nach oben

Erschienen in:

01.06.2012

Immune-Mediated Diseases and Immunodeficiencies Associated with Thymic Epithelial Neoplasms

verfasst von: Muhammad Rizwan Khawaja, Robert P. Nelson Jr., Nicholas Miller, Sunil S. Badve, Elizabeth Loehrer, Magdalena Czader, Susan M. Perkins, Kenneth Kesler, Patrick J. Loehrer Sr.

Erschienen in: Journal of Clinical Immunology | Ausgabe 3/2012

Einloggen, um Zugang zu erhalten

Abstract

Introduction

This retrospective study aimed to characterize the clinical hematological and immunological features of patients with thymic epithelial neoplasms.

Methods

From a cohort of 512 patients with thymic epithelial neoplasms, 79 patients diagnosed with autoimmune/immunodeficiency conditions or signs and/or symptoms suggesting an autoimmune or immunodeficiency state were evaluated by standard immunological and hematological testing.

Results

Elevated percentages of CD2+, CD3+, and CD8+ lymphocytes were observed in 44 (57.1%), 33 (41.8%), and 32 (40.5%) patients. Low CD4+ and CD19+ percentages were observed in 25 (31.6%) and 36 (46.2%), respectively; CD4+:CD8+ ratios were inverted in 18 (22.8%). IgG, IgA, and IgM levels were low in 12 (15.8%), 9 (11.7%) and 15 (19.7%) patients, respectively. Patients with immunodeficiency condition(s) were more likely to have high CD8+ percentages (p = 0.040), low CD19+ percentages (p = 0.025), and/or inverted CD4+/CD8+ ratios (p = 0.034). Patients with autoimmune condition(s) were more likely to have a high/normal CD4+ percentage (p = 0.038). High CD2+ percentages were associated with lower mean IgG and IgA levels (p = 0.030 and p = 0.017, respectively). High CD3+ and CD8+ percentages were associated with lower mean IgA levels (p = 0.046 and p = 0.013, respectively). Low CD19+ percentages were associated with lower mean IgG and IgA levels (p = 0.004 and p < 0.001, respectively).

Conclusion

Signs/symptoms and history of autoimmune and immunodeficiency conditions among patients with thymic epithelial neoplasms are associated with high frequencies of abnormalities in immunoglobulin levels and lymphocyte immunophenotypes, suggesting a role for their assessment.

Suster S, Moran CA. Thymoma, atypical thymoma, and thymic carcinoma. A novel conceptual approach to the classification of thymic epithelial neoplasms. Am J Clin Pathol. 1999;111:826–33.PubMed

Travis WD, Brambilla E, Müller-Hermelink HK, Harris CC. Pathology and genetics of tumors of the lung, pleura, thymus and heart. Lyon: IARC; 2004.

Suster S, Moran CA. Histologic classification of thymoma: the World Health Organization and beyond. Hematol Oncol Clin N Am. 2008;22(3):381–92.CrossRef

Masci AM, Palmieri G, Perna F, Montella L, Merkabaoui G, Sacerdoti G, et al. Immunological findings in thymoma and thymoma-related syndromes. Ann Med. 1999;31 Suppl 2:86–9.PubMed

Barnes EW, Irvine WJ. Clinical syndromes associated with thymic disorders. Proc R Soc Med. 1973;66:151–4.PubMed

Souadjian JV, Enriquez P, Silverstein MN, Pépin JM. The spectrum of diseases associated with thymoma. Coincidence or syndrome. Arch Intern Med. 1974;134:374–9.PubMedCrossRef

Marx A, Schultz A, Wilisch A, Helmreich M, Nenninger R, Müller-Hermelink HK. Paraneoplastic autoimmunity in thymus tumors. Dev Immunol. 1998;6:129–40.PubMedCrossRef

Evoli A, Minicuci GM, Vitaliani R, Battaglia A, Della Marca G, Lauriola L, et al. Paraneoplastic diseases associated with thymoma. J Neurol. 2007;254:756–62.PubMedCrossRef

Nelson Jr RP, Pascuzzi RM. Paraneoplastic syndromes in thymoma: an immunological perspective. Curr Treat Options Oncol. 2008;9:269–76.PubMedCrossRef

10.

