Peds HemeOnc

banannie's version from 2016-03-08 22:48

Fetal Erythropoisis

Question Answer
Where does RBC synthesis take place in the fetus?Yolk Sac
Bone Marrow
[Young Liver Synthesizes Blood)
Week 3-8Yolk Sac
8-28 weeksLiver and Spleen
28 weeks+ Bone Marrow
Which bones are actively erythropoising in childhood?Sternum, Pelvis, Ribs, Vertebrae, Cranial bones, Long bones
Fetal hemoglobinHbF (2 alpha, 2 gamma):
↓ affinity for 2,3 DPG
↑ affinity for O2
Facilitates exchange
Adultssternum, vertebrae, ribs, pelvis

Childhood Anemias

Question Answer
picky eater, drinks cow's milkFe deficiency
rx fe deficiencyoral ferrous salts
italian momthalessemia
rx thalessemiatransfusion+deferoxamine
irritable, glossitis, drinks goat's milkfolate deficiency
rx folate deficiencydaily folate
triphalangeal thumbsblackfan diamond
web neck, shield chest, and anemiablackfan anemia
increased RBC ADAblackfan diamond
low hgb, low retics, nl plts and wbcblackfan diamond
rx blackfan diamond anemiacorticosteroids
stem cell transplant
low platelets, low wbc, profound anemia +cafe au lait spotsfanconi anemia
no thumbs, no radius, microcephalyfanconi anemia
diagnose fanconi anemiabone marrow shows hypoplasia
cytogenetic studies for chromosomal breaks
rx fanconi anemiacorticosteroids
bone marrow transplant
complications fanconi anemiaAML and other cancers
hyperactivity, low growth, constipationlead poisoning
diagnose lead poisonvenous blood sample
rx lead poisoning>45 succimer
>70 admit and EDTA+dimercaprol
anemia and hyperbilirubinemiahereditary spherocytosis
confirm hereditary spherocytosisosmotic fragility test


Question Answer
sudden onset petechiae and purpuraITP
only low plateletsITP
rx ITPIVIG 1-2 days
then splenectomy
normal platelets, increased PTTvon willebrands
rx hemophilaDDAVP, replace factors
bleeding umbilical stump and diathesisvitamin K deficiency (II, VII, IX, X) or CF
rx vitamin k deficiencyFFP + vitamin K shot
wilson's disease+fulminant liver disease, first factor depletedVII
which factors are normal in fulminant livervWB and VIII
petechiae and bloody diarrhea after eating hamburgersHUS
mc cause HUS0157:H7 E. coli (shigella, salmonella, campylobacter)
rx HUSearly peritoneal dialysis!
don't give platelets!
purpura on butt and currant jelly stoolHSP w/ intussusception

Microcytic Anemia

Question Answer
↓Fe ↓Ferritin ↑TIBCIron Deficiency Anemia
↓Fe ↑Ferritin ↓TIBCAnemia of Chronic disease
Sideroblastic Anemia
What is iron bound to in the stored form?Ferritin
Iron deficiency in babiesBreast milk
Esophageal web+Bright red tonguePlummer Vinson: Iron Deficiency Anemia
Koilonychia?Spoon shaped nails: Iron deficiency anemia
Pica?Chew on weird things: Iron deficiency anemia
↑Free Erythrocyte Protoporphyrin (FEP)Anemia of Chronic disease.
Decreased available iron?Anemia of chronic disease
Iron laden mitochondria form a ring around the nucleus of RBC precursors?Ringed sideroblasts: Sideroblastic Anemia
What defect causes sideroblastic anemia?ALAS (converts succinyl-CoA to ALA)
Acquired causes of sideroblastic anemia?)
Lead poisoning (inhibits ALAD and ferrochelatase)
Vitamin B6 deficiency (cofactor for ALAS)
Blue gums and colicky painLead poisoning: Sideroblastic Anemia
Where is the iron in sideroblastic anemia?Trapped in the mitochondria around the nucleus
Isoniazid causes what type of anemia?Sideroblastic Anemia: decreases vitamin B6
Defect in Globin?Thalassemia
Who gets alpha thalassemia?Asians (cis trait) and Africans (trans trait)
HbH Beta chain tetramers (HbH) that damage RBCs: Alpha thalassemia with 3 deletions
Hb BartsGamma tetramers damage RBCs: Alpha thalassemia with 4 deletions
4 alpha gene deletions cause?Hydrops fetalis
Alpha thalassemia mutationChromosome 16: deletion
Beta thalassemia mutationChromosome 11: Splice site mutation
Target cells?Beta Thalassemia
Tear drop cells?Beta Thalassemia
Who gets Beta Thalassemia?Blacks, Greeks and Italian
Crewcut appearance of skullexpansion of hematopoesis into the skull: Beta Thalassemia
Chipmunk facies?Expansion of hematopoesis: Beta Thalassemia

