Diseases in the Follow-up
Infectious and noninfectious diseases of patients during follow-up
period were given in supplementary table 2.
Infections: During the follow-up, recurrent URTI (60.6%) was
the most common finding followed bypneumonia (36,3 %). The frequencies
of infections in sIgMD are shown in Table 3.
Malignancy: Malignancy developed in three (9%) out of 33
patients. These were acute myeloid leukemia (n=2), and tubular adenoma
in sigmoid colon (n=1). A 4 year-old patient presented with
neuroblastoma, and after four years of follow-up, AML developed in the
same patient. He was treated successfully.
Autoimmune and Inflammatory Diseases: Six patients with sIgMD
was additionally diagnosed with autoimmune and inflammatory diseases,
such as, Behcet’s disease (n=2), immune thrombocytopenic purpura (ITP)
(n=1), diabetes mellitus, Guillain Barre syndrome (n=1) and Crohn
disease (n=1) respectively.
Genetic disorders: Six (18%) patients was found to have
genetic disorders, trisomy 21 (n=2), 22q11.2 deletion (n=1), 1p deletion
(n=1), Cohen syndrome (n=1) and CHARGE syndrome (n=1).
Among sIgMD patients who were younger than five years old, two more
patients had the diagnosis of partial DiGeorge syndrom.
Atopy: Five patients (15,1%) had asthma, and one was wheezy
infant. One out of 5 patients was treated also for allergic rhinitis.
Others: One patient was diagnosed with osteopetrosis, 12
patients (36,3%) had sIgMD with no additional disorder or disease.
Laboratory Findings
Complete blood counts: Lymphopenia was detected in fifteen
(35,7%), neutropenia was detected in fourteen (33,3%), leukopenia was
seen in ten (23%) and hypereosinophilia was seen in four patients
(9,5%).
Immunoglobulin levels: The IgG and IgA levels were in normal
range as all the patients fullfilled the sIgMD criteria. Six (18%)
patients have high IgE levels. Immunoglobulin levels of patients are
given in Supplementary Table 1.
Lymphocyte subsets: They were in normal range on admission in
all patients.