Hematology, Bilirubin Flashcards
DDx neonatal thrombocytopenia
early 72 hrs
lateafter 72 hours
Bleeding disorders
- isolated high PT
- isolated high PTT
- both
blood vol for exchange transfusion
Hemolytic anemias
- pyruvate kinase deficiency
- glucose 6 phosphate deficiency
Other ddx hemolytic anemia
More ddx NB thrombocytopenia
Physiologic mechanisms of neonatal jaundice
FActors that influence efficacy of phototherapy
Contributions to anemia of prematurity
What has been associated with neuroblastoma
presentation of acute bilirubin encephalopathy
Describe hemorrhaghic disease of newborn
- time periods of presentation
- causes
- presentation
- mgmt
stages of kernicterus
Bilirubin metabolism
Review thalassemias
Review thalassemias
Changes that lead to RBC storage lesions
Continuing to rise BR - on ptx
HyperBR, phototherapy risk factors
Review thalassemias
- clinical presentation
chromosome - encodes alpha globin
chromosome - encodes beta globin
chromosome 16
chromosome 11
most common worldwide hemoglobinopathy
Hgb E - decrease PRODUCTIONof beta chains - chronic, mild hemolytic anemia; esp SE asia
21glu ->lys
can still form tetramer with alpha 2 chains
KB test calc
% Hgb F = # fetal cells / # maternal cells x 100 (assumes maternal cells 500 mL)
Ask to calculate fetal blood loss by KB test -> take % hgb fetus and x 5000
Rhogam vials = mL blood / 15
Congenital erythrocyte underproduction
- Diamond Blackfan
- Fanconi Anemia
Diamond Blackfan - AD, AR
- pure RED CELL aplasia
- may have increased Hgb F
- Increased RBC adenosine deaminase levels
- MACROcytoci anemia at 2 months, low retic,BM no erythroid precursors + TRIPHALANGEAL THUMBS, abnormal facial, maybe cardiac, renal
Increase risk MDS, AML; chronic transfusions, steroid, BM tx possible
Fanconi anemia
- chromosomal INSTABILITY - lots of breaks
- hypoplastic BONE MARROW - anemia then pancytopenia
- MACROCYTOSIS, TRIPHALANGEAL thumbs, RADIAL hypoplasia, renal, ear malformations, strabismus
Test chromosome with mitomycin for BREAKS; Rx ANDROGENS, GF, BM tx
RBC membrane defect
RBC biochemical defect
Her spherocytosis - AD, membrane instability, aplastic crisis, vaccinate ENCAPSULATED ORGANISMS, +osmotic fragility test
G6PD defn - #1enzyme deficiency, XLR -> cannot make NADPH so decrease levels, worse with oxidant STRESS; HEINZ bodies; FN if not in crisis
Pyruvate kinase deficiency - AR, not convert PEP to pyruvate so not generate ATP; chronic anemia;
most common solid tumor
second most common
Teratoma - 50% SCT, malignant potential <10% neonates
Neuroblastoma - adrenal, neural crest (horner HTN, flushing, dd, subcutaneous nodules), < 12 mo = better prognosis
Prognosis depends on stage, age, N-myc amplification; Stage IV S - < 1 yo better prognosis (although mets)
Ax NF, central hypoventilation, Hirschsprung
Other tumors
- pheochromocytoma
- Wilms
- hepatoblastoma
- retinoblastoma – inheritance
- rhabdomyosarcoma
P - von hippel lindau, catecholamine
W - GU, hemihypertrophy ASS - mets - good prognosis - BW, Denys Drash, WAGR, pearlman
H - increased AFP, thrombocytosis
R - sporadic > AD; 45% passing on if bilateral, 6% unilateral; 2nd sibling if 1st bilateral - 3%
FFP contains…
Cryo contains…
FFP - all factors + gamma globulins + albumin + protein factors
Cryo - VIII, vWF, fibrinogen, XIII, fibronectin
Congenital leukemia
Histiocytosis
Langerhans - F,pancytopenia, multi organ infiltration
HLH - F, pancytopenia, low fibrinogen, high ferritin/high TG, HSM, adenopathy
BR - increase production or increase circulating BR
Production usually 2/2 increased RBC breakdown
Increased enterohepatic circulation due to bowel obstruction, delayed enteral feeds, HD, mec ileus
OTHER - cong hypothyroidism, tyrosinemia, liver failure, sepsis
Drugs that uncouple BR from albumin
ceftriaxone (although confusion in Q - uncouples but also decreases unconjugated BR?)
Drugs where hyperbilirubinemia will influence its dose
