Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

Ped RC > Heme > Flashcards

Heme Flashcards

1
Q

When is physiologic Nadir for Hgb?

A

Term: 8-10 wks. Pre-term 6-8 wks.

*Note f-Hgb has half the half-life, that’s why

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

Microcytic Anemia DDX

A 
*TAILS*: 
Thalassemia
Anemia of CD
IDA
Lead poisoning
Sideroblastic anemia.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

Macrocytic Anemia DDX

A
  • Megaloblastic: B12 def, Folate def
  • Marrow Failure: Myleodysplasia, Fanconi, Diamond-Blackfan, Aplastic
  • Other: Hypothyroid, DS, liver disease, drugs (AZT, etoh), Normal Newborn
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

Normocytic Anemia DDX

A
  • Anemia of CD
  • Chronic renal failure
  • Transient erythroblastopenia of childhood (TEC)
  • Malignancy/marrow infiltration
  • Other: HIV, HLH
  • Bleeding/hemolysis (except if massive reticulocytosis then macro)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

Classify hemolytic disorders

(based on problem in RBC)

A

Problem INTRINSIC to the Red Cell
• Membranopathy: hereditary spherocytosis, elliptocytosis
• Enzymopathy: G6PD deficiency, PK deficiency
• Hemoglobinopathy: Hb SS, SC, S-βthal

Problem EXTRINSIC to the Red Cell
• Immune hemolysis: autoimmune, iso-immune, drug-induced.
• Non-immune hemolysis: HUS, TTP, DIC, Burns, Wilson, Vit E def, etc.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

IDA: why does cow’s milk lead to IDA

A

Iron is absorbed at 50% efficiency from breast milk and 10% from cow’s milk. Excessive cow’s milk also
interferes with balanced nutrition, and causes GI blood loss.
Limit cow milk intake to 16-24 oz/day.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

IDA: causes

A

1) Inadequate iron endowment at birth
2) Insufficient iron in diet
3) Blood loss
GI tract (cow milk, parasitic infection, varices, Meckel’s, polyp, ulcer, H pylori)
Epsitaxis, menorrhagia, rarely pulmonary or renal loss
4) Malabsorption of iron
Celiac disease, antacids, giardiasis, IBD, IRIDA.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

IDA: treatment

A
  • Usual dose is 4-6 mg/kg/day of elemental iron. Vit C helps with absorption.
  • Check CBC + retics within 1-2 weeks of treatment

A positive response:
– Rapid subjective improvement within 2 days
– Reticulocytosis within 3-7 days
– Increased hemoglobin (usually 7 to 30 days)
– Repletion of iron stores (usually by 3 months).

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

Features on CBC indicative of IDA

A 
Microcytotic, hypochromic
MCV/RBC >13 (Mentzer index) is suggestive of iron def, (<13 suggests thal trait).
Increased RDW
Thrombocytosis
Pencil cells
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

Difference bw IDA and alpha-that trait?

A

1) Iron studies: alpha normal, IDA abnormal
2) RDW: alpha normal, IDA high.
3) RBC count: alpha increased (~5), MCV very low.
Mentzer index (MCV/RBC):
alpha <13, IDA >13
4) Plt: alpha normal, IDA increased

Note: Hb electrophoresis is normal in both.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Typical age group for “Transient Erythroblastopenia of Childhood” (TEC)

A

1-3 years

Often after a viral trigger (not necessarily parvo)
• Onset gradual, may become severe
• Recovery is spontaneous within 1-2 months
• A single transfusions may become necessary.
• The main ddx is diamond-blackfan anemia (vs aplastic crisis)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

Which anemia is associated with goat milk intake

A

Folate deficiency macrocytic/megaloblastic anemia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Types of hemolytic anemias caused by ‘membranopathy’

A

hereditary spherocytosis, elliptocytosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Types of hemolytic anemias caused by ‘enzymopathy’

A

G6PD deficiency, PK deficiency

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Types of hemolytic anemias caused by ‘Hemoglobinopathy’

A

Hb SS, SC, S-βthal

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

Hereditary Spherocytosis Treatment

A
  • Splenectomy: corrects the anemia + normalizes the RBC survival, even though the spherocytes persist.
  • should be deferred until after age 5y if possible to minimize risk of sepsis. Partial splenectomy may achieve a balance.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

Most common presentation of h. spherocytosis in a newborn?

