Heme/Onc Flashcards
Common types of soft tissue masses in children
Rhabdomyosarcoma
The botryoid variant (sarcoma botryoides), arising within the wall of the bladder or vagina, is seen almost exclusively in infants.
By what age do patients with sickle cell disease usually have functional asplenia
5yo
Who is at risk of acute splenic sequestration crisis and when does it go away
HbSS, HbS beta thal (S Beta-0), HbSC, HbSB+
Goes away when spleen atrophies in HbSS and HbSb0
how much blood to transfuse in Sickle cell crisis
3-5 ml/kg aliquots
low platelet, high mean platelet volume indicates
presence of autoantibodies
risk of administering anti-D immunoglobulin (in the setting of giving in ITP)
worsen anemia
Differential diagnosis in child with anemia and thrombocytopenia
leukemia
when to consider steroids in ITP
when leukemia is not on ddx, in older kids not at high risk of bleeding (because it takes a while to start working)
Causes of isolated neutropenia in infants (before 1yo)
Severe congenital neutropenia
Cyclic neutropenia (every 21d)
both are autosomal dominant
Treatment for febrile neutropenia other than antibiotics
G-CSF
most common childhood cancer
ALL/AML
When should you consider working up a child for leukemia?
unexplained fever, pain, pallor, fatigue, petechiae, bruising, and pathologic fractures
Specific tests for leukemia aside from CBC/BMP
LP
Flow cytometry
Boys: testicular exam
Factors for good prognosis in ALL (Age, WBC, CSF)
Children < 10 and >1 yo
WBC: <50,000
CSF not involved
No testicular disease
ALL treatment
Boys longer tx than girls (risk of testicular mass)
Syndromes associated with increased risk of ALL
trisomy 21
Klinefelter sd
Fanconi anemia
Bloom sd
When is prednisone urgently needed in ALL
mediastinal involvement causing respiratory compromised
Hemophilia A is a deficiency in factor ___
VIII
Most common congenital coagulation factor deficieny
hemophilia A
Female carriers of hemophilia A have symptoms of ___
menorrhagia
Level of factor VIII and risk classification
mild 6-30%
Mod 1-5%
Severe <1%
Triad of thrombocytopenia, eczema, and infections dx
wiskott-aldrich syndrome
X-linked disorder
How to diagnose HLH
an identified genetic mutation OR 5 of the following 8 criteria
- fever
- splenomegaly
- Cytopenia 2/3 blood cell lines
- Hypertriglyceridemia +/- hypofibrinogenemia
- Hemophagocytosis in spleen/bone marrow or lymph node without evidence of malignancy
- low or absent NK cell activity
- Elevated ferritin level
- elevated soluble CD25 (IL-2 receptor) level
Next steps after diagnosing HLH
- genetic testing for primary HLH
- Bone marrow aspirate and biopsy r/o malignancy and look for classic hemophagocytsis macrophage
- LP (see if intrathecal therapy is indicated(
- brain imaging to assess central nervous system disease
- eval of infectious cause of secondary HLH
Autoimmune hemolytic anemia dx
low Hb, nucleated RBC
high retic
IgG + DAT
Tx of AIHA
steroids
vitamin k is essential for factors ____
II, VII, IX, X
When does vitamin K deficiency bleeding occur
newborn to 6 mo of age
What’s the risk of severe VKDH and when does it happen
<1 week of age (early) an can involve intestinal or head bleed
In TLS, what are the levels of potassium, phos, and uric acid?
all high
where are craniopharyngiomas derived from?
pituitary embryonic tissue
Sequelae of tx of craniopharyngiomas
pan hypopituitarism
risk of hypothalamic hyperphagia
Tx of cranipharyngioma
Resection and radiation if not fully resected
Risk of untreated brainstem glioma
herniation and resp failure
Child with severe neutropenia comes to the ED, next steps
cultures and IV abx
Neutropenia, is it innate or adaptive? What are you at risk of developing?