Macrocytic Anemia

Question Answer
Hypersegmented neutrophilsMegaloblastic anemia
↑HomocysteineMegaloblastic anemia
MC Megaloblastic anemia?Folate deficiency
Folate malabsorption happens inJejunum: Celiacs
MethotrexateFolate deficiency
Birth controlFolate deficiency
PhenytoinFolate deficiency
High methylmalonic acidB12 Deficiency
Neurologic symptomsB12 Deficiency
Pancreatic insufficencyB12 Deficiency
Pernicious anemiaAutoimmune destruction of parietal cells: B12 Deficiency
Diphyllobothrium LatumB12 Deficiency
VegansB12 Deficiency

Sickle cell

Question Answer
Swollen hands and feetdactylitis
Low hct, low reticsaplastic crisis (parvo b19)
rx aplastic crisisblood transfusion
point tenderness on femurosteomyelitis (salmonella!)
hip painosteonecrosis of femoral head (aka avascular necrosis)
RUQ after mealpigment gallstones (from chronic hemolysis)
respiratory distress + tonsillectomyWaldyer ring hyperplasia (hyperplasia of lymph tissue obstructing airway)
proteinuria, increased Crkidney infarct
sepsis bugs. pneumo
fever, cough, sobacute chest syndrome (pulmonary infarct)
mc cause of deathacute chest syndrome
confusion + focal neurostroke from sludging
rx for sickle cell strokeexchange transfusion
fatigue + megaloblastic anemiafolate deficiency anemia (use folate for RBC turnover)
vaccinesS. pneumo, HiB, Neisseria (SHiNSKiS)
PPXpenicillin until age 5
maintenance treatment for SSvaccination, penicillin (until age 5), folic acid supplementation, hydroxyurea
acute pain crises treatmenthydration
+/- transfusion


Question Answer
ALL symptomsbone and joint pain
viruses that causes hodgkinEBV virus
tumor lysis syndromehyperkalemia, hyperphosphatemia, hyperuricemia.
corticosteroids are contraindicated because they can precipitate tumor lysis syndrome
diagnostic hallmark of hodgkinsRS cell
viruses that cause non hodgkinEBV
mc brain tumor in kidsastrocytoma
prognosis of astrocytomagood, 90%
2mc brain tumor in kidsmedulloblastoma
where is medulloblastoma?cerebellar vermis, obstructing 4th ventricle
bitemporal hemianopsiacraniopharyngioma
calcifications in sella turcicacraniopharyngioma
- remnant of rathke's pouch
eyelid retraction and impaired upward gazepinealoma (parinaud syndrome)
hypertensive child with asx abdominal massWilm's tumor!
what is wilm's tumor associated withanirida, GU anomaly, Beckwith wiedemann, hemihypertrophy
best test wilm'sabdominal CT
treatment wilm'sresect, chemo, radiation
tender abdominal massneuroblastoma
jerking movements eyes/legs, blue skin nodulesneuroblastoma
dx neuroblastomaincrease urine homovanillic or vanillylmandelic acid
dx ALLbone marrow biopsy shows >30% lymphblasts
rx ALLVDP (Vincristine+Daunorubicin+Prednisone)
intrathecal methotrexate (for CNS)
ALL: who has poor prognosis?less than 1 and older than 10
rubbery nodes and drenching feverhodgkin lymphoma
nonproductive cough and large anterior mediastinal mass on cxrnon-hodgkin lymphoma
dx non-hodgkin lymphomabiopsy mass
bone marrow biopsy


Question Answer
Wilms tumor associated withaniridia and hemihypertrophy and does not cross the midline
neuroblastomaassociated with clonus and may cross the midline
onion skinningEwing sarcoma
sunburst lytic bone lesionsosteosarcoma

Pathologic RBC forms

PictureCell typeAssociation
Dacrocyte (Teardrop cell)Bone marrow infiltration (myelofibrosis)
Target cellThalassemia
Liver disease
SpherocyteHereditary spherocytosis
Hemolytic anemia (drug and infections)
Sickle cellSickle cell anemia
Reed-sternbergHodgkins lymphoma
Ringed sideroblastSideroblastic anemia
Macro -OvalocyteMegaloblastic anemia: B12 or Folate deficiency (also hypersegmented PMNs)
Bone Marrow failure
Howell Jolly
= basophilic remnants found in RBCs
Hyposplenia or asplenia
Helmet cell (schistocyte)DIC
HELLP syndrome
Mechanical hemolysis (prosthetic heart valve)
Heinz bodies
= Oxidized hemoglobin
G6PD deficiency
Alpha thalassemia
ElliptocyteHereditary elliptocytes
(Bite cell)
G6PD deficiency
Basophilic stippling
= Denatured RNA
Lead poisoning
Anemia of chronic disease
Iron Deficiency
Acanthocyte (Spur cell)
= Irregular spikes
Liver disease
Echynocyte (Burr cell)
= Regular spikes
Rouleaux formationMultiple myeloma