Newman - Non-neoplastic WBC Disorders in Pediatrics

Question 1

WBC numbers

Answer 1

Most = Never > Let > Monkeys (monos) > Eat > Bananas = Least

Question 2

Three criteria for “Worrisome” recurrent infections (start thinking about immunodeficiencies).

Answer 2

1. Two or more severe infections in one year
2. Three or more respiratory infections in one year (that not URI’s, like bad pneumonias)
3. The need for antibiotics for 60 days per year.

Question 3

Three clinical features suggestive of a primary immunodeficiency

Answer 3

1. FH of immunodeficiency or unexplained death *
2. Failure to gain wt or grow normally
3. IV antibiotics and/or hospitalization to clear infections

Question 4

Condition with complete T cell deficiency

Answer 4

SCID - severe combined immunodeficiency

Question 5

Three conditions with partial T cell deficiency

Answer 5

1. DiGeorge
2. **Ataxia telangiectasia (combined B and T cell)
3. Wiskott-Aldrich syndrome (combined B and T cell)

Question 6

Main pathogens in T cell deficiency infections

Answer 6

1. Intracellular organisms (HSV, mycobacterium, listeria)

2. Fungi

Question 7

Primary immunodeficiencies are… congenital or acquired?

Answer 7

congenital

Secondary - meds

Question 8

Five pertinent things about BIRTH HISTORY in someone with immunodeficiency

Answer 8

1. Maternal illness (HIV, CMV, drugs, alcohol)
2. Neonatal history
3. Feeding history
4. Duration of breast feeding
5. WHEN THE CORD FELL OFF (leukocyte adhesion defect)

Question 9

***Chediak Higashi syndrome

Answer 9

***Immunodeficiency + shows progressive NEUROLOGIC dysfunction in young adults + albinism

Question 10

Amish people associated with ___. This is AR or AD?

Answer 10

Ataxia telangiectasia, AR

Question 11

What is Leukocyte Adhesion Defect? High count in what leukocyte?

Answer 11

Rare autosomal recessive disorder of leukocyte function (1/10,000,000).
Cord doesn’t fall off. Recurrent bacterial and fungal infections without pus accumulation. Depressed inflammatory response despite striking blood neutrophils.

Question 12

***Between 2yo-6yo with recurrent URI, think what main deficiency?

Answer 12

***IgA deficiency

Question 13

URI that cause/signs of immunodeficiencies

Answer 13

nystatin-resistant or recurrent candidiasis (after 3 months of age), gingivitis, stomatitis, oral ulcerations occur in T cell and phagocytic cell disorders

fungal infection=T ells, HIV

Question 14

RECURRENT BACTERIAL MENINGITIS and SEPSIS blood and brain infection) suggest.

Answer 14

An antibody deficiency or COMPLEMENT DEFECT.

Question 15

Signature organisms in: B cell abnormalities

Answer 15

recurrent sinopulmonary infections
with encapsulated organisms (pneumococcus, H.
influenzae type b)

Question 16

Signature organisms in: Agammaglobulinemia or complement defect

Answer 16

recurrent pneumococcal disease

Question 17

Signature organisms in: SCID and other primary or secondary T cell immunodeficiencies (like HIV).

Looks like what on CXR?

Answer 17

Pneumocystis jiroveci (carinii) pneumonia

CXR = ground glass

Question 18

Signature organisms in: Phagocytic disorders or profound antibody or T cell immunodeficiency

Answer 18

pseudomonas sepsis, fungus

Question 19

Signature organisms in: X-linked agammaglobulinemia (XLA) –>

Recurrent bacterial meningits, is either:

Answer 19

enteroviral meningoencephalitis

1. XLA, no immunoglobulins
2. complement

Question 20

Signature organisms in: CGD (Chronic Granulomatous Disease)

Answer 20

abscesses or soft tissue infections secondary to
Aspergillus, Staphylococcus aureus, coagulase-negative
staphylococcus, Serratiamarcescens, Chromobacterium
violaceum

Question 21

Signature organisms in: Hyperimmunoglogulin E syndrome

Answer 21

recurrent staphylococcal

skin infections, abscesses, lung cysts, or pneumonia

Question 22

Signature organisms in: Abnormal T cell immunity

Answer 22

prolonged and severe candidiasis

involving the buccal mucosa, tongue, and palate may be the earliest sign

Question 23

Signature organisms in: Deficiencies of the late components of complement (C5 to
C9)

Answer 23

invasive infection with Neisseria species (meningitidis,

gonorrhoeae)

Question 24

Signature organisms in: PID

Answer 24

Primary Immunodeficiency suggested with vaccine failure or with infection with
vaccine strains following the administering of LIVE VACCINES (oral rotavirus, oral MMR –> live vaccines) polio, measles, varicella, BCG)

Question 25

Clinical patterns suggestive of immunodeficiency in what: Ataxia, telangiectasia, and developmental delay

Answer 25

in ataxiatelangiectasia

Question 26

***Clinical patterns suggestive of immunodeficiency in what: Congenital heart disease, developmental delay, dysmorphic facies with low-set ears, hypertelorism, downturning eyes and micrognathia **

Answer 26

in DiGeorge syndrome

Question 27

Clinical patterns suggestive of immunodeficiency in what: Extensive warts or **molluscum contagiosum**

Answer 27

in T cell disorders, innate immune defects, or the **warts** hypogammmaglobulinemia infections myelokathexis (WHIM) syndrome

Question 28

**ANC less than what has serious risk of infection?

Answer 28

**less than 500

Question 29

Kostmann Syndrome

Answer 29

AR. Severe neutropenia , ELA-2 mutations, **increased risk of AML.** Manifests as life threatening pyogenic infections in infancy.

Question 30

Cyclic neutropenia

Answer 30

Neutropenia count less than 200 for 3-7 days every 3 weeks (15-35 days).

Question 31

***Schwachman-Diamond syndrome is what and what inheritance?**

Answer 31

* **AR** * **Triad of (1) neutropenia, (2) exocrine pancreas insufficiency, and (3) skeletal abnormalities** - Inc risk of MDS or leukemia

Question 32

Fanconi anemia

Answer 32

perfect example of COMPLETE BM failure.

Question 33

Partial oculocutaneous albinism - think what?

Answer 33

Chediak Higashi syndrome | AR

Question 34

Recurrent purulent infections with fungal or bacterial catalase-positive organisms, usually starting in infancy, chronic inflammatory granulomas - this is what?

Answer 34

chronic granulomatous disease

Question 35

**Nitroblue tetrazolium test** and absence of generation of superoxide is what disease?

Answer 35

chronic granulomatous disease

Question 36

common viruses causing neutropenia -**common other cause of neutropenia

Answer 36

EBV other - DRUG INDUCED NEUTROPENIA - cytotoxic agents for treatment of malignancy

Question 37

DNAAACP - causes of eosinophila

Answer 37

- Drugs - Neoplasm - Atopic Disease - allergy, asthma, eczema - Addison disease - primary adrenal insufficiency - AIN - acute interstitial nephritis - Collagen Vascular disease - -SLE, Churg-Strauss vasculitis - Parasites - ascaris (loeffler eosinophilic pneumonitis)