Dr. Newmann - Red/White Cell Disorders Pediatrics

Question 1

When does the infant have the lowest amount of hemoglobin

Answer 1

2mos

Question 2

2,3-DPG concentration does what to O2 distribution curve

Answer 2

Shifted to the right (lower affinity of O2, since tissues signal they need O2)

Question 3

Anemia does what to the heart

Answer 3

Increase CO

Murmur can be heard due to low viscosity

Question 4

history that can point to anemia

Answer 4

Infection , travel, exposures, chronic illness, anemia in family, splenomegally, jaundice, early onset gallstones

Question 5

Anemia SX that are common to see on someone

Answer 5

Pale, irritability, sleepiness, low exercise tolerance, bloody stools, LAD, fever, viral Sx, heavy menses, SOB, cough

Question 6

Diamond-Blackfan Syndrome

Answer 6

Congenital only RBC aplasia

• presents in infancy (erythroid precursor apoptosis)
• low reticular count
• macrocytic anemia

Question 7

Fanconi Anemia

Answer 7

Inherited aplastic anemia (most common)**

• macrocytic anemia
• LOW reticular count
• leukopenia + thrombocytopenia (low RBC, plt, WBC)= apoptosis in BM
• usually young up to 10yo when DX**
• can become panocytopenia

Question 8

IDA in pediatric patients

Answer 8

• usually from not eating enough Fe
• microcytic, hypochromic high RDW
• HIGH transferrin, LOW ferritin and FE sat
• SX : pale, and big cow milk drinker (you get Fe from mothers breast milk)**

Question 9

Mentzer Index

Answer 9

When pt had mild microcytic anemia : MCV/RBC

1. <13 = IDA
2. > 13 = B-thalassemia trait
3. = 13 = indeterminate

Question 10

How to calculate ANC (Absolute Neutrophil Count)

Answer 10

ANC = (%N +%Bands) (WBC) / 100
Mild : 1000-1500
Moderate : 500-1000
Severe neutropenia : 500

Question 11

What drug causes Neutropenia

Answer 11

Chemotherapy

Question 12

Kostmann Syndrome

Answer 12

(Severe Congenital Neutropenia) AR

• Life threatening pyogenic infections early in life
• Impaired myeloid differentiation caused by maturation arrest of N precursors
• ANC <200
• high risk of AML

Question 13

Cyclic Neutropenia

Answer 13

Cyclic Fever, oral ulcers, gingivitis, periodontal disease, bacterial infections **
- stem cell regulatory defect resulting in defective maturation **
- ANC <200 for 3-7 days every 15-35 days **
(NO RISK for any AML)**

Question 14

Schwachman- Diamond Syndrome

Answer 14

TRIAD**
1. Neutropenia
2. Exocrine pancreas insufficiency
3. skeletal abnormalities
= X N mobility, migration, chemotaxis, count
- (higher risk to get MDS or other leukemia)

Question 15

Fanconi Anemia SX and when do you usually see it, and in risk of what

Answer 15

BM cant make any cells lines normally (aplastic anemia) = can become panocytopenia

1. GU + Skeletal anomalies, increased chr fragility
2. First 10 years of life
3. AML, Brain tumors, Wilms tumor

Question 16

Leukocyte adhesion deficiency
SX
Why
Risks

Answer 16

Rare

1. • Delayed separation of umbilical cord stump (3weeks)**
   • bacteria and fungal infections with NO PUS**
   • poor wound healing
2. N can adhere to cross BV
3. No risks

Question 17

Hyper-immunoglobulin E syndrome (Job syndrome)
What
Why
Risk

Answer 17

• TRIAD **
  1. Severe eczema
  2. Recurrent staph skin infection (or other bacteria)
  3. Recurrent pulmonary infections (bacteria/fungal)
• no N chemotaxis and high IgE
• Hodgkin lymphoma risk

Question 18

Chediak-Higashi Syndrome
SX
Why
Risks

Answer 18

1. Oculocutaneous albinism, neuropathies, recurrent pyogenic infections
2. No N chemotaxis/degranulation, X granulopoisis (die dry age 20yo)LYSOSOME BREAKDOWN PROBLEM
3. No risks

Question 19

Chronic granulomatous disease
SX
Why
Risks

Answer 19

1. Recurrent purulent infections (fungal/bacterial), CATALASE +
   (Usually infancy, chronic inflammatory granulomas)
2. X oxidative metabolism, X superoxide in N
   (X-linked)
3. No risks

