illness, fever, WL and pain, non-tender mass in abdomen, flank or neck, if spreads to bones of orbit - bruising around eyes

What is involved in the w/u for neuroblastoma?

CBC, abdominal xray, urine catecholamines - tumor markers: urine homovanillic acid (HVA) and vanillylmandelic acid (VMA)

Who do you refer patients with neuroblastoma to? treatment?

oncology - based on risk classification, surgery, +/- chemo/radiation

What is the most common intraocular tumor in children?

retinoblastoma

There is a _________ increased risk for osteosarcoma for individuals with hereditary retinoblastoma

500-fold

What is the clinical presentation of retinoblastoma?

leukocoria (white pupillary reflex), stabismus, nustagmus and red eye

if not treated - globe destroyed and mets - death

Who do you refer retinoblastomas to?

ophthalmology and oncology

How do you diagnose retinoblastoma?

dilated eye exam and imaging

What is the most common malignant renal tumor of childhood?

Wilms tumor (nephroblastoma)

What is the clinical presentation of a Wilms Tumor?

abdominal mass, abdominal pain, fever, HTN, and hematuria

Who do you refer Wilms Tumor to? Treatment?

oncology

nephrectomy (can be curative), chemo, +/- radiation

What tumor has an increased risk with prematurity?

hepatoblastoma

What is the clinical presentation of a hepatoblastoma?

abdominal mass, abdominal pain, N/V

What is involved in the w/u for hepatoblastoma?

CT or MRI

tumor marker - serum alpha-fetoprotein (AFP)

LFTs - normal/slightly elevated

What are the two types of sarcomas?

soft tissue cancers

bone cancers

-Ewing Sarcoma (peaks age 10 and 20 years)

-osteosarcoma (adolescents)

Who is at an increased risk for sarcomas?

NF-1, retinoblastoma (hereditary), prior radiation or chemo

What is the clinical presentation of sarcomas?

depends on site, mass effect and mets

fatigue, fever, WL

orbital tumor - periorbital swelling, proptosis and limited EOMs

osteosarcoma - epiphysis and metaphysis of long bones pain and associated palpable mass

ewing sarcoma - femur and pelvis

What is involved in the w/u for sarcomas?

XRAY - lytic lesions

Mets/staging (MRI, chest CT and bone scan) or PET scan

What should you consider in a patient with an abdominal mass?

neuroblastoma, hepatoblastoma or Wilms tumor

What is the CP of malignant lymph nodes?

large, firm, fixed, and nontender

T/F: almost all cancers require a bx of some type to make a definitive dx

True!

What are the tumor markers for neuroblastoma?

urine homovanillic (HVA) and vanillylmandelic acid (VMA)

What is the tumor marker for hepatoblastoma?

serum alpha-fetoprotein (AFP)

What are s/sx you should NEVER ignore in a patient?

s/sx of increased ICP

leukocoria

abdominal mass

painless, firm, fixed, nontender masses

abnormal bruising/bleeding

persistent bone pain

WL