Peds Hem/Onc Flashcards
What is sickle cell disease?
Vaso-occlusive phenomena and hemolysis are the clinical hallmarks of sickle cell disease (SCD). Can lead to acute and chronic pain and tissue ischemia or infarction. Splenic infarction leads to functional hyposplenism early in life.
Presentation of sickle cell disease?
recurrent painful episodes (previously called sickle cell crisis
abnormality seen in sickle cell disease?
sickle point mutation in the beta globin gene results in the production of sickle hemoglobin. HbS
Complications of sickle cell disease?
infxs
severe anemia
vaso-oculsive phenomena: stroke, MI, dactylitis, lots more
Tx of sickle cell disease?
vaccinations
abx prophylaxis: first 3 months of life- 5yrs
folic acid, MVI without iron replacement vit D and Ca
Hydroxyurea
When would you give a blood transfusion to a child with sickle cell disease?
therapy (typically for life-threatening SCD related comp) or for prophylaxis, to decrease the incidence of specific SCD related complications
What is the gold standard for assessment of pain in sickle cell disease?
individual’s report
What is henoch-schonlein purpura?
Inflammatory vasculitis, Immunoglobulin A vasculitis
Presentation of henoch-schonlein purpura?
usually 3-15 y/o
distinct rash, arthritis, GI, renal
Work up for Henoch-Schönlein purpura?
CBC, CMP, ESR, Coags, UA
Rare Skin Bx or Renal Bx
Tx of Henoch-Schönlein purpura?
sxs pain control
nephro consult if renal involvement
surg consult if worrisome abd pain (intussusception common)
What is Idiopathic Thrombocytopenic Purpura?
aka Immune ThrombocytoPenia (ITP)
immune-mediated thrombocytopenia (peripheral blood platelet count <100,000/microL)
IgG directed to patient’s own platelets
MC cause of thrombocytopenia in children
Epidemiology of ITP?
peak in kids 2-5yrs
can present at any age
Presentation of ITP?
sudden appearance of a petechial rash, bruising, and/or bleeding in an otherwise healthy child
look at mucosa & gingiva
ITP is usually seen after…
viral infection, in otherwise healthy child
If you see low platelets + other abnormalities on CBC, should you suspect ITP?
NO, not if there are other abn. on CBC
management of ITP?
Hem consult
activity restriction
avoid antiplatelets/anticoags
regular monitoring of platelets
+/- steroids, IV IG
(usually spontaneously recovering 3 mos)
What is the MC malignancy of childhood? epidemiology?
ALL
peak 4 yrs
M >F
caucasians > African American
14x risk in Down’s pts
survival 85&
pathogenesis of ALL
Proliferation of immature lymphocytes.
Leukemia = >25% malignant hematopoetic cells (blasts) in the bone marrow aspirate.
s/s of ALL?
Fever, bleeding, bone pain (esp. long bones), lymphadenopathy
Hepatosplenomegaly
rare: testicular enlargement
unexplained cytopenias, atypical cells, blasts
Dx of ALL? Tx?
bone marrow examination: immature lymphoblasts
chemo
Risk factors for AML?
Children w/exposure to ionizing radiation, benzenes, Down’s at increased risk
less common than ALL
S/s of AML?
same as AML
Fever, bleeding, bone pain (esp. long bones), lymphadenopathy
Hepatosplenomegaly
rare: testicular enlargement
unexplained cytopenias, atypical cells, blasts
Dx of AML?
bone marrow biopsy
Tx of AML?
very aggressive and includes intense chemotherapy, and in some cases HCT, and has high rates of acute toxicity, especially infections
less responsive to chemo, px 65-75% 5 yr survival
Epidemiology of CML?
MC in teens and adults
genetic abnormality seen in CML?
Philadelphia chromosome (translocation of chromosomes 9 & 22) fusion of BCR gene on 22 & ABL gene on 9 > fusion protein
s/s of CML?
bone pain, nt sweats, fever, fatigue or Asymptomatic
Severe Sx: dyspnea, priapism, neurologic findings
Dx of CML?
predominance of myeloid cells on peripheral smear, basophils high, low blasts
Tx of CML?
hydroxyurea (eliminates Ph+ cells)
bone marrow transplants; Imatinib mesylate (Gleevec)
tyrosine kinase inhibitor (TKI);
targeted TKIs: dastinib, erlotinib, nilotinibm, ponatinib
px of CML?
20 yrs. old with bone marrow transplant from matched donor – 70%-80%, unmatched 50%-60%
What is lymphoma?
Refers to a malignant proliferation of lymphoid cells
Usually arising from lymphoid tissues (lymph nodes, thymus, spleen
epidemiology of hodgkin’s disease?