Question 20

Parovirus B19

Answer 20

5th disease of childhood
- bright red cheeks, diffuse lacy red rash)
Anemia/ ( can cause thrombocytopenia +neutropenia), I think aplastic anemia

Question 21

Digeorges Syndrome

What is it and causes what

Answer 21

X thymus

Causes T-cell immunodeficiency

Question 22

Leading cause of death in infancy in US

Leading cause of death in due to disease

Answer 22

1. Unintentional injuries (accidents)

2. Brain Cancer and Leukemia

Question 23

Most common malignancy in 15-19yo and risk factors to this

Answer 23

1. Hodgkin Lymphoma

2. HHV-6, cytomegalovirus, EBV

Question 24

B signs for Hodgkin’s lymphoma

Answer 24

1. Unexplained fever (102.2F, 39C)
2. Wt loss > 10% over 6 months
3. Drenching night sweats

Question 25

When should I do a chest X-ray in a pt with lymphadenopathy

Answer 25

1. Any pt with unexplained LA, with no association to inflammation or infection process (rule out mediastinal mass*) 2. Any pt with persistent LAD and with cough, SOB, CP 3. Pt with lymphoma

Question 26

Most common type of lymphoma in children and adolescents

Answer 26

Non-Hodgkin lymphoma

Question 27

Wiscott-Aldrich syndrome

Answer 27

- X-linked**** - TRIAD******** 1. Ear infections/ sino-pulmonary infections 2. Severe atopic dermatitis 3. Thrombocytopenia (bleeding) - RISK TO GET NON-HODGKIN LYMPHOMA

Question 28

3 types of Burkitts lymphoma

Answer 28

1. Sporadic : abd disease, EBV, Africa 2. Endemic Type : head,neck, Africa, EBV/ Malaria 3. Immunodeficiency- restated: immunosuppressive drug use

Question 29

Number 1 malignancy in children is what

Answer 29

Leukemia (ALL is 97%)

Question 30

Which genetic condition can one be born with that is associated with AML and ALL

Answer 30

Trisomy 21

Question 31

Plt counts normal Severe Life threatening

Answer 31

150,000-450,000 20,000 10,000

Question 32

Decreased Plt production caused by what 3 causes

Answer 32

1. VIT B12, folate, FE deficiency 2. BM problem : AA, leukemia, lymphoma 3. Genetic problem : Wisconsin-Aldrich, Fanconi, schwachman-diamond, thrombocytopenia-absent-radius syndrome (TAR)

Question 33

Causes for increased plt destruction and consumption

Answer 33

1. ITP 2. HUS/DIC 3. TTP 4. Kasabach-Merritt phenomenon

Question 34

Reasons for plt sequestration

Answer 34

Hypersplenism | Von-Willebrand disease

Question 35

ITP

Answer 35

Immune thrombocytopenia purpura ANTI-plt antibodies (50% filled by viral infection lasting 1-3 weeks) <20,000plt - normal PT and PTT - normal other cell lines (otherwise check BM) SX : usually normal, bleeding

Question 36

Kasabach- Merritt phenomenal

Answer 36

Thrombocytopenia + hypo-fibrinogenemia + GIANT HEMANGIOMAS + intravascular coagulation

Question 37

3 red flag sx in thrombocytopenia pt

Answer 37

1. Panocytopenia sign 2. Raised LAD (cancer can be it) 3. Renal impairment

Question 38

Acute vs chronic ITP | ACUTE

Answer 38

``` ACUTE ITP : 2-6yo 1-3 week infection before <20,000 plt High lymphocytes 80% spontaneous remission (2-6 weeks) ```

Question 39

Acute vs chronic ITP | Chronic

Answer 39

``` 20-40yo Female No infection before 30,000 - 80,000plt Low lymphocytes Mo-years (no spontaneous remission usually) ```

Question 40

HUS

Answer 40

Hemolytic uremic syndrome - after acute gastroenteritis - TRIAD 1. Microangiopathic hemolytic anemia 2. Thrombocytopenia 3. Acute renal failure - blood in urine, if proteinuria then worse prognosis

Question 41

Which organism is usually the cause of gastroenteritis cussing HUS

Answer 41

E. coli 0157:H7