Tormoehlen LM, Pascuzzi RM. Thymoma, myasthenia gravis, and other paraneoplastic syndromes. Hematol Oncol Clin N Am. 2008;8:509–26.CrossRef

11.

Rosenow EC, Hurley BT. Disorders of the thymus. A review. Arch Intern Med. 1984;144:763–70.PubMedCrossRef

12.

Kelesidis T, Yang O. Good's syndrome remains a mystery after 55 years: a systematic review of the scientific evidence. Clin Immunol. 2010;135:347–63.PubMedCrossRef

13.

Castro KG, Ward JW, Slutsker L, Buehler JW, Jaffe HW, Berkelman RL, et al. 1993 revised classification system for HIV infection and expanded surveillance case definition for AIDS among adolescents and adults. MMWR Recomm Rep. 1992;41(RR-17):1–19.

14.

Jones JL, Hanson DL, Dworkin MS, Alderton DL, Fleming PL, Kaplan JE, et al. Surveillance for AIDS-defining opportunistic illnesses, 1992–1997. MMWR CDC Surveill Summ. 1999;48(2):1–22.PubMed

15.

Baker CJ, Kasper DL, Edwards MS, Schiffman G. Influence of preimmunization antibody levels on the specificity of the immune response to related polysaccharide antigens. N Engl J Med. 1980;303(4):173–8.PubMedCrossRef

16.

Engels EA, Pfeiffer RM. Malignant thymoma in the United States: demographic patterns in incidence and associations with subsequent malignancies. Int J Cancer. 2003;105(4):546–51.PubMedCrossRef

17.

Salyer WR, Eggleston JC. Thymoma: a clinical and pathological study of 65 cases. Cancer. 1976;37(1):229–49.PubMedCrossRef

18.

Rubin M, Straus B, Allen L. Clinical disorders associated with thymic tumors. Arch Intern Med. 1964;114:389–98.PubMedCrossRef

19.

Ammus SS, Yunis AA. Acquired pure red cell aplasia. Am J Hematol. 1987;24(3):311–26.PubMedCrossRef

20.

Yip D, Rasko JE, Lee C, Kronenberg H, O'Neill B. Thymoma and agranulocytosis: two case reports and literature review. Br J Haematol. 1996;95(1):52–6.PubMedCrossRef

21.

Kelleher P, Misbah SA. What is Good's syndrome? Immunological abnormalities in patients with thymoma. J Clin Pathol. 2003;56(1):12–6.PubMedCrossRef

22.

Tarr PE, Sneller MC, Mechanic LJ, Economides A, Eger CM, Strober W, et al. Infections in patients with immunodeficiency with thymoma (Good syndrome). Report of 5 cases and review of the literature. Medicine (Baltimore). 2001;80(2):123–33.CrossRef

23.

Miyakis S, Pefanis A, Passam FH, Christodulakis GR, Roussou PA, Mountokalakis TD. Thymoma with immunodeficiency (Good's syndrome): review of the literature apropos three cases. Scand J Infect Dis. 2006;38(4):314–9.PubMedCrossRef

24.

Di Renzo M, Pasqui A, Bruni F, Voltolini L, Rottoli P, Perali G, et al. Thymoma and immunodeficiency. N Z Med J. 2004;117(1188):U750.PubMed

25.

Vitiello L, Masci AM, Montella L, Perna F, Angelini DF, Borsellino G, et al. Thymoma-associated immunodeficiency: a syndrome characterized by severe alterations in NK, T and B-cells and progressive increase in naive CD8+ T cells. Int J Immunopathol Pharmacol. 2010;23(1):307–16.PubMed

26.