Rare in kids < 5
most common childhood cancer in the 15- to 19-year-old age group
bimodal peak: adolescents and >50yo
What are the 4 types of hodgkin’s disease?
Nodular sclerosis (NS)
Mixed cellularity (MC)
Lymphocyte depleted (LD)
Lymphocyte rich (LR)
Hodgkin’s disease almost always presents at…
the site above the diaphragm
s/s of hodgkin’s disease?
unexplained weight loss of more than 10% body weight
persistent/recurrent fever
night sweats
MC sign: painless cervical or supraclavicular adenopathy
mediastinal mass causing cough/SOB
What is used to stage hodgkin’s disease?
ann armor staging system
Dx of hodgkin’s disease?
H&P
imaging: CXR, CT chest/abd/pelvis, PET
tissue bx: reed sternberg cells*
labs: CBC w/ dif, ESR, renal, LFTs, LDH, UA
tx of hodgkin’s disease?
comprehensive peds oncology center
chemo +/- radiation
What is non-hodgkin lymphoma?
consists of a diverse group of malignant neoplasms of the lymphoid tissues variously derived from B cell progenitors, T cell progenitors, mature B cells, or mature T cells
NHL may present with?
potentially emergency complications
i.e. pericardial tamponade, acute airway obstruction
s/s of NHL?
Varies
sxs develop quickly, usually over one to three weeks
commonly presents as enlarging, non-tender lymphadenopathy or as sxs due to the compression of surrounding structures
What are the different types of non-hodgkin’s lymphoma?
burkitt lymphoma
diffuse large b cell lymphoma
t cell lymphoma
anaplastic large cell lymphoma
What is a neuroblastoma?
spectrum of neuroblastic tumors (including neuroblastomas, ganglioneuroblastomas, and ganglioneuromas) that arise from primitive sympathetic ganglion cells
have the capacity to synthesize and secrete catecholamines
Where do neuroblastomas arise from?
anywhere throughout the sympathetic nervous system
s/s of neuroblastomas?
presenting sxs reflect the location
abd mass, abd pain
periorbital ecchymosis
anemia
bone pain
etc.
work up for neuroblastoma?
CBC CMP urine or serum catecholamine metabolite levels VMA HVA Ferritin lDH Biopsy
Staging of neuroblastoma?
International Neuroblastoma Risk Group Staging System (INRGSS)
need: CT or MRI, I123 MIBG
Avg. age of pt presenting with wilms tumor?
2-5 yrs
How are wilms tumors discovered?
usually incidentally by child/parent
HTN- 25%
can rupture after minor/major abd trauma, causing life threatening situation
Work up for Wilm’s tumor?
US CT/MRI UA CBC CMP, LDH, Uric acid AFP, hCG Coags
Rhamdomyosarcomas: Head and neck RMS are MC in ____.Extremity tumors present MCly in ___.
younger children
adolescents
Histology of rhabdomyosarcoma?
to that of other small round blue cell tumors of childhood that involve bone and soft tissue
Dx of rhabdomyosarcoma?
tissue dx
no FNA - not enough tissue
Describe osteosarcomas
uncommon primary malignant tumor
characterized by production of osteoid or immature bone by the malignant cells
Epidemiology of osteosarcoma?
bimodal
early teens (13-16 y/o) -adults over 65
Presentation of osteosarcoma?
localized pain, typically of several months’ duration
PE: soft tissue mass
10-20% have metastatic disease, most often in the lung
Dx of osteosarcoma?
xray: 1st dx test
- Codman’s triangle
- “sunburst” pattern
biopsy- definitive
tx for osteosarcoma
surg
chemo
(radiation usually not helpful)
Where do Ewing’s sarcomas usually arise?
in the long bones of the extremities
Presentation of Ewing’s sarcoma?
Localized pain or swelling of a few weeks’ or months’ duration. Nighttime bone pain.
trauma
fever, fatigue, weight loss, anemia
work up same as osteosarcoma
S/s of retinoblastoma
White pupillary reflex (leukocoria) is most common sign.
1/3 are bilateral
may seen strabismus
deadily if untreated, rapid mets
tx of retinoblastoma?
Enucleation (if large tumor)
chemo, laser reduction
px overall 95% survival
What is important about sickle cell crisis?
it HURTS
be liberal with pain meds
HSP can have…
renal failure and or surgical abdomen
labs seen in ITP?
low PLTs, at risk for life threatening bleeding
lymphadenopathy where is never normal?
SUPRACLAVICULAR
EPITROCHLEAR
Philadelphia chromosome is seen in___
CML
Reed sternberg cells are seen in ___
Hodgkins
When might you see a child with raccoon eyes?
Neuroblastoma
What may cause incidental acquired von willebrand?
wilms tumor
