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2019 RC Specialties > Hematology > Flashcards

Flashcards in Hematology Deck (113)
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1
Q

When do consider partial exchange transfusion for polycythemic newborns?

A

Hct > 70%

Or lower w/ symp.

2
Q

If you are giving a partial exchange transfusion- what fluid as diluent?

A

Normal Saline

3
Q

How much blood do you give to decrease Hct from 0.75 to 0.5 in 2kg BB?

A

BB Blood vol= 80 mL x kg

Volume exchange (mL)
= Blood vol x (observed-desired Hct)/ Observed Hct
= (80x2) x (0.75-.05)/0.75
= 53 mL

Or generally 10-20 cc/kg.

4
Q

What are suggested Transfusion level for infants with anemia of prem?

A

*start at 115 w/ resp support and minus 15 if none= 100
For each wk start w/ last no resp support (100 for wk 2) threshold for resp support and minus 15.

> Wk 1: Resp support= 115; No resp= 100

> Wk 2= 100 w/ resp support; 85 if not

> Wk 3= 85 or 75 no resp

5
Q

List 3 AE to RBC transfusion:

A

> AE of leukocyte

  • anaphylaxis
  • graft vs. host
  • TRALI (transfusion related lung injury)

> Acute volume or lyte
- TACO (transfusion related circulatory overload)

> transfusion infection
- virus, bacterial, parasite

> blood group in compatible
- aute hemolytic rxn

6
Q

Which blood group do you give neo in ED?

A

Group O Rh (-)

7
Q

T or F: you try to maintain Hgb of 75 in prem.

A

True; regardless of age.

8
Q

T or F: infants with resp or cyanotic heart dx may need higher transfusion threshold.

A

True

9
Q

Most common RBC transfusion rxn?

A
  1. Febrile non hemolytic rxn
  2. Allergic rxn
  3. TACO (circulatory overload)
10
Q

Most common cause of febrile rxn in blood transfusion?

A

Sensitization to WBC

> due to presence of cytokine made by passenger leuk

NOT hemolysis.

11
Q

Child getting blood. Become febrile and chills. Management?

  • stop it; run TKVO
  • stop it; steroids
  • continue at slow rate
  • continue w/ methylpred
A

Stop it and run TKVO

+/- antipyretic if fever bothersome
+/- ensure no other cause (hemolytic rxn, sepsis)

12
Q

Which infection is most at risk to get from pRBC?

  • Hep B
  • Hep C
  • HIV
  • Parvo B19
A

Parvo B19

HIV: 1 in 8 million
Hep C: 1 in 5 million
Hep B: 1 in 1 million

Parvo: 1 in 20 K

13
Q

Child with that major thal and recurrent transfusion. On desfuroxime. Most serious complication?

A

Cardiac hemosiderosis

** transfusion induced
Fe deposited; liver, endo, cardiac after 10 yr

14
Q

How are thalassemias inherited?

A

AR

15
Q

What are Beta thal minor (trait), intermedia, major.

A

Beta Thal= beta lacking.

Minor/Trait= 1 abN allele
B/B+, B/B0
= Silent; No Tx

Beta Intermedia
B+/B+, B+/B0
= two abnormal allele
may need transfusion

Beta Major= Cooley
= homozygous
B0__/B0__
> CC: 6-12 mon. when HbA replace HbF= anemia, jaundice, DD, HSM
Tx: transfusion, iron chelation, bone marrow transplant

16
Q

What Hgb do you find in beta thal major patients?

A

Hb F

0-10% HbA only

17
Q

What can you expect on beta thal major BW?

A
  • anemia
    BUT low retic (unstable erythroid precursor die)
  • ++ HbF
18
Q

Which ethnicity is beta thal dominant in?

A

Mediterranean

Indian

19
Q

What ethnicity is alpha-thal dominant in?

A

S.E Asia

Africa

20
Q

Describe the 4 types of alpha Thal

A

aa/-a= 1 bad gene= silent

  • a/-a= 2 bad gene= trait
  • a/–= Hb H= 3 bad gene

–/– = Hb Barts/ hydrops fetalis

21
Q

What do you see with HbH:

  • inclusion body
  • heinz body
  • howell-jolly body
A

Inclusion body

(Hgb= beta 4 because three alpha missing= seen in cells)

Heinz Body= G6PD

Howell-Jolly= asplenia (sickle cell), amyloid, hemolytic anemia, prem infant

22
Q

How do you a dx a Thalassemia?