A

Jaundice

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

Most common complication of hereditary spherocytosis?

A

Gallstones

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

What can cause a false negative in G6PD testing?

A

If done while hemolysing.

Must be done few weeks after.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

G6PD treatment

A
  • Supportive care + avoidance of acute triggers
    (Splenectomy is not useful.)
  • Most common oxidants: sulfas, nitrofurantoin, dapsone, naphthalene (moth balls), anti-malarials, rasburicase, fava beans, and infection.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

Types of AIHA (Auto-Immune Hemolytic Anemia)

3 types

A

1) Warm (IgG, extravascular hemolysis)
2) Cold (IgM, called “cold agglutinin disease”)
3) Biphasic (Donath-Landsteiner IgG, called “paroxysmal cold hemoglobinuria”).

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

AIHA: treatment

A
  • Warm AIHA:
    • supportive care, transfusions (“least incompatible”), steroids, ?IVIG, rituximab, splenectomy.
  • Cold or biphasic AIHA:
    - supportive care, transfusions prn. If severe: plasmapheresis.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

Etiology of SS anemia

A

Single nucleotide substitution (GTG for GAG, valine for glutamic acid) at codon 6 of the beta-globin gene on chromosome 11
• QUALITATIVE DEFECT (as opposed to quantitative )

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

SS anemia: List as many acute complications as you can

A
  • Acute vase-occlusive crises
  • Infections: strep pneumo, OM (staph, salmonella), Parvo aplastic crises
  • ACS (50%)
  • Stroke (10% by 20)
  • Splenic Sequestration
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Q

SS anemia: List as many chronic complications as you can

A
  • immunodeficiency sec to asplenia
  • resp: lung disease of SCD (asthma-like), pHTN
  • Neuro: sequelae from stroke
  • Retinopathy
  • Cholelithiasis (40%), Renal Insufficiency (20%), Priapism (10-40%)
  • Osteonecrosis of femoral head (50-60% by adult)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
26
Q

Rates of functional asplenia in SS

A

30% of SS by 1 y and 90% by 6 y.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
27
Q

SS ACS: management

A
  • IV Antibiotics (cephalosporin and macrolide)
  • O2
  • hydration
  • incentive spirometry
  • bronchodilators.
  • Simple transfusion for hypoxia needing O2 or a Hct<18%.
  • Exchange transfusion for progressive multilobe infarcts and hypoxia.
  • Recurrent ACS: consult resp for PFTs.
28
Q

SS ACS: features + causes

A

Def: fever and infiltrate. Hypoxia not necessary for the dx.

Causes:

  • 16% are due to bacterial infections (mostly atypicals) and 6% due to viral.
  • 10% are due to fat embolism (after bone marrow infarction from a VOC.)
  • Too much analgesia for VOC can cause hypoventilation leading to ACS.
29
Q

SS: splenic sequestration dx

A
  • sudden enlargement of the spleen + 20 pt drop in Hb with reticulocytosis.
  • Can result sudden circulatory collapse and death.

**
Occurs in children with SS <3 y, with other types at any age. Recurrence is frequent.

30
Q

SS anemia: splenic sequestration managt

A
  • NS bolus, blood transfusions.

- 2+ splenic sequestrations –> splenectomy.

31
Q

SS anemia: list 5 preventative care measures

A

1) Penicillin prophylaxis should begin at 3 mo- 5 years.

2) Pneumococcal vaccination
– Prevnar-13 at 2, 4 and 12 mo
– Pneumovax-23 should be given at 2 y and 5 y.

