CASE DESCRIPTION
Herein, we introduce a 9-year-old female patient who started to have
symptoms at the age of 5, with arthritis being her first presenting
condition. She was hospitalized with the impression of pneumonia and
unclassified rheumatic disease at 7 years old due to cough,
polyarthritis, and splenomegaly. Her laboratory tests (including
CBC-DIFF, ESR, and FANA) and bone marrow aspiration were normal. Her
bone scan suggested a systemic disease with multi-articular involvement.
She was discharged with ibuprofen and methotrexate (MTX). She also
received some courses of intra-articular corticosteroids.
At the age of 8, she developed pneumonia and was hospitalized again.
Pulmonary consolidation with pleural effusion was reported on her chest
X-ray at the time. On her chest HRCT scan, mediastinal and hilar
lymphadenopathy was reported on the right side with a slight left-side
deviation of the trachea and marked extrinsic compression upon the right
middle lobe (RML) bronchus. RML collapse was noted with cylindrical
bronchiectasis. Collapse-consolidation in the posterior and medial basal
right lower lobe (RLL) was present. Some scattered bilateral ill-defined
pulmonary nodules–mostly pleural-based–were seen.
The patient’s parents were 1st cousins and other than
the mother having a history of a single spontaneous abortion, the
parents had no history of any kind of disease. Given the patient’s
condition and the severity of her presenting symptoms, the appropriate
laboratory tests including the flow cytometric study of immunological CD
markers were ordered. The results of these tests are represented in
Table 1 in detail.
The results represent an immunodeficient state characterized by
hypogammaglobulinemia with a normal number of circulating B cell
lymphocytes. Also, a normal percentage of leukocyte adhesion markers was
reported, and the total number of T cell lymphocytes as well as CD4+ and
CD8+ T cells were in the normal range. This is suggestive of an
immunodeficiency disorder affecting most prominently the function of B
cells. Other findings in this patient include a low hemoglobulin level,
thrombocytopenia, positive stool calprotectin indicative of inflammation
of the bowels, and a slight increase in liver enzymes.
Based on these results, appropriate treatment with a monthly dose of
Intravenous Immunoglobulin (IVIG), and prophylactic antibiotic was begun
and after one month, she had an IgG level in the normal range in
combination with a negative CRP. On her follow-up chest CT scan
performed after 4 months, multiple bilateral pulmonary nodules
(innumerous) with a maximum diameter of 25 mm in both lungs were seen.
Nine months after the patient’s last hospitalization with pneumonia, she
was hospitalized again due to fever and chills, and a productive cough
that had lasted for 2 weeks. On physical examination, she had
splenomegaly and clubbing of the fingers. She was admitted to the COVID
PICU with a positive COVID-19 PCR. On her Chest HRCT scan, patchy ground
glass opacities with crazy pattern in both lungs was shown, which could
be due to bronchopneumonia (suggestive of COVID-19 infection). She
received vancomycin, meropenem, interferon beta 1-alpha, remdesivir,
dexamethasone, IVIG, ganciclovir, ASA, and voriconazole. She was
discharged from the COVID ward after 14 days with the treatment plan of
monthly IVIG and a prophylactic dose of cefixime.