Heme & Onc Flashcards
Hematopoiesis sites
Yolk sac 1* until 6 (10 weeks)
Liver 1* 6-22 weeks
BM 22+ (starts 8-19 weeks)
Thalassemia
Quantitative defect of globin chain synthesis
Alpha thalassemia
One gene absent - silent carrier
2 absent - alpha thal trait
3 absent - Hb H (4 beta)
None - hydrops
Beta thalassemia
Trait
Disease - zero have increased Hb F and A2 but severe anemia ; plus have some Hb A and moderate anemia
Sickle cell disease
Qualitative abnormality of beta globin chain gene
Valine for glutamic acid at position 6
Fetal maternal hemorrhage calculation for Rhogam dose (kleihauer betke)
% Hb F = #fetal cells/maternal cells x100
1% = 50mL
The number of vials of rhogam is 1mL fetal blood/15
1 vial= 360mcg
Apt test
Done on fetal sample and detects maternal blood
Diamond Blackfan
Congenital hypoplastic anemia
Or congenital erythroid aplasia
Macrocytic anemia with reticulocytopenia
LBW, short stature, abn facies, triphalangeal thumbs, abn cardiac and renal systems
Congenital anomalies in ~40% of patients
Need chronic transfusions, steroids and BMT
Constitutional aplastic anemia
Fanconi anemia
Ch instability with breaks common
Triphalangeal thumbs, radial hypoplasia, short stature, hyperpigmentation, renal anomalies
Test ch with mitomycin c - increased ch breaks
Treat androgens and hematopoietic growth factors
Acquired erythrocyte underproduction
Erythroid aplasia (parvo) Aplastic anemia Infectious Treatment with growth factors BMT Iron, folic acid, vit b12 deficiency Lead toxicity
Indirect vs direct Coombs
Direct performed on infants blood - ab noted on pt RBC
Indirect performed on mothers blood - detects ab in serum
Polycythemia blood volume exchange
Blood volume = (observed - desired Hct)* infant blood volume / observed Hct
Sick
Bleeding with
Dec PLT inc PT inc PTT
DIC
Sick
Bleeding with
Dec PLT nl PT nl PTT
Platelet consumption
Sick
Bleeding with
Nl PLT inc PT inc PTT
Liver disease
Sick
Bleeding with
Nl PLT nl PT nl PTT
Compromised vascular integrity
Healthy
Bleeding with
Dec PLT nl PT nl PTT
ITP, occult infection, thrombosis, BM hypoplasia or infiltration
Healthy
Bleeding with
Nl PLT nl PT nl PTT
Vit K deficiency
Healthy
Bleeding with
Nl PLT nl PT inc PTT
Hereditary clotting factor deficiencies
Healthy
Bleeding with
Nl PLT nl PT nl PTT
Local bleeding, plt abn, factor 13 deficiency, VWD
Langerhan cell histiocytosis
Fever
Pancytopenia
Multiorgan infiltration
Hemophagic lymphohistiocytosis (hlh)
Familial - AR, fever, pancytopenia, HSM, adenopathy, fatal
Virus associated - fever, pancytopenia, HSM, CMV EBV HAV adenovirus
Solid tumors
Teratoma Neuroblastoma Pheochromocytoma Wilms Hepatoblastoma Retinoblastoma Rhabdomyosarcoma
Neuroblastoma
Second most common solid tumor in neonatal period
Prognosis depends on stage, age, n-myc amplification
Stage IV-S = <1 yo, localized primary tumor with mets limited to live skin or BM (favorable)