A

Hemoglobin electrophoresis

(i.e. HbA2 and HbF up= beta chain missing)

Confirmation: Gene mapping

23
Q

What type of treatment is required for Hb Barts?

A

Intrauterine transfusion

as no alpha gene
= fetal hydrops otherwise

24
Q

What is gold standard for beta thal major?

A

Transfusion
+ Hydroxyurea (increase Hb F)
+/- hematopoietic stem cell transplant curative

25
Q

What do you need to be aware of with beta thal major transfusions?

A

Keep Hgb > 90.
Transfuse q4wk. 4 life.

Watch for Fe overload (screening serum ferritin and MRI)
Watch liver, endo, cardiac deposits

Start Iron cheating as soon as overload seen (deferoxamine)

26
Q

T or F: hematopoietic stem cell is CURATIVE for beta thal major?

A

True

27
Q

Girl with microcytic anemia. Ferritin and iron normal. Next test? DDX?

A

Hgb electrophoresis

Microcytic Anemia

  • Iron deficiency
  • Thalassemia (AR dx)
  • Sideroblastic (granular deposit Fe in mitochondria; high transferrin, ferritin, Fe)
  • Lead intoxication (old paint)
  • Anemia of chronic dx
28
Q

What is the Mentzer Index?

A

MCV/ RBC count

> 13= Fe deficiency
< 13= beta thal likely

29
Q

What is the BW for thal?

A

Microcytic anemia

High RBC

Low or normal retic

Mentzer (MCV/RBC) < 13

30
Q

Normocytic anemia ddx.

A

Retic high= Hemolysis
Bleeding
Hypersplenism

In the RBC
Hemoglobin….
>Thalassemia
> Sickle Cell

Enzyme….
> G6PD
> Pyruvate Kinase Deficiency

Memb….
> Hereditary spherocytosis
> Eliptocytosis

Outside RBC (antibody)
> Immune = Coomb +
- AI hemolytic anemia

> Non immune= Coom (-)

  • HUS (uremic syn)
  • TTP (thrombotic thrombocytopenia purpura)
  • DIC (disseminated intravascular coag)
  • Hemangioma (Kasabach-Merrit syn)

Retic low/normal

  • Acute bleed
  • Anemia of chronic dx (RA, SLE, TB, malignancy)
  • Low cell lines= leukaemia, aplastic anemia
  • High Lines= infection
  • Mixed WBC + Plt= RF, meds
  • Normal WBC + Plt= TEC, early Fe deficiency
31
Q

What is TEC anemia?

A

Transient erythroblastopenia of childhood

  • temp cessation in RBC production
  • healthy
  • normocytic
  • low retic
  • usually 1 mo-6 year
  • last 1-2 month
  • recover
  • typically bone marrow as dx of exclusion (r/o bone marrow infiltrative dx)
32
Q

2 yo. well. Pallor. Hgb 80. Normocytic. Low Retic. Next investigation:

  • Bone marrow aspirate
  • Hgb electrophoresis
  • Coombs
  • Serum ferritin
A

Bone marrow aspirate (or ferritin?)

Even if TEC need to R/O bone marrow infiltrative like leukaemia.

DDX:

  • Acute bleed
  • Anemia of chronic dx (RA, SLE, TB, malignancy)
  • Low WBC + Plt= leuk, aplastic anemia
  • High WBC + Plt= infection
  • Mixed WBC + Plt= RF, meds
  • Normal WBC + Plt= TEC, early Fe deficiency
33
Q

list three features on CBC consistent with Iron deficiency:

A
  1. Microcytic hypo chromic **anemia (Hgb, MCV low)
  2. Low ferritin (low iron stores)
  3. Serum iron low
  4. Increased TIBC and low transferrin
  5. **RDW increased
  6. ** RBC low
  7. Retic normal or moderately up
  8. **WBC normal
  9. +/- Plt up
34
Q

Low energy. Pallor. Viral illness 1-2 wk ago. Sclera yellow. Hgb 70. Norm WBC. Norm Plt. Retic 24%. Type of anemia? Test to confirm? Tx?

