Common Variable Immunodeficiency with the Concomitant Diagnosis of an Inflammatory Myofibroblastic Tumor
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LETTER TO EDITOR
Common Variable Immunodeficiency with the Concomitant Diagnosis of an Inflammatory Myofibroblastic Tumor Craig Sewell 1,2 & Phuong Daniels 3
&
Jason Cottrell 2 & Robert Hostoffer 1,3,4
Received: 24 July 2020 / Accepted: 28 September 2020 # Springer Science+Business Media, LLC, part of Springer Nature 2020
Abbreviations IMT Inflammatory myofibroblastic tumor CVID Common variable immunodeficiency CT Computed tomography SMA Smooth muscle actin ALK Anaplastic lymphoma kinase
To the Editor, Common variable immunodeficiency (CVID) is a heterogenic, B cell–derived immunodeficiency that presents in adulthood. CVID primarily manifests with recurrent infections and induces a chronic inflammatory state that leads to end organ damage. CVID has also been shown to be associated with autoimmunity and malignancies, particularly gastric cancers and lymphomas [1]. Inflammatory myofibroblastic tumor (IMT) is a rare inflammatory pseudotumor that is diagnosed in less than 1 in one million people with prevalence of 0.04% worldwide. IMT typically presents in the pediatric or young adult population as a benign self-limited solitary nodule of the lung or on mucosal surfaces [2]. Although malignancies are known to be associated with CVID, this particular tumor has never been previously associated. We present the first case of an invasive IMT of the lung in a patient concurrently diagnosed with CVID. A 28-year-old female presented to the emergency department with 3 weeks of persistent cough with green sputum that
* Phuong Daniels [email protected] 1
University Hospitals Cleveland Medical Center, Cleveland, OH, USA
2
Riverside Methodist Hospital, Columbus, OH, USA
3
Lake Erie College of Osteopathic Medicine, Erie, PA, USA
4
Allergy/Immunology Associates Inc., Mayfield Heights, OH, USA
progressed to hemoptysis, fever, night sweats, emesis, and decreased appetite. Upon admission, the patient was tachycardic, tachypneic, and febrile with T-max of 100 °F. The physical examination revealed bronchus sound throughout the bilateral chest wall and painful cervical lymphadenopathy, with absence of hepatomegaly or splenomegaly. The laboratory studies and cultures from blood, bronchoscopy lavage, and biopsy samples were without evidence of viral, fungal, or bacterial growth. The patient’s past medical history was significant for selective IgA deficiency (< 20 mg/dL) diagnosed when she was 4 years old due to recurrent sinusitis and bronchitis. She has not had any follow-up in the past 15years. Based on this significant medical history, an immunoglobulin panel was assessed and demonstrated new findings of unmeasurable IgA (< 10 mg/dL), low IgG levels (< 300 mg/dL), and low IgM (12 mg/dL). The patient was subsequently diagnosed with CVID, and an intravenous immunoglobulin replacement was initiated. A chest computed tomography (CT) demonstrated a 4.5 × 3.9 cm right hilar mass encasing the right lower lobe pulmonary artery with narrowing of the bronchus intermedius. A positron emission tomography-computed tomography s
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