Thymoma-associated immunodeficiency: a diagnostic challenge for the clinician

Narahari, Narendra Kumar; Gongati, Paramjyothi Kruparao; Kakarla, Bhaskar; Nizami, Mohammed Ismail; Boddula, Rajendra Prasad; Sattavarapu, Laxma Reddy

doi:10.1177/0218492316687934

Cited by 8 publications

(8 citation statements)

References 6 publications

(14 reference statements)

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“…Often, these tumors are incidental findings upon physical examination or when patients present with chest pain and shortness of breath. Sometimes thymomas are detected while investigating symptoms associated with paraneoplastic syndrome or autoimmune disorder, such as myasthenia gravis (4, 5). Computed tomography (CT) plays a major role in identifying and characterizing thymomas (6, 7).…”

Section: Introductionmentioning

confidence: 99%

Relationship Between Computed Tomography Imaging Features and Clinical Characteristics, Masaoka–Koga Stages, and World Health Organization Histological Classifications of Thymoma

et al. 2019

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Objectives: Computed tomography (CT) is an important technique for evaluating the condition and prognosis of patients with thymomas, and it provides guidance regarding treatment strategies. However, the correlation between CT imaging features, described using standard report terms, and clinical characteristics, Masaoka–Koga stages, and World Health Organization (WHO) classifications of patients with thymomas has not been described in detail nor has risk factor analysis been conducted.Methods: Overall, 159 patients with thymomas who underwent preoperative contrast-enhanced CT between September 2011 and December 2018 were retrospectively reviewed. We assessed the clinical information, CT imaging features, and pathological findings for each patient. A total of 89 patients were specially used to evaluate postoperative recurrence or metastasis between September 2011 and December 2015 to obtain an appropriate observation period. The relationship between CT imaging features and clinical characteristics, Masaoka–Koga stage, and WHO histological classification were analyzed, and related risk factors based on CT imaging features were identified.Results: CT imaging features did not significantly differ based on sex or age. Some imaging features demonstrated significant differences between the groups with and without related clinical characteristics. Contour (odds ratio [OR] = 3.711, P = 0.005), abutment ≥50% (OR = 4.277, P = 0.02), and adjacent lung abnormalities (OR = 3.916 P = 0.031) were independent risk factors for relapse or metastasis. Among all imaging features, there were significant differences between stage I/II and III/IV lesions in tumor size, calcification, infiltration of surrounding fat, vascular invasion, pleural nodules, elevated hemidiaphragm, and pulmonary nodules. Tumor size (odds ratio = 1.261, P = 0.014), vascular invasion (OR = 2.526, P = 0.023), pleural nodules (OR = 2.22, P = 0.048), and pulmonary nodules (OR = 3.106, P = 0.006) were identified as independent risk factors. Tumor size, contour, internal density, infiltration of surrounding fat, and pleural effusion significantly differed between low- and high-risk thymomas. Tumor size (OR = 1.183, P = 0.048), contour (OR = 2.288, P = 0.003), internal density (OR = 2.192, P = 0.024), and infiltration of surrounding fat (OR = 2.811 P = 0.005) were independent risk factors.Conclusions: Some CT imaging features demonstrated significant correlations with clinical characteristics, Masaoka–Koga clinical stages, and WHO histological classifications in patients with thymomas. Familiarity with CT features identified as independent risk factors for these related clinical characteristics can facilitate preoperative evaluation and treatment management for the patients with thymoma.