Innate
fungal and bacterial
When to evaluate for immunodeficiencies (7)
- > 2 SYSTEMIC bacterial infections (sepsis, meningitis, osteomyelitis)
- > 2 serious respiratory infections (PNA)
- Multiple bacterial infections (cellulitis, draining otitis media, lynphadenitis)
- Unusual infections involving the liver or a brain abscess
- Infections by unusual pathogens (aspergillus, disseminated candidiasis, serratia, nocardia, bukholderia)
- Infections of unusual severity
- Chronic gingivitis or recurrent aphthous ulcers
Hb electrophoresis for B thalassemia major
HbA 0*
HbA2 elevated
HbF +/-
How to treat/cure beta thal major Hb B-0
blood transfusions
HSCT
How to differentiate between iron deficiency and beta thal on mentzer index (MCV/RBC)
Iron deficiency >13
Beta thal <13
Macrocytic anemia ddx
nutritional (B12/Folate)
Diamond Blackfan anemia
Anemia due to destruction ddx
Intrinsic to RBC: hereditary spherocytosis, G6PD deficiency, sickle cell anemia, pyruvate kinase deficiency
Extrinsic to the RBC: antibody-mediated destruction (e.g. autoimmune hemolytic anemia), Hemolytic uremic syndrome (mechanical)
Transfusion reactions (acute) and how to minimize
Febrile transfusion reaction (cytokine in donor plasma react to antigen on donor leukocyte) – leukoreduction
Allergic
Hemolytic
Bacterial contamination – should send blood product back to culture
Transfusion-associated graft vs host disease (donor lymphocutes recognize host as foreign) – irradiation
When to refer bone pain to Oncology
lytic lesion
Periosteal reaction: Osteosarcoma, Ewing, Osteomyelitis
NO Periosteal reaction: Histiocytic disorder, neuroblastoma
When to NOT refer patient to oncology for bone pain
solitary lesion
eccentric lytic destructive lesion and metaphyseal expansion (non-neoplastic)
DO get CT if it is a sharply marginated radiolucent lesion. May be osteochondroma, osteoid osteoma, osteoblastoma, fibroma
How to treat pain from osteoid osteoma
NSAID
Worsens at end of day, not related to activity
Resect lesion
Boils, recurrent pneumonia, recurrent bacterial infections/abscesses. What do you suspect is the issue?
What if there are family members who have it?
Innate immune system - CHRONIC GRANULOMATOUS DISEASE (CGD)
- issue with oxidative radicals
Neutrophils, monocytes, NK cells
X-linked (all males)
Mutation CYBB
How do you test for CBD/ oxidative burst?
Flow cytometric assessment of oxidative burst
- dihydroxy-rhodamine123 reduction
Mutations in ELANE causes ___
severe congenital neutropenia
What causes HbSS mutation
Replacement of glutamic acid with valine at position 6 of B-globulin
How to diagnose adrenal mass in neonates/prenatally identified adrenal mass
urine vanillymandelic acid (VMA)
homovanillic acid (HVA)
or positive metaiodobenzylguanidine (MIBG) scan
likely neuroblastoma
most common extracranial solid tumor in children
neuroblastoma
Factors that are favorable for neuroblastoma
< 18mo
Lower stage
NO MYCN amplification
DNA index that is not diploid
Heterozygosity at 1p and 11q
Management of neonatal neuroblastoma
reassurance and periodic US
should self-resolve
Serum marker for hepatoblastoma
alpha fetoprotein
Serum marker for germ cell tumor
HCG
Where can neuroblastoma show up
Adrenals
Paraspinal ganglia (near the spine)
Triad of botulinum Toxin
bulbar palsy
Descending paralysis
Clear sensorium
If you see pancytopenia, next step?
Bone marrow biopsy
Differential for pancytopenia – hypoproliferation (6)
Leukemia
Aplastic anemia
Nutritional deficiency
Metastatic cancer infiltrating bone marrow (neuroblastoma/rhabdomyosarcoma)
Fulminant sepsis
MDS
Differential for pancytopenia – increased destruction (3)
Splenomegaly
Paroxysmal Nocturnal Hemoglobinuria (complement mediated)
Acquired hemophagocytic lymphohistiocytosis
Normal MVCs by age (roughly)
Higher than adult range as newborns - 3d >95
2wk >86
2mo >77
3-6 mo >74
NADIR 2-6 yo >70
Side effect of etoposide
AML
MDS
Side effect of daunorubicin (anthracycline)
cardiomyopathy
Side effects of cytarabene and cyclophosphamide
gonadal failure, bladder cancer
Side effects of cytarabene and cyclophosphamide
gonadal failure, bladder cancer
At what serum lead level should a child be started on iron supplementation
> 5
Anticholinergic toxidrome
mad as a hatter
dry as a bone
hot as a hare
red as a beet
blind as a bat (pupillary dilation)
Anticholinergic toxidrome
mad as a hatter
dry as a bone
hot as a hare
red as a beet
blind as a bat (pupillary dilation)
Tx for acute methanol toxicity
fomepizole