3) TC doppler should begin at 2 y- 16 y for all SS and S-β0 thals.
4) Routine screening for end-organ damage should begin in middle-childhood.
5) Evaluated by SC program by 3 mo. Parent education re fever mgt and spleen palpation

32
Q

SS anemia: 5 indications for transfusion

A

Simple transfusion:

1) Aplastic crisis
2) Splenic sequestration
3) Pre-op (high risk surgeries)
4) Stroke – if low Hb, awaiting exchange
5) ACS – if low Hb, awaiting exchange
6) Also used chronically to prevent stroke by keeping HbS under 30%.
7) Persistent priapism, with caution

Exchange transfusion:
- Reserved for stroke, severe ACS, and pre-op some major surgeries.

  • transfusions NOT indicated for vaso-occlusive crisis.
33
Q

What increases risk of stroke in SS anemia

A

Hx of ACS (recent, frequency)

Other RFs include SS genotype, low baseline Hb, previous TIA, high blood pressure,

34
Q

SS anemia: 2 disease modifying tx

A

1) hydroxyurea

2)

35
Q

Questions to ask for BLEEDING HX

(+age group based)

A

Neonate: Cord separation, IVH, subgaleal hemorrhage, procedural
• Infant: Vaccines, circumcision
• Toddler: Intracranial bleed after small trauma, joint or soft tissue bleeds
• Child: Epistaxis, joint or soft tissue bleeds, exfoliation of primary dentition
• Adolescent/Adult: Menorrhagia, peripartum bleed
• All: Bleed requiring transfusion, excessive bleed during surgery
• Family Hx: Consanguinity
• Other History: Recent drug use (including natural + OTC), recent viral illness or diarrhea (for thrombocytopenia)

36
Q

Thrombocytopenia: DDX based on physiology

A

1) Decreased production:
- marrow infiltration (leukemia)
- marrow injury (aplastic anemia)
- congenital syndromes of ineffective thrombopoiesis (eg TAR, hereditary thrombocytopenias, Wiskott-Aldrich syndrome)

2a) Increased destruction, Immune:
- ITP, SLE, NAIT, Infection, Heparin, drugs (eg VPA, anticonvulsants, chemo).
2b) Increased destruction, Non-Immune:
- DIC, HUS, TTP, Infection, Kasabach-Merritt syndrome, artificial heart valve.

3) Sequestration:
- Hypersplenism from liver disease, storage disease, portal vein thrombosis.

4) Consumption:
- thrombosis, eg clot around central line, type 2B vonWillebrand disease.

5) Platelet loss or dilution
- eg. after massive dilution for major trauma

37
Q

Neonatal Thrombocytopenia: DDx based on physiology

A

1) Decreased production: congenital syndromes.
- TAR (thrombocytopenia absent radii syndrome) in which megakaryocytes are absent in the bone marrow.

2) Non-immune destruction:
- TORCH eg CMV
- NEC, RDS, and transiently after maternal pre eclampsia. (In general, a sick neonate).

3) Immune destruction:
- Maternal ITP – check mom’s CBC
- NAIT - alloantibody directed against an antigen on the newborn’s platelets made by mother

38
Q

Most common cause of isolated thrombocytopenia in children

A

ITP: immune thrombocytopenic purpura

39
Q

ITP: 4 indications for BMA

A

1) Low WBC
2) Low Hgb
3) Blasts on the peripheral smear
4) Lymphadenopathy
5) Hepatosplenomegaly

(or suspicious history such as long fevers, bone pain or weight loss.)

40
Q

ITP: counselling

A

1) Reassure parents about the chances of recovery and rarity of serious events.
2) Avoid contact sports and restrict activities if possible. 3) Avoid NSAIDs and aspirin.

41
Q

ITP: Management

A

1) Counselling
2) Increasing plt count (3-4 ways)
- (transfusion, steroids, IVIG, Anti-D)
3) Splenectomy
4) Immunomodulation:
- CSA, MMF, rituximab, vincristine,
cyclophosphamide, others
5) Increase Production – Thrombopoietin analogues

CPS Statement:
- Treat based on severity (mild, mod, severe) and NOT based on plt count.

42
Q

ITP: ways to increase platelets

A

1) Platelet transfusions:
– not effective (destroyed immediately); should only be used in cases of actual intracranial hemorrhage
2) Oral steroids x 4 days.
3) IVIG may work slightly faster then steroids.
4) Anti-D
— works by coating red cells in people who are Rh+ (so it can only be used in 80% of people) and causing an immune hemolysis. It keeps the spleen busy
chewing red cells so that the platelets can slip by.