A

Hemolytic normocytic anemia.

Likely: Acute idiopathic autoimmune hemolytic anemia

Test to confirm: DAT

Other: Smear (spherocytosis etc.)

Tx: Prednisone and IVIG
(If Hgb < 100 and Retic > 10%)

35
Q

What is fanconi Anemia?

A
AR or X linked
Most common inherited bone marrow failure (aplastic anemia sun)
Low IQ
Small eye, abN ear
CAM, hypo pigmented, 
Absent or abN thumb
36
Q

List long-term consequence of fanconi anemia?

A

Pancytopenia
AML
Squamous cell carcinoma of head and neck

37
Q

Aplastic Anemia + Hypopigmented skin or CAM + abnormal Thumbs

A

Fanconi Anemia

Note: aplastic anemia= bone marrow aplasia= pancytopenia.

  • Fanconi one congenital cause
  • Shwachman-Diamond (pancreatic, bone marrow, skeletal)
38
Q

All are features of iron deficiency anemia EXCEPT:

  • pica
  • koilonychia
  • cheilosis
  • mild scleral icterus
  • psychomotor retardation
A

Icterus= hemolysis + jaundice.

CC:

  • pallor of palm, creases, conjunctive
  • skull bosing
  • angular cheilitis
  • koilonychia (spoon nail)
  • pica, pagophagia (ice)
  • irritable, anorexia
  • tachy HR and high CO
  • cognitive defect
39
Q

What is the progression of Iron deficiency anemia BW?

A

Reduced serum ferritin (iron storage protein)

Serum iron down

Binding capacity of serum (serum transferrin) UP

Transferrin sat DROP

Not enough iron for heme= Anemia

Less Hgb in each= Microcytosis

Variation in cell size= RDW

Decrease in MCV

40
Q

Blood smear of iron deficiency of anemia show all BUT:

  • Heinz body
  • Poikilocytes
  • Microcytosis
  • Hypochromasia
  • Normal or low retic
A

Heinz body (inclusion in RBC due to damaged Hgb i.e. G6PD)

  • normal/high retic but low when iron substrate for RBC completely gone
  • poikilocyte (general term for abnormal shape RBC= can see elliptocytes/cigar shape)
41
Q

List 3 DDX on micro anemia that fail to respond to oral iron

A
  1. **poor compliance
  2. **poor dose
  3. **malabsorption of administered iron (taking with milk)
  4. ongoing blood loss
  5. B12 or folate deficiency
  6. **other: thal, anemia of chronic dx, lead poisoning etc.
42
Q

Kid with high intake milk. CBC show normocytic anemia with Hgb AS (sickle trait). Likely dx:

  • TEC
  • Iron deficiency
  • sickle cell dx
A

TEC

Transient erythroblastopenia of childhood

43
Q

Full term. Mom O(+). Well but pale. Hgb 70. Stable. Likely dx:

  • ABO incompatible
  • Chronic fetal maternal hemm
  • RH incompability
A

ABO incompatible

44
Q

Term BB. Midwife. Present at 7d. Hgb 70. MCV 112. Plt normal. Likely dx?

A

Vitamin K deficiency leading to hemorrhagic dx of newborn.

45
Q

List 4 ppt of neonatal hemophilia:

A
  • *1. bleed w/ circumcision
  • *2. Prolonged bleeding with BW (heel stick or venipuncture)
    3. Cephalohematoma
  • *4. Subgaleal Hemorrhage
  • *5. Intracranial hemorrhage
46
Q

What is Hemophilia A and B. Inheritance?

A
Hemophilia A= Factor 8
Hemophilia B= Factor 9
X chromosome (*M)

= low # or defective
= impaired secondary hemostasis

47
Q

Which BW is abnormal in hemophilia:

  • Plt
  • Bleeding Time
  • INR
  • aPTT
  • specific assay for Factor 8 and 9
A

PTT= UP
AND
Specific assay Fact 8+/- 9

48
Q

Who do you treat Hemophilia acutely and prophylaxis?