Tarr PE, Lucey DR. Good's syndrome: the association of thymoma with immunodeficiency. Clin Infect Dis. 2001;33(4):585–6.PubMedCrossRef

27.

Del Pozo N, Sarmiento E, Lanio N, Gallego A, Largo J, Carbone J. Immunophenotypic abnormalities of CD8+ T-cell subsets in a patient with unusual Good's syndrome. Allergol Immunopathol (Madr). 2010;38(2):102–5.CrossRef

28.

Baumert E, Schlesier M, Wolff-Vorbeck G, Peter HH. Alterations in lymphocyte subsets in variable immunodeficiency syndrome. Immun Infekt. 1992;20(3):73–5.PubMed

29.

Hanafusa T, Umegaki N, Yamaguchi Y, Katayama I. Good’s syndrome (hypogammaglobulinemia with thymoma) presenting intractable opportunistic infections and hyperkeratotic lichen planus. J Dermatol. 2010;37(2):171–4.PubMedCrossRef

30.

Jaffe JS, Eisenstein E, Sneller MC, Strober W. T-cell abnormalities in common variable immunodeficiency. Pediatr Res. 1993;33 Suppl 1:24–7.CrossRef

31.

Burbelo PD, Browne SK, Sampaio EP, Giaccone G, Zaman R, Kristosturyan E, et al. Anti-cytokine autoantibodies are associated with opportunistic infection in patients with thymic neoplasia. Blood. 2010;116(23):4848–58.PubMedCrossRef

32.

Nagvekar N, Moody AM, Moss P, Roxanis I, Curnow J, Beeson D, et al. A pathogenetic role for the thymoma in myasthenia gravis. Autosensitization of IL-4- producing T cell clones recognizing extracellular acetylcholine receptor epitopes presented by minority class II isotypes. J Clin Invest. 1998;101:2268–77.PubMedCrossRef

33.

Okereke IC, Kesler KA, Morad MH, Mi D, Rieger KM, Birdas TJ, et al. Prognostic indicators after surgery for thymoma. Ann Thorac Surg. 2010;89(4):1071–9.PubMedCrossRef

34.

Mateo-Montoya A, Stanescu D, Wolff B, Sahel JA, Bonnel S. Cytomegalovirus retinitis associated with Good's syndrome. Eur J Ophthalmol. 2010;20(2):479–80.PubMed

35.

Squintani G, Ferrari S, Bazzoli E, Eleopra R, La Monaca C, Cagliari E, et al. Progressive multifocal leukoencephalopathy in a patient with Good's syndrome. Int J Infect Dis. 2010;14(5):e444–7.PubMedCrossRef

36.

Kuribayashi K, Fujimi A, Kobune M, Takimoto R, Kikuchi S, Iyama S, et al. Pure red cell aplasia associated with Good's syndrome accompanied by decreased stem cell factor production in the bone marrow. Intern Med. 2010;49(5):377–82.PubMedCrossRef

37.

Marx A, Hohenberger P, Hoffmann H, Pfannschmidt J, Schnabel P, Hofmann HS, et al. The autoimmune regulator AIRE in thymoma biology: autoimmunity and beyond. J Thorac Oncol. 2010;5(10 Suppl 4):S266–72.PubMedCrossRef

38.

Ströbel P, Helmreich M, Menioudakis G, Lewin SR, Rüdiger T, Bauer A, et al. Paraneoplastic myasthenia gravis correlates with generation of mature naive CD4(+) T cells in thymomas. Blood. 2002;100(1):159–66.PubMedCrossRef

39.

Ströbel P, Bauer A, Puppe B, Kraushaar T, Krein A, Toyka K, et al. Tumor recurrence and survival in patients treated for thymomas and thymic squamous cell carcinomas: a retrospective analysis. J Clin Oncol. 2004;22(8):1501–9.PubMedCrossRef

40.