43
Q

ITP: 2 indications for splenectomy

A

1) older child (> 4 yr) with severe ITP >1 yr + symptoms not controlled with medical tx
2) life-threatening hemorrhage (intracranial hemorrhage) complicates acute ITP, plt count not corrected other means

44
Q

Heparin acts on what part of the fibrinolysis/coag pathway?

A

Activates on Antithrombin (which anti-coagulates)

45
Q

Warfarin: mechanism of action

A

Activates Vit-K dependant clotting factors

46
Q

How to clinically differentiate primary hemostasis problem from secondary hemostasis?

A

Primary: mucocutaneous bleeds (oral, nasal), petechiae
Secondary: deep bleeds (muscular bleeds, joints, large hematomas)

47
Q

Vit K: What does CPS recommend (dose and timing) for newborn

A

All be given 0.5 to 1.0 mg of IM vit K within 6 hrs of birth. Those who refuse: give IM dose as 2 mg PO, and repeat at 2-4 wk, 6-8 weeks.

48
Q

Types (and timing) of Vit-K def bleeding (newborn)

A

• Early HDNB:

- first 24 hours. 
- Usually due to maternal anticonvulsants or antiTB meds. Give vit K to mother.

• Classic HDNB:

- from 1 day to 1 week. 
- Observed in up to 2% of infants not received vit K proph at birth.

• Late-onset HDNB:

- 2 weeks to 2 months or longer. 
- breast-fed infants not received vit K prophylaxis.
- Present with IC bleeding.
49
Q

vWD : types

A
  • Type 1 : quantitative. (most common, mild, women)
  • Type 2: qualitative (many subtypes)
  • Type 3 (AR, consanguinity)
50
Q

vWD: testing

A

1) CBC, coags, PFA.
2) - vWillebrand antigen (to test quantity),
- ristocetin cofactor (to test quality or function of vWF),
- factor VIII level
- blood group (because levels vary with BG).

51
Q

vWD: management (tx, counselling)

A
  1. Desmopression acetate (DDAVP): IV or IN
    - need DDAVP challenge
  2. Factor VIII-vWF concentrate (Humate P – human plasma derived)
  3. OCP and treatment for anemia (eg iron) if needed for menorrhagia.
  4. General supportive care: avoid contact sports, wear helmets with any wheels or contact, avoid anti-platelet meds such as Advil/ASA
  5. Antifibrinolytics (eg TXA) useful for mucocutaneous bleeds.
52
Q

Hemophilia: types (deficiencies)

A
  • Hemophilia A = Factor VIII deficiency = Classic hemophilia, 85% of cases.
  • Hemophilia B = Factor IX deficiency = Christmas disease, 15% of cases.
53
Q

Hemophilia: severity classification

A 
MILD disease (5-30% factor activity level): 
       - may have bleeding after surgery or trauma.
MOD disease (1-5%): 
       - occasional hemarthroses &amp; spontaneous hematomas 
SEVERE disease (less than 1%): 
       - spontaneous bleeding into joints and deep tissues.
54
Q

Hemophilia: management

A

(• Prophylactic factor after delivery can be considered; not routine)

  • Give vitamin K! – lots of pressure on IM site
  • Education: parents are taught to give IV factors. Ports may be necessary.
  • Acute treatment: for severe: factor should be given immediately, even before evaluation.

• Factor replacement: patients who develop target joints should be placed on a regimen of prophylactic factor replacement to avoid progression.
– For minor, achieve factor levels of 50%.
– For major hemorrhage, achieve factor level of 100%.
– 1 U/kg of FVIII = 2% rise in levels.
– 1 U/kg of FIX = 1% rise in levels.