A

Acute bleeding= fresh frozen plasma (clotting factor) + Cryoprecipitate (factor 8C, vWF, factor 13)

Known: factor 8 or 9 concentrate

Prophylaxis infusions only if severe dx

*No ASA + NSAID

49
Q

4 etiology for non hemorrhagic (clot) stroke

A

Congenital heart dx

Inherited prothrombotic
> factor 5 leiden
> prothombin gene mutation

Acquired prothrombotic
> Protein C or S deficiency
> Antithrombin 3 deficiency

Other older:
> Sickle cell anemia
> Antiphospholipid antibodies
> OCP
> Pregnancy
50
Q

What is one serious complication of severe ITP:

A

CNS intracranial bleed

51
Q

What is an indication for bone marrow bx in child with ITP w/ plt 12.

  • ANC < 1
  • Previous steroid
  • Hgb < 115
  • Fever > 39
A

ANC < 1

Bx if R/O malignancy, bone marrow failure.
Steroid not indication for bx.

52
Q

Term baby with petechiae and bruising. Plt 12; CBC otherwise normal. Given plt and repeat 16. Mom plt 80. Best tx:

  • PLA-1 negative plt
  • IVIG
  • Washed maternal plt
A

IVIG

Reminder: PLA-1 negative plt NAIT and mom plt then normal.

53
Q

In which is plt transfusion indicated:

  • aplastic anemia
  • acute ITP
  • chronic ITP
  • HUS
  • ITP due to quinidine toxicity
A

Aplastic anemia

acute ITP only if intracranial bleed

54
Q

When do you infuse plt

A
  1. Overt bleeding and < 50
  2. Major procedure < 50
  3. Minor (LP, IV) <25
  4. Bone marrow failure and high risk (infection, organ failure, clot, mucosal bleeding) + < 20
  5. Bone marrow failure + low risk + < 10
55
Q

List 3 causes of aplastic anemia:

A

= bone marrow failure = pancytopenia

  1. Fanconi anemia
  2. Shwachman-Diamond Syn
  3. Sickle cell infected by parvovirus B19
56
Q

What is ITP?

A

Immune destruction of normal plt (usually < 100)

*autoantibody against plt membrane antigen

57
Q

T or F: ITP is the most common acute cause of thrombocytopenia in well kids.

A

True.

58
Q

T or F: young child with ITP more likely to have chronic ITP or systemic cause (like SLE).

A

False.

Teens with insidious onset likely to have chronic ITP or systemic dx.

Classic age: 1-4 y.o.

59
Q

Sudden bruising or petechiae in healthy child after viral illness or MMR vaccine. Likely dx?

A

ITP

= Idiopathic thrombocytopenia purpura

60
Q

T or F: You can see schistocytes in ITP.

A

False.

Plt cleared by reticuloendothelial system (spleen, liver).
If RBC break down= hemolysis= HUS, TTP, DIC

61
Q

T or F: Most acute ITP resolve on its own.

A

True.

70-80% within 3 mo.

No therapy
Educate
Counsel if mild/mod dx
- restricted contact sport if < 30
- d/c NSAID, aspirin
- careful w/ head trauma
62
Q

T or F: In acute ITP pharma decrease chance of serious bleed or chronic ITP

A

False.

Note: Chronic ITP= 12 mon.

63
Q

When do you give plt transfusion in ITP?

A

Life-threatening bleed

i.e. intracranial hemorrhage
> plt, IVIG, high dose steroids (methylpred), prompt consult to neuroSx and Sx

64
Q

T or F: further testing should be done to R/O secondary cause of ITP if chronic (min. 12 month).

A

True

  • Infection (HIV, hep C, CMV, EBV)
  • Bone marrow aspirate (aplastic anemia, bone marrow failure, CA)
  • AI (ANA, dsDNA)
  • Immunodeficiency (immunoglobulin level)
65
Q

Microangiopathic hemolytic anemia + thrombocytopenia + Acute kidney injury. E coli toxin etiology.

A

HUS

Hemolytic uremic syndrome
CC: abo pain, vomiting, diarrhea

66
Q

Which bacteria strain is most classic for HUS?

A

E coli O157:H7.

67
Q

T or F: PT test also called INR

A

True

68
Q
List typical pattern for Hemophilia vs. vWD vs. Vitamin K deficient vs. DIC.
_INR (PT)
_ aPTT
_ Bleeding Time
_ Other
A

Hemophilia
** aPTT HIGH. Rest Normal

vWD
**Normal INR/PT.
Rest N/high.

