Heme/Onc & Infxns Flashcards
Sickle cell disease MC in ___ and ___ ethnicities
AA; mediterranean
SCD–pt w/ swollen, painful hands/feet
Dactylitis. 2/2 necrosis of small bones
SCD–Excruciating pain in the extremities, ulcers, hip pain
Pain crises. Ischemic damage 2/2 sickling
SCD–Point tenderness on femur, fever, and malaise
Osteomyelitis; MCC salmonella
SCD–seen on blood smear
inc retics, nl MCV sickles, targets, HJ bodies
SCD–acute drop in HCT w/ dec retics
aplastic crisis due to Parvo B19
SCD–recurrent RUQ pain after meals; tx
pigment gallstones; do cholecystectomy
SCD–resp distress & emergent tonsilectomy
Waldyer Ring hyperplasia
SCD–proteinuria and inc creatinine and recurrent UTIs
kidney infarcts due to sickled RBCs
SCD–MCC sepsis
strep pneumo
SCD–pt w/ fever, cough, chest
pain, chills, and SOB; tx
Acute Chest syndrome (pulm infarction = MCC DEATH); O2, abx and exchange transfusion
SCD–MCC death
pulmonary infarction (Acute chest syndrome)
SCD–acute confusion and focal neuro deficits; tx
stroke; EXCHANGE TRANSFUSION (not tPA)
SCD–stroke assessment
transcranial doppler, keep HbS
SCD–vaccination and prophylaxis
2yo–23valent pneumo vaccine, H. flu, N. mening; 2mo to 6yrs–PCN prophylaxis
SCD–pt w/ fatigue and megaloblastic anemia (MC)
folate deficiency 2/2 inc retics
SCD–tx
Hydroxyurea incr prod HbF, tx infx aggressively, manage pain, BMT cures
SCD–BMT cures but has a ___ post-op mortality rate
10%
When is anemia benign?
for first 2-3months of life; physiologic drop in H&H
Anemia–transient erythroblastopenia 3mo-6yrs means that there is ___ after viral infxn (not B19)
immune suppression
Anemia–18 mo, picky eater, drinks lots of cow’s milk.
↓H&H, MCV 75, ↓ferritin, ↑TIBC
Iron deficiency [tx PO iron]
Anemia–18 mo, eats a varied diet. Mom is Italian.
↓H&H, MCV 60, ↓RDW
Thalessemia
Thalessemia tx
transfusion & deferoxamine
Anemia–8 mo is irritable, has glossitis & FTT. Picky eater, drinks lots of goat’s milk
folate deficiency (see low serum RBC folate), tx w/ daily folate PO
Dx–4mo pale baby, normal plts, WBCs but hemaglobin is 4. Incr RBC ADA and low retics. Triphalangeal thumbs
Blackfan-Diamond anemia
Blackfan-Diamond anemia tx
corticosteroids, transfusions, stem cell transplant
Dx–18mo baby presents w/ low plts, low WBCs and profound anemia. He has café-au-lait spots, microcephaly, and absent thumbs
Fanconi anemia
Fanconi anemia–dx
Bone marrow shows hypoplasia, Cytogenetic studies for chr breaks
Fanconi anemia–tx
Corticosteroids, androgens, bone marrow transplant
Fanconi anemia complications
Incr risk for AML and other cancers
Dx–2 y/o baby presents w/ hyperactivity, impaired growth, abdominal pain and constipation
Lead poisoning (basophilic stippling)
Lead poisoning–dx
venous blood sample, ck lead level
Lead poisoning–tx
Pb>45 = tx w/ succimer; Pb>70 = admit and tx w/ EDTA + dimercaprol
Lead poisoning–screening
Test blood lead levels btwn 12-24 mo if low SES, live in old house (
Thrombocytopenia–15 y/o F recurrent epistaxis, heavy menses & petechiae. ↓plts only
ITP
Idiopathic thrombocytopenic purpura–tx
IVIG for 1-2 days, then prednisone, then splenectomy. NO plts!!
Thrombocytopenia–15 y/o F recurrent epistaxis, heavy menses, petechiae, normal plts, ↑ bleeding time and PTT
vonWilebrand Dz
VWD–tx
DDAVP for bleeding or pre-op. Replace factor VIII (contains vWF) if bleeding continues
Thrombocytopenia–7 y/o M recurrent bruising, hematuria & hemarthroses, ↑ PTT that corrected w/ mixing studies
Hemophilia; if mild, replace w/ DDAVP, otherwise replace w/ factors
Thrombocytopenia–1wk old newborn, born at home, comes in with bleeding from the umbilical stump & bleeding diathesis
vit K def (↓ II, VII, IX and X)
Vit K def–tx
FFP acutely + vit K shot
Vit K def–what other pathology can cause this besides low vit K supplement?
CF kid w/ malabsorbtion
Thrombocytopenia–9 y/o F with Wilson’s disease developed fulminant liver disease, what is first factor depleted?
VII (PT increases first, then PTT)
Thrombocytopenia–9 y/o F with Wilson’s disease developed fulminant liver disease, what factors are NOT depleted?
VIII and vWF b/c they are made by ENDOTHELIAL CELLS
Dx–3 y/o w/ petechiae, abd pain, vomiting and lethargy. Had bloody diarrhea 5 d ago after eating hamburgers. +Thrombocytopenia and ↑creatinine
Hemolytic uremic syndrome
MCC HUS
E. Coli O157H7 [Shigella, Salmonella, Campylobacter]
Tx HUS
Tx w/ aggressive nutrition (TPN) and early peritoneal dialysis. NO abx for bloody diarrhea (↑ risk of HUS), NO platelets!
Dx–5 y/o w/ purpura on legs and buttocks, abd pain, joint pain, current jelly stool. Blood smear nl, coag studies nl and electrolytes nl. IgA and C3 are deposited in the skin.
Henoch Schonlein Purpura
Henoch Schonlein Purpura–MCC; tx
following URI; symptomatic tx, can use steroids for GI/renal dz
Dx–New onset seizure, ataxia and HA worse in the AM with vomiting for a month
brain tumor, most likely infratentorial
MC brain tumor in kids; tx; px
Pilocytic astrocytoma of cerebellum. Resect. ~90% survive.
Dx–Adolescent with height in 5th %, w/ bitemporal hemianopsia. See calcifications in sella turcica
Craniopharyngioma. Suprasellar; remnant of Rathke’s pouch
Dx–2 year old hypertensive child with asymptomatic abdominal mass
Wilm’s tumor
Wilm’s tumor–associations (4)
Aniridia, GU anoms, Hemihypertrophy, Beckwith-Weidemann
Wilm’s tumor–best test; tx
abd CT + CXR to see lung involvement; sx, chemo, rads
Dx–4 year old with jerking movements of eyes and legs, bluish skin nodules and an tender abdominal mass
neuroblastoma