Hematology

Question 1

Iron deficiency Anemia: term infants

Answer 1

@ 12 weeks: 9-11 mg/dL (due to EPO suppression at birth)

-No treatment required

Question 2

Iron deficiency Anemia: preterm infants

- exaggerated and earlier

Answer 2

@ 3-6 weeks: 7-9mg/dL; may require transfusion

Question 3

Cows Milk -

Answer 3

start @ 12 months;

not before: iron deficiency anemia, hemorrhage in gut

Question 4

Iron deficiency anemia:

• dietary cause
• treatment
• first thing to improve: reticulocyte count

Answer 4

-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

Question 5

Lead Poisoning: signs and symptoms

Answer 5

Hyperactivity, aggression, learning disability (mistaken for ADHD), impaired growth, constipation, abdominal pain (lead colic); CNS: memory loss, confusion, peripheral neuropathy

Question 6

Lead poisoning: Risk factors

Answer 6

• immigrant -international adoptee
• home built before 1978 - PICA
• under renovation -sibling with lead poisoning
• with peeling paint -low socioeconomic status

Question 7

Lead poisoning: testing

Answer 7

initial: venous blood lead level @ 12 & 24 mos if high risk
labs: microcytic, hypochromic, sideroblastic, basophilic stip.
XR of long bones: dense lead lines

Question 8

Lead poisoning: levels/Tx
Succimer = Oral Meso 2,3, dimercaptosuccinic acid
EDTA = calcium sodium edetate

Answer 8

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

Question 9

Long term risk of lead poisoning?

Answer 9

long-term cognitive impairment

Question 10

Lead poisoning:

- treat according to levels

Answer 10

> 45 = chelation therapy
< 70 oral succimer, 70: IV dimercaprol/edta
* refer to department of health: blood level > 15 mcg/dL

Question 11

Hb disorders:
HB electrophoresis
-newborn screening & best diagnostic test

Answer 11

HbSC: 50% HbS; 50% HbC
TRAIT: 40% HbS; 60% HbA
   SSD: 86% HbS; 0% HbA; 12% HbF
Bthal: HbA2, HbF increased
Athal: Normal

Question 12

Hb disorders:

prenatal diagnosis for parents with trait

Answer 12

Chorionic villous sampling @ 10-12 weeks gestation

Amniocentesis @ 14-18 weeks

Question 13

Hb disorders (sickle cell): 
MCC mortality in pediatric cases

Answer 13

1. Splenic Sequestration Crisis
2. Infection
3. Acute chest syndrome

Question 14

Decreased reticulocyte count in S.S:

Answer 14

Aplastic Crisis (parvovirus B19)

Question 15

Hb disorders: treatment

Answer 15

• 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)

Question 16

Hb disorders: Hydroxyurea - give to:

• > /= 3 crises per year
• symptomatic anemia (COB, chest pain)
• life-threatening complications

Answer 16

• Increases HbF
• Reduces painful crises
• Reduces number of transfusions
• Does not reduce risk of stroke

Question 17

Hb disorders: Routine care

Answer 17

• Daily folate + Penicillin (prophylaxis) 2 months - 5 yo
• Immunizations: Regular
  (+) pneumococcal @ 2 mos
  (+) influenza @ 6 mos & yearly
  (+) meningococcal @ 2 yrs

Question 18

Beta Thalessemia Major (Cooley Anemia)

- presents in 2nd month of life (not as newborn)

Answer 18

• progressive anemia, hypersplenism, cardiac decomp.
• expanded medullary space (skull, face)
• extramedullary hematopoiesis
• hepatosplenomegaly

Question 19

B Thal: testing

Answer 19

Best initial: Hb electrophoresis -

HbF, HbA2 increased; HbA decreased

Question 20

B Thal: Treatment

cap on pencil/lead; defer the iron

Answer 20

Transfusion: maintain Hb > 9 g/dL
Iron chelation: Deferoxamine plus Vitamin C (by 8yo)
Splenectomy: hypersplenism
Bone marrow transplant: curative

Question 21

B Thal: Routine care

Answer 21

• Daily folate; Penicillin prophylaxis
• Vaccinations: pneumococcal, hep B
• Growth hormone: excess iron = decreased GH

Question 22

Neonatal Polycythemia:

Hb > 65% or Hb > 22

Answer 22

• Screening: heel prick; If increased venous sample
  h/p: drowsy, poor feeding, irritable, jaundice
  TX: IV hydration + Partial exchange transfusion

Question 23

Hemophilia A: (factor VIII) TX

Answer 23

Minor bleeding: desmopressin + aminocaproic acid

Major bleeding: factor VIII

Question 24

Hemophilia B: (factor IX)

Answer 24

Minor/Major bleeding: factor IX

Question 25

Von Willebrand’s Disease

Answer 25

-Avoid cryoprecipitate
Minor bleeding: desmopressin
Major bleeding: plasma-derived vWF containing FVIII

Question 26

Immune Thrombocytopenic Purpura (ITP)

• most pt recover platelet count w/i 6 months
• transfusion contraindicated
• do not treat platelet count, treat clinical bleeding

Answer 26

1st choice: prednisone
2nd choice: IVIG
10-20% develop chronic ITP: rituximab or splenectomy