Neutropenia is characterized by decrease in the absolute number of circulating
neutrophils and an increase susceptibility to
infections. The current study was performed in order to explain the clinical and
laboratory findings of
patients with antibody
deficiency disorders associated
neutropenia. The
patients'
records of 19 neutropenic cases out of 207
patients with antibody deficiencies,
who had been referred to
Children's Medical Center and enrolled in Iranian primary immunodeficiency
registry, were reviewed. Nineteen cases [14
male and 5
female], with a mean age of 10.7 +/- 5.7 years, were associated with
neutropenia [9.2%]. The disorders with associated
neutropenia were Hyper
IgM syndromes [3 of 8],
Common variable immunodeficiency [13 of 109], and X-linked
agammaglobulinemia [3 of 45]. The median age for the onset of
disease and
diagnosis age were 15 months [1-134] and 3.8 years [6 months-13 years], respectively. The most common
infections during the
course of illness were
pneumonia [13 cases],
diarrhea [12 cases],
oral candidiasis [9 cases],
otitis media [6 cases],
sinusitis [6 cases], cutaneous
infections [5 cases], and
abscess [5 cases]. Other less frequent
infections were
conjunctivitis,
oral ulcers,
meningitis, and
osteomyelitis. Three neutropenic
patients died because of
recurrent infections.
Neutropenia may occur in any of the primary immunodeficiency disorders. Persistent or severe
infections always pose a supposition, which deserves further evaluation for detecting an underlying immune
deficiency syndrome and
neutropenia, since a delay in
diagnosis may result in a serious organ damage or even
death of the
patient