Ströbel P, Murumägi A, Klein R, Luster M, Lahti M, Krohn K, et al. Deficiency of the autoimmune regulator AIRE in thymomas is insufficient to elicit autoimmune polyendocrinopathy syndrome type 1 (APS-1). J Pathol. 2007;211(5):563–71.PubMedCrossRef

Titel: Immune-Mediated Diseases and Immunodeficiencies Associated with Thymic Epithelial Neoplasms
verfasst von: Muhammad Rizwan Khawaja
Robert P. Nelson Jr.
Nicholas Miller
Sunil S. Badve
Elizabeth Loehrer
Magdalena Czader
Susan M. Perkins
Kenneth Kesler
Patrick J. Loehrer Sr.
Publikationsdatum: 01.06.2012
Verlag: Springer US
Erschienen in: Journal of Clinical Immunology / Ausgabe 3/2012
Print ISSN: 0271-9142
Elektronische ISSN: 1573-2592
DOI: https://doi.org/10.1007/s10875-011-9644-1

Leitlinien kompakt für die Innere Medizin

Mit medbee Pocketcards sicher entscheiden.

^{Seit 2022 gehört die medbee GmbH zum Springer Medizin Verlag}

Kostenlos registrieren

Neu im Fachgebiet Innere Medizin

Bei Herzinsuffizienz muss „Eisenmangel“ neu definiert werden!

16.05.2024 Herzinsuffizienz Nachrichten

Bei chronischer Herzinsuffizienz macht es einem internationalen Expertenteam zufolge wenig Sinn, die Diagnose „Eisenmangel“ am Serumferritin festzumachen. Das Team schlägt vor, sich lieber an die Transferrinsättigung zu halten.

Herzinfarkt mit 85 – trotzdem noch intensive Lipidsenkung?

16.05.2024 Hypercholesterinämie Nachrichten

Profitieren nach einem akuten Myokardinfarkt auch Betroffene über 80 Jahre noch von einer intensiven Lipidsenkung zur Sekundärprävention? Um diese Frage zu beantworten, wurden jetzt Registerdaten aus Frankreich ausgewertet.

ADHS-Medikation erhöht das kardiovaskuläre Risiko

16.05.2024 Herzinsuffizienz Nachrichten

Erwachsene, die Medikamente gegen das Aufmerksamkeitsdefizit-Hyperaktivitätssyndrom einnehmen, laufen offenbar erhöhte Gefahr, an Herzschwäche zu erkranken oder einen Schlaganfall zu erleiden. Es scheint eine Dosis-Wirkungs-Beziehung zu bestehen.

Erstmanifestation eines Diabetes-Typ-1 bei Kindern: Ein Notfall!

16.05.2024 DDG-Jahrestagung 2024 Kongressbericht

Manifestiert sich ein Typ-1-Diabetes bei Kindern, ist das ein Notfall – ebenso wie eine diabetische Ketoazidose. Die Grundsäulen der Therapie bestehen aus Rehydratation, Insulin und Kaliumgabe. Insulin ist das Medikament der Wahl zur Behandlung der Ketoazidose.

Update Innere Medizin

Bestellen Sie unseren Fach-Newsletter und bleiben Sie gut informiert.

Newsletter bestellen

Springer Medizin

Abstract

Introduction

Methods

Results

Conclusion

Bitte loggen Sie sich ein, um Zugang zu diesem Inhalt zu erhalten

Weitere Artikel der Ausgabe 3/2012

Dendritic and T Cell Response to Influenza is Normal in the Patients with X-Linked Agammaglobulinemia

Subcutaneous Immunoglobulin Replacement Therapy with Hizentra® is Safe and Effective in Two Infants

Lessons Learned from Phagocytic Function Studies in a Large Cohort of Patients with Recurrent Infections

B-Cell Tolerance Defects in