Vitamin K:
**INR/PT HIGH
** apTT HIGH
Normal plt + fibrinogen

DIC
*** INR and apTT high but plt + fibrinogen down
D-dimer UP

69
Q

T or F: you can use intranasal desmopressin for mild hemophilia A (factor 8)?

A

True

Otherwise factor concentrate, or FFP + cryoprecipitate if acute bleeding dx

70
Q

vWD patient with bleeding. Best treatment:

  • FFP
  • DDAVP
  • Cryoprecipitate
  • Plt
A

DDAVP

Type 1 (# issue) tx with this.

If type 2 or 3= vWF concentrate (plasma derived factor 8 concentrate have vWF, or recombinant vWF)

Cryoprecipitate otherwise reasonable if none above.

71
Q

Most consistent with DIC:

  • fibrinogen ++
  • PTT ++
  • PT/INR down
  • Low fibrin split product
A

PTT +++

DIC: inflammatory response + innate immune response + coat = DYSFN
= consume clotting factors, plt = tissue ischemia + hemolytic anemia.

ABC
+/- pRBC, plt, cryo (fibrinogen, vWF, factor 8C), FPP (replace coag factors + natural inhibitors)

72
Q

Most common inherited bleeding dx?

A

vWF

73
Q

15 y.o. with menorrhagia + epistaxis. Check for:

  • vWF
  • hemophilia C
  • factor 5 leiden mutation
A

vWF

Hemophilia C = deep bleeding
Factor 5 Leiden= clots

74
Q

Immunization with kid with hemophilia A:

  • defer
  • give Factor 8
  • pressure over site x 10 min. post-immunization
A

Pressure x 10 min.

+ clotting factor concentrate if needed

75
Q

List 5 causes of neutropenia

A
  1. Bone marrow suppression= **post viral infectious, **drug induced (pen, anticonvulsant), *** Chemo
  2. Bone marrow injured= aplastic anaemia (Fanconi), B12 and folate deficiency, ***Malignancy (leuk, lymph, mets)
  3. Destroyed= ***Autoimmune (SLE)
  4. Sequestered= ***Hypersplenism
Full DDX:
....ACQUIRED
> Malignancy
> Infections
- viral (EBV, RSV, Flu etc.)
- Bacterial sepsis
> Autoimm
- primary
- secondary (SLE, IBD, RA, Sjogren) 
> Drugs
- Chemo
- antipsychotic, anticonvulsant, antidepressant, Abx (pen, cephalo, vanco)
- gold, antithyroid med destroy neutro

Other
> Hypersplenism (sequester)
> Nutritional (vit B12, folic acid def)

… CONGENITAL
> Cyclic neutropenia
> Kostmann Syn (recurrent bacterial infection)
> Shwachman-Diamond (pancreas, metaphyseal dysostosis, neutropenia)
> Bone marrow aplasia (Fanconi, Blackfan-Diamond)
> TAR (lot plt, absent radii)

76
Q

DDX for neonatal thrombocytopenia:

A
  1. Sepsis and DIC, NEC
  2. TORCH infection
  3. NAIT (mom (-) plt antigen but dad (+))
  4. Maternal ITP
  5. Genetics (TAR syndrome, Fanconi anemia)
  6. Thrombosis
  7. IUGR, placental insufficiency
  8. Kasabach-Merritt syn
77
Q

What is NAIT?

A

Neonatal ALLO-immune thrombocytopenia

  • mom plt antigen (-)
  • dad plt antigen (+); BB (+)
  • mom destroy BB plt
    can occur in 1st preg
  • fall < 50 in days; rise 1-4 wk as antibody decline
  • R/O IVH via HUS
  • Tx: plt if < 30 (or <50 if prem)
    HPA (-) plt ( AKA PLA-1 neg)
    Option= IVIG.

Next pregnancy counselling KEY! Wkly IVIG in preg if BB (+) in next preg.

78
Q

What is neonatal autoimmune thrombocytopenia?

A

No severe low plt

Maternal ITP, SLE, Graves etc.
Mom antibody pass to body.

