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.