Hematology Flashcards
Iron deficiency Anemia: term infants
@ 12 weeks: 9-11 mg/dL (due to EPO suppression at birth)
-No treatment required
Iron deficiency Anemia: preterm infants
- exaggerated and earlier
@ 3-6 weeks: 7-9mg/dL; may require transfusion
Cows Milk -
start @ 12 months;
not before: iron deficiency anemia, hemorrhage in gut
Iron deficiency anemia:
- dietary cause
- treatment
- first thing to improve: reticulocyte count
-occurs @ 9-24 months (adequate stores for 4-6 months)
Tx: trial of ferrous sulfate; limit cow’s milk
-continue iron replacement for 8 weeks after blood value normalizes to replete bone marrow iron stores
Lead Poisoning: signs and symptoms
Hyperactivity, aggression, learning disability (mistaken for ADHD), impaired growth, constipation, abdominal pain (lead colic); CNS: memory loss, confusion, peripheral neuropathy
Lead poisoning: Risk factors
- immigrant -international adoptee
- home built before 1978 - PICA
- under renovation -sibling with lead poisoning
- with peeling paint -low socioeconomic status
Lead poisoning: testing
initial: venous blood lead level @ 12 & 24 mos if high risk
labs: microcytic, hypochromic, sideroblastic, basophilic stip.
XR of long bones: dense lead lines
Lead poisoning: levels/Tx
Succimer = Oral Meso 2,3, dimercaptosuccinic acid
EDTA = calcium sodium edetate
Mild: 5-44 mg/dL - repeat in 1 month, no medication
Mod: 45-69 mg/dL - Oral Meso 2,3 dmcts acid
Severe: 70 and up - IV dimercaprol + EDTA
Long term risk of lead poisoning?
long-term cognitive impairment
Lead poisoning:
- treat according to levels
> 45 = chelation therapy
< 70 oral succimer, 70: IV dimercaprol/edta
* refer to department of health: blood level > 15 mcg/dL
Hb disorders:
HB electrophoresis
-newborn screening & best diagnostic test
HbSC: 50% HbS; 50% HbC TRAIT: 40% HbS; 60% HbA SSD: 86% HbS; 0% HbA; 12% HbF Bthal: HbA2, HbF increased Athal: Normal
Hb disorders:
prenatal diagnosis for parents with trait
Chorionic villous sampling @ 10-12 weeks gestation
Amniocentesis @ 14-18 weeks
Hb disorders (sickle cell): MCC mortality in pediatric cases
- Splenic Sequestration Crisis
- Infection
- Acute chest syndrome
Decreased reticulocyte count in S.S:
Aplastic Crisis (parvovirus B19)
Hb disorders: treatment
- Transfuse: symptomatic (SOB, Chest pain)
- Exchange transfusion: life-threatening complications (stroke, acute CS, SSC), before high-risk surgery
- Curative: bone marrow transplant (10% mortality risk)
Hb disorders: Hydroxyurea - give to:
- > /= 3 crises per year
- symptomatic anemia (COB, chest pain)
- life-threatening complications
- Increases HbF
- Reduces painful crises
- Reduces number of transfusions
- Does not reduce risk of stroke
Hb disorders: Routine care
- Daily folate + Penicillin (prophylaxis) 2 months - 5 yo
- Immunizations: Regular
(+) pneumococcal @ 2 mos
(+) influenza @ 6 mos & yearly
(+) meningococcal @ 2 yrs
Beta Thalessemia Major (Cooley Anemia)
- presents in 2nd month of life (not as newborn)
- progressive anemia, hypersplenism, cardiac decomp.
- expanded medullary space (skull, face)
- extramedullary hematopoiesis
- hepatosplenomegaly
B Thal: testing
Best initial: Hb electrophoresis -
HbF, HbA2 increased; HbA decreased
B Thal: Treatment
cap on pencil/lead; defer the iron
Transfusion: maintain Hb > 9 g/dL
Iron chelation: Deferoxamine plus Vitamin C (by 8yo)
Splenectomy: hypersplenism
Bone marrow transplant: curative
B Thal: Routine care
- Daily folate; Penicillin prophylaxis
- Vaccinations: pneumococcal, hep B
- Growth hormone: excess iron = decreased GH
Neonatal Polycythemia:
Hb > 65% or Hb > 22
- Screening: heel prick; If increased venous sample
h/p: drowsy, poor feeding, irritable, jaundice
TX: IV hydration + Partial exchange transfusion
Hemophilia A: (factor VIII) TX
Minor bleeding: desmopressin + aminocaproic acid
Major bleeding: factor VIII
Hemophilia B: (factor IX)
Minor/Major bleeding: factor IX
Von Willebrand’s Disease
-Avoid cryoprecipitate
Minor bleeding: desmopressin
Major bleeding: plasma-derived vWF containing FVIII
Immune Thrombocytopenic Purpura (ITP)
- most pt recover platelet count w/i 6 months
- transfusion contraindicated
- do not treat platelet count, treat clinical bleeding
1st choice: prednisone
2nd choice: IVIG
10-20% develop chronic ITP: rituximab or splenectomy