Recheck in 1 week
Spontaneous resolution

79
Q

Newborn w/ plt 12. Transfused plt and still 16. Mom CBC normal. What do you do?

  • Transfuse single donor plt
  • transfuse PLA-1 negative (HPA-1)
  • IVIG
  • Steroids
A

Transfuse PLA-1 negative

HPA-1 for NAIT

80
Q

T or F: Neonatal ALLoimmune 1st line is steroids and IVIG.

A

False.

Monitor
Transfuse if < 30
Either...
- washed maternal plt
- cross match for mom's plt
- PLA-1 negative
- random plt
81
Q

4 indications for bone marrow aspiration in setting of likely ITP:

A
  1. Abnormal WBC/diff (i.e. neutropenia)
  2. Unexplained anemia
  3. Hepatosplenomegaly or lymphadenopathy
  4. Bone or joint pain
82
Q

T or F: Splenectomy can sometimes be used for ITP

A

yes.

If > 4 and > 1 yr without response to tx w/ severe ITP

83
Q

14 y.o. with sickle and dacytlitis. Acute management. Prevention med?

A

Dactylitis= hand-foot
Pain: tylenol, NSAID, opioids
Hydration: PO or iV

Prevention: Hydroxyurea

84
Q

T or F: hydroxyurea reduce frequency of crisis and acute chest crisis?

A

True

85
Q

List 4 indications for blood transfusion in sickle cell

A

Acute Chest Syndrome
Aplastic Crisis
Splenic
Acute stroke

86
Q

Mom healthy. Her father has hemophilia. Risk of transmitting to own child:

  • none
  • 50% son will have and all daughters carrier
  • 50% son will have and 50% daughters carriers
A

Hemophilia A + B= X linked recessive

Maternal GF= XY= affected
Mom= carrier 
- Her kid's
50% boy will get X= affected
50% of F will get X= carrier
87
Q

Most common complication of beta thal major:

  • iron overload
  • megaloblastic anemia
  • cardiomyopathy
  • poor hematopoiesis
A

Iron overload

88
Q

Sickle. Cough. Tachypnea. R/O which complication

A

Acute Chest Crisis

89
Q

Sickle cell w/ recurrent chest crisis. What are they at increased risk of?

A

Recurrent cerebral infarct/ Stroke

PPT: neurocognitive and behavioural deficits

90
Q

Most common complication of hereditary spherocytosis

A

gallstones

91
Q

What is hereditary spherocytosis?

A
  • AD
  • most common RBC memb abN
  • North Europe esp
  • asymp -> hemolysis
  • gallstone common
  • rarely splenic crisis, gout, cardiomyopathy
  • spherocyte on smear
  • U/S gallstones
  • monitor growth, exercise tolerance, spleen size, anticipatory guidance for parvovirus aplastic crisis
92
Q

Severity of Rh incompatibility at birth predicted by:

  • cord Hgb
  • cord bili
  • prematurity
  • splenomegaly
  • maternal anti-D antibody
A

Cord Hgb

Note: cord bili can be high and expect rapid raise in bili in first 6hr

Note: high or rapid rise in IgG titre to D antigen suggest hemolytic dx

93
Q

Do IgM or IgG antibodies cross placenta?

A

IgG (GOOD)

94
Q

Sickle cell. Obese. RUQ pain. LFT up. Recommendation:

  • Cholecystectomy
  • Low fat diet
  • Ursodiol
A

Cholecystectomy for recurrent chole and gallstones.

High risk of black pigment stones and unconjug. bill. Obesity also RF.

95
Q

What tx proven to prevent stroke in sickle cell:

  • Transfusion
  • Folate
  • Hydroxyurea
  • Iron
A

Transfusion

96
Q

T or F: sickle cell associated strokes are usually subclinical.

A

True.

Hydroxyurea: reduce painful episodes, ACS and need for transfusions

97
Q

What is sickle cell disease?

A

AR

HbSS

RF: African, Caribbean, Mediterranean etc.

Deoxygenated = distort into sickle shape = stuck
1st ppt= dactylitis common

98
Q

What is sickle cell prevention protocol?

