History

History (1 of 2 slides)

History (2 of 11 slides)

A 38 year-old woman was recently told by her PCP that her white cell count is low. She is not sure whether she has had a previous complete blood count or whether her white cell count has low in the past. She has no history of infections, fevers, night sweats or weight loss. She denies a history of mouth ulcers. She has no past medical or surgical history. There is no family history of leukopenia, neutropenia or other hematological conditions. She is an non-smoker and drinks 1-2 glasses of wine per week. She works as an administrative assistant. She is on no medication and does not have any known allergies to drugs.

Which statements are true regarding neutropenia and infections?

The most frequent sites are mucocutaneous

Oral manifestations (gingivitis, chronic periodontitis, mouth ulcers) are almost constant in cases of profound neutropenia

Infections are most commonly bacteria, particularly Gramnegative bacilli and staphylococci

Fungal infections are uncommon in chronic neutropenia, except in the context of chemotherapy.

Viral infections are also rare in chronic neutropenia unless the neutropenia is associated with a deficit in cellular or humoral immunity

patients with neutropenia may present with nonspecific signs and symptoms of infection (due to any cause) including:

fever
oral inflammation (mouth ulcers, gingivitis, periodontitis, tooth loss and replacement)
cellulitis
sinusitis
otitis
pharyngitis
pneumonia
gastrointestinal symptoms
perirectal infections
deep tissue infections
septicemia
tonsillitis

Patients may present with fever only; neutropenia is associated with reduced inflammatory response and may present with less erythema, fluctuance, or pus

What important piece of data are we missing from the history?

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Ethnicity: African

Clinical presentation according to underlying cause of neutropenia:

Cause of neutropenia (adult)	Presentation
*Congenital*
Benign ethnic neutropenia	Usually incidental detection with no recurrent infections; mild, chronic neutropenia (ANC > 1 × 10⁹/L) with no history of recurrent infections
Cyclic neutropenia	Usually diagnosed during second year of life, may be later; main manifestation recurrent acute stomatological disorders; 3- to 6-day periods of mild-to-severe neutropenia occurring every 21 days; history of painful mouth ulcers, fever, and bacterial infections during period of neutropenia
Severe congenital neutropenia	Usually diagnosed in children with history of recurrent severe infections; extremely low neutrophil counts (often < 0.2 × 10⁹/L) or frank agranulocytosis; severe, chronic infections developing in first months of life; if untreated, may present with chronic gingivitis, oral ulcers, skin abscesses, septicemia, or recurrent pneumonia; presentation associated with congenital neutropenias with nonmyeloid manifestations, e.g. Kostmann syndrome
*Acquired*
Primary autoimmune neutropenia (also called benign chronic neutropenia)	May present in infants and children; most common form of chronic neutropenia in childhood; incidental diagnosis on routine CBC, without history of infection in adults
Secondary autoimmune neutropenia	Usually diagnosed incidentally on a routine complete blood count without history of recurrent infections

History:

ask about prior history of neutropenia or recurrent infections
obtain records of all prior lab panels for comparison of complete blood count (CBC)/evaluation for chronic neutropenia
ask about symptoms associated with infections
ask about medication history; drug-induced neutropenia usually occurs rapidly, ranging from within 1-2 hours to up to 1-2 days after drug exposure
ask about history of autoimmune diseases, for example:
- rheumatoid arthritis (RA)
- Felty syndrome
- systemic lupus erythematosus (SLE)
ask about dietary habits or history of:
- anorexia nervosa
- vitamin B12 deficiency
- folate deficiency
- gastric bypass surgery (copper deficiency)
assess ethnic background to determine possibility of constitutional/ethnic neutropenia
inquire about family history
- congenital neutropenia (severity of illness may vary between family members who have the same genetic defect)
- hematologic malignancies
- severe infections