Newman - Non-neoplastic WBC Disorders in Pediatrics Flashcards
WBC numbers
Most = Never > Let > Monkeys (monos) > Eat > Bananas = Least
Three criteria for “Worrisome” recurrent infections (start thinking about immunodeficiencies).
- Two or more severe infections in one year
- Three or more respiratory infections in one year (that not URI’s, like bad pneumonias)
- The need for antibiotics for 60 days per year.
Three clinical features suggestive of a primary immunodeficiency
- FH of immunodeficiency or unexplained death *
- Failure to gain wt or grow normally
- IV antibiotics and/or hospitalization to clear infections
Condition with complete T cell deficiency
SCID - severe combined immunodeficiency
Three conditions with partial T cell deficiency
- DiGeorge
- **Ataxia telangiectasia (combined B and T cell)
- Wiskott-Aldrich syndrome (combined B and T cell)
Main pathogens in T cell deficiency infections
- Intracellular organisms (HSV, mycobacterium, listeria)
2. Fungi
Primary immunodeficiencies are… congenital or acquired?
congenital
Secondary - meds
Five pertinent things about BIRTH HISTORY in someone with immunodeficiency
- Maternal illness (HIV, CMV, drugs, alcohol)
- Neonatal history
- Feeding history
- Duration of breast feeding
- WHEN THE CORD FELL OFF (leukocyte adhesion defect)
***Chediak Higashi syndrome
***Immunodeficiency + shows progressive NEUROLOGIC dysfunction in young adults + albinism
Amish people associated with ___. This is AR or AD?
Ataxia telangiectasia, AR
What is Leukocyte Adhesion Defect? High count in what leukocyte?
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.
***Between 2yo-6yo with recurrent URI, think what main deficiency?
***IgA deficiency
URI that cause/signs of immunodeficiencies
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
RECURRENT BACTERIAL MENINGITIS and SEPSIS blood and brain infection) suggest.
An antibody deficiency or COMPLEMENT DEFECT.
Signature organisms in: B cell abnormalities
recurrent sinopulmonary infections
with encapsulated organisms (pneumococcus, H.
influenzae type b)
Signature organisms in: Agammaglobulinemia or complement defect
recurrent pneumococcal disease
Signature organisms in: SCID and other primary or secondary T cell immunodeficiencies (like HIV).
Looks like what on CXR?
Pneumocystis jiroveci (carinii) pneumonia
CXR = ground glass
Signature organisms in: Phagocytic disorders or profound antibody or T cell immunodeficiency
pseudomonas sepsis, fungus
Signature organisms in: X-linked agammaglobulinemia (XLA) –>
Recurrent bacterial meningits, is either:
enteroviral meningoencephalitis
- XLA, no immunoglobulins
- complement
Signature organisms in: CGD (Chronic Granulomatous Disease)
abscesses or soft tissue infections secondary to
Aspergillus, Staphylococcus aureus, coagulase-negative
staphylococcus, Serratiamarcescens, Chromobacterium
violaceum
Signature organisms in: Hyperimmunoglogulin E syndrome
recurrent staphylococcal
skin infections, abscesses, lung cysts, or pneumonia
Signature organisms in: Abnormal T cell immunity
prolonged and severe candidiasis
involving the buccal mucosa, tongue, and palate may be the earliest sign
Signature organisms in: Deficiencies of the late components of complement (C5 to
C9)
invasive infection with Neisseria species (meningitidis,
gonorrhoeae)
Signature organisms in: PID
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)
Clinical patterns suggestive of immunodeficiency in what: Ataxia, telangiectasia, and developmental delay
in ataxiatelangiectasia
***Clinical patterns suggestive of immunodeficiency in what: Congenital heart disease, developmental delay,
dysmorphic facies with low-set ears, hypertelorism,
downturning eyes and micrognathia **
in DiGeorge syndrome
Clinical patterns suggestive of immunodeficiency in what: Extensive warts or molluscum contagiosum
in T cell disorders, innate immune defects, or the warts hypogammmaglobulinemia infections myelokathexis (WHIM) syndrome
**ANC less than what has serious risk of infection?
**less than 500
Kostmann Syndrome
AR. Severe neutropenia , ELA-2 mutations, increased risk of AML.
Manifests as life threatening pyogenic infections in infancy.
Cyclic neutropenia
Neutropenia count less than 200 for 3-7 days every 3 weeks (15-35 days).
*Schwachman-Diamond syndrome is what and what inheritance?
- AR
- Triad of (1) neutropenia, (2) exocrine pancreas insufficiency, and (3) skeletal abnormalities
- Inc risk of MDS or leukemia
Fanconi anemia
perfect example of COMPLETE BM failure.
Partial oculocutaneous albinism - think what?
Chediak Higashi syndrome
AR
Recurrent purulent infections with fungal or bacterial catalase-positive organisms, usually starting in infancy, chronic inflammatory granulomas - this is what?
chronic granulomatous disease
Nitroblue tetrazolium test and absence of generation of superoxide is what disease?
chronic granulomatous disease
common viruses causing neutropenia
-**common other cause of neutropenia
EBV
other - DRUG INDUCED NEUTROPENIA - cytotoxic agents for treatment of malignancy
DNAAACP - causes of eosinophila
- 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)