A

Pens till min. 5 y.o. (pneumococcal infection)
Folic acid till 5 y.o.
Hydroxyurea after that (increase HbF)
Vaccines + pneumo 23 (at 2 and 5 y.o.) and yearly influenza

99
Q

How do you manage acute stroke in sickle cell?

A

Apply O2 to keep sat > 96%
pRBC till 100g/L
Exchange to keep HbS < 30%
Peds Neuro

100
Q

What organisms are kids with Sickle at risk for?

A

Encapsulated:

“YES, Some Killers Have Pretty Nice Capsules”

Yersinia Petis
E coli. (some species)
Strep agalactiae (GBS)

Strep pneumoniae
Klebsiella
Haemophilus influnzae
Pseudomonas Aeuroginosa
Neisseria Meningitidis
Cryptococcus Neoformans
101
Q

If febrile sickle cell:

A

> 38.4
= Blood Culture
= IV Abx (ceftriaxone)
= +/- Admit min. 24 h

102
Q

How do you define chest crisis

A

Fever
New CXR infiltrate
+/- hypoxia/WOB/pain

  • 1X IVF
  • blood
  • O2
  • cephalosporin + macrolide
103
Q

List 3 indications for splenectomy:

A
Splenic trauma
Hereditary spherocytosis
Primary hypersplenism
Chronic ITP resistant to tx
Splenic abscess
Sickle Cell Disease w/ recurrent aplastic sequestration
104
Q

Teen with B symptoms. Supraclavicular node. Tx:

  • AUS
  • CXR
  • CT chest
  • Bone marrow aspirate
A

CXR

R/O mediastinal mass

105
Q

Name on virus associated with lymphoma

A

EBV
CMV
HHV-6

106
Q

Node in superclavicular area. Most helpful test:

  • Bx
  • Bartonella
  • Monospot/EBV
  • TB skin test
A

bx

107
Q

List 5 things on ddx for splenomegaly:

A
  1. Structural
    - congenital splenic hemangioma
    - hamartoma
    - cyst
  2. *Splenic trauma/ hemm
  3. Infectious
    * EBV
    * Bacterial (typhoid, cat scratch, TB)
    * Malaria
  4. Heme*
    - spherocytosis, G6PD, pyruvate kinase deficiency
    - sickle cell sequestration
    - thalassemia, osteoporosis
  5. *Blood/portal HTN
    - HF
    - portal HTN
    - cirrhosis
  6. Storage dx
    - Gaucher dx, Niemann-Pick
  7. Systemic
    - ** Malignancy (leuk, hodgkin)
    - Grandulomatous
    - Sarcoid
    - Amyloid
    - SLE, RA
108
Q

VERY white bones on XR. Pale. Hepatosplenoegamly. Likely Dx?

A

Osteopetrosis

  • AR
  • +++ Ca2+ bones
  • RTA
  • anemia, pancytopenia
  • compression of cranial nerves possible of skull sclerosed
  • no tx.
109
Q

BB with abdo mass. Low Plt. LLSB murmur and murmur over liver. US show cm mass. Next?

  • DIC
  • Bone marrow
  • Pul Imaging
  • Liver F’n
A

DIC

R/O Kasabach-Merritt Syn

110
Q

Child fell off bike. Right knee. Large yellow bruise on chest with R knee swelling.

  • Hemophilia
  • vWF
  • Thrombocytopenia
  • Child abuse
  • Herbal remedies
A

Hemophilia

111
Q

4 y.o. new refugee. H/a, dizzy, vague abdo pain and constipated. Short. Microcytic anemia with stifling. Next?

  • bone marrow aspirate + bx
  • lead level
  • Hgb electrophoresis
  • iron and ferritin level
A

Lead level

112
Q

Which is NOT a serious side effect of IVIG:

  • acute pancreatitis
  • aseptic meningitis
  • hemolytic anemia
  • transfusion related acute lung injury
  • thromboembolism
A

NOT: acute pancreatitis

113
Q

What do you expect on Hgb electrophoresis for alpha and beta thalassemia?

A

Alpha Thal
- Normal in adults w/ trait
- Newborn: +/- HbH or Barts
Suspect when: low MCV, normal iron, Normal HPLC/ electrophoresis

Beta Thal:

  • Absent or Down HbA
  • UP HbA2
  • +/- HbF UP