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Section: Introductionmentioning

confidence: 99%

Relationship Between Computed Tomography Imaging Features and Clinical Characteristics, Masaoka–Koga Stages, and World Health Organization Histological Classifications of Thymoma

et al. 2019

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“…Since 1954, GS has been classified as an immune deficiency of adult onset with a CD8:CD4 imbalance, low CD4 count, low to absent B cells, and association with thymoma; however, no diagnostic criteria have been established. 4 The etiology of this immune dysfunction remains elusive. GS can be distinguished from other common immunodeficiencies such as autoimmune enteropathy by the presence of both hypogammaglobulinemia and low B-cell populations.…”

Section: Discussionmentioning

confidence: 99%

Improvement of Good’s syndrome by fecal microbiota transplantation: the first case report

et al. 2019

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Good’s syndrome (GS) is a rare primary immunodeficiency defined as hypogammaglobulinemia associated with the presence of a thymoma. Patients with GS usually have increased susceptibility to a wide range of infections, and clinical treatment is a challenge for physicians. Fecal microbiota transplantation (FMT), which is a safe strategy for reconstruction of the gut microbiota, has a positive influence on the treatment of refractory infections such as those in patients with GS. We herein report a case involving a 73-year-old woman who had been previously diagnosed with a thymoma. After thymectomy, she complained of respiratory and gastrointestinal symptoms. Her laboratory analysis strongly suggested GS. Infusion of immunoglobulin and albumin was the only treatment of choice until FMT was considered as an alternative therapy. The patient’s manifestations were subsequently relieved, and several FMTs were required to maintain clinical remission. Management of GS remains quite challenging to physicians because of the intricate organ involvement and limited and costly existing therapies. FMT is usually well tolerated by patients, and its cost-effectiveness and safety profile allow it to be considered as an alternative therapy for GS.

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“…Good’s syndrome has since been classified as an adult-onset immune deficiency with low to absent B-cells, derangement in cell-mediated immunity (CD8:CD4 imbalance, low CD4 count), and thymoma, without formal diagnostic criteria. 3 The etiology of this immune dysfunction remains elusive. Good’s syndrome is distinct from common variable immunodeficiency in the presence of both hypogammaglobulinemia and reduced B-cell populations.…”

Section: Discussionmentioning

confidence: 99%

“…Thymectomy is recommended to prevent other immunological manifestations of thymoma including myasthenia gravis, pure red cell aplasia, and pernicious anemia. 3 , 10 However, several reports suggest that hypogammaglobulinemia persists following thymectomy, and patients with Good’s syndrome remain at risk for OI. 12 , 13 …”

Section: Discussionmentioning

confidence: 99%

A Good Case of Recurrent Pneumonia

Bhargava

Eisenstadt

Shih

et al. 2018

Journal of Investigative Medicine High Impact Case Reports

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Bordetella bronchiseptica infection is a common cause of pneumonia in animals but rarely causes disease in humans. Additionally, coinfection with Pneumocystis jirovecii is very uncommon and is occasionally seen in patients with acquired immunodeficiency syndrome (AIDS). We report a case of a 61-year-old HIV-negative man, who presented with hypoxic respiratory failure 2 days after completion of systemic intravenous antibiotic treatment for B bronchiseptica. His past medical history was significant for a benign thymoma. The patient was found to be coinfected with B bronchiseptica and P jirovecii. Laboratory results showed panhypogammaglobulinemia and low absolute B- and CD4 T-cells. Therefore, the patient was diagnosed with Good’s syndrome. However, despite treatment with intravenous antibiotics and intravenous immunoglobulin, the patient continued to deteriorate and expired. This patient demonstrates the importance of recognizing this rare immunodeficiency early in order to improve morbidity and mortality. Furthermore, this case highlights the importance of early immunoglobulin screening in the presence of asymptomatic thymoma.

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Thymoma-associated immunodeficiency: a diagnostic challenge for the clinician

Cited by 8 publications

References 6 publications

Relationship Between Computed Tomography Imaging Features and Clinical Characteristics, Masaoka–Koga Stages, and World Health Organization Histological Classifications of Thymoma

Relationship Between Computed Tomography Imaging Features and Clinical Characteristics, Masaoka–Koga Stages, and World Health Organization Histological Classifications of Thymoma

Improvement of Good’s syndrome by fecal microbiota transplantation: the first case report

A Good Case of Recurrent Pneumonia

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