• DDAVP can be used for mild/moderate hemophilia A → challenge first

55
Q

List 5 neonatal presentations of hemophilia

A
  • Intraventricular hemorrhage
  • Circumcision bleeding
  • IM hematoma after vit K injection
  • Bleeding at the umbilical stumb
  • Large caput or subgaleal bleed
  • Excessive bleeding with phlebotomy
56
Q

Indications for transfusions

  • pRBC
  • plt
  • FFP
  • Cryo
A

Transfuse Red Blood Cells:

  • For Hb 70-100: signs of impaired oxygen delivery. Few situations.
  • For Hb < 70, likely to be appropriate.

Transfuse Platelets:

  • If plt < 10. (except ITP)
  • If plt < 20 and fever or coagulopathy
  • If plt < 50 for surgery, epidurals, LPs
  • If plt < 100 for neurosurgery or head trauma

Transfuse FFP:

  • For emergency reversal of warfarin
  • For active bleeding or surgery, and coags > 1.5x normal

Transfuse Cryoprecipitate (factor 8, fibrinogen, vWF):

  • For bleeding in pt with fibrinogen less than 0.8 to 1.0.
  • For bleeding with vWD or hemophilia ONLY if factor or DDAVP is unavailable.
57
Q

Most common reaction to transfusion

A

urticaria

fever

58
Q

alpha Thal subtypes

A

Based on # of defective a-genes
1 –> carrier
2 –> trait (cis vs trans), (minor disease)
3 –> HgH disease (moderate disease)
excess beta –> clumping/tetramers –> hypoxia:
- hemolysis in marrow + extravascular
- High O2 affinity –> not released. –> severe anemia
- Triggers increase RBC production –> HSM
4 –> hydrops fetalis (Barts)
- in fetal: gamma chain tetramers –> extreme O2 affinity (don’t release)
- hypoxia –> Cardiac Failure + HSM + edema
- incomp w life –> transf, BM Tx

59
Q

alpha-thalassemia: CBC features

A
  • low Hgb
  • Low MCV
  • Low MCH

(Mentzer index: MCV/RBC <13 )

Smear:
- microcytic, hypochromic, target cells

60
Q

beta-thalassemia subtypes

A

Minor: 1 defective beta (b0, or b+)

Intermedia: 2 defective (function): b+b+

Major: 2 defective (loss) b0b0

61
Q

how can you differentiate alpha and beta TRAIT based on testing

A

Hgb electrophoresis is normal in alpha and abnormal in beta.

62
Q

Diamond Blackfan Anemia: presentation

A

▪ Pallor with profound anemia usually becomes evident by 2 to 6 months of age; 90% of cases are recognized in the first year of life
▪ Growth retardation occurs in about one-third of children
▪ Congenital malformations are detected in about 35% to 45% of children
▪ Most commonly, craniofacial abnormalities (hypertelorism) and thumb abnormalities (flattening of thenar eminence, triphalangeal thumb)

63
Q

Diamond Blackfan anemia: Labs

A

● Anemia (macrocytic, low retic, insufficient RBC precursors)
● Otherwise normal bone marrow
● Erythrocyte ADA activity is ↑ (helps to distinguish from TEC)
● Thrombocytopenia and neutropenia can present initially
● RBC precursors are markedly ↓ in BM, Serum iron levels are ↑
● unlike in Fanconi A, there is no ↑ in chromosomal breaks when lymphocytes are stressed with
alkylating agents

64
Q

Diamond Backfan Anemia: DDX

A

● Transient erythroblastopenia of childhood (TEC)
- Macrocytosis, congenital anomalies, fetal red cell characteristics, and elevated erythrocyte ADA are generally associated with DBA and not with TEC
● Fanconi anemia - usually pancytopenia and absent radius
● Schwachman Diamond
● Myelodysplastic syndrome
● ParvoB19 – need to rule this out when diagnosing DBA
● HIV, as well as drugs, immune processes, and Pearson syndrome should also be ruled out

65
Q

Diamond Blackfan Anemia: Tx

A

● Corticosteroids are mainstay of treatment
● If fail steroids → transfusions
● Stem cell transplant is curative

66
Q

Hemophilia: complications

A

1) Intracranial bleeding: neurologic sequelae
2) Intramuscular bleeding: can lead to compartment syndrome
3) Intra Articular bleeding: can lead to arthritis and reduced ROM

Ped RC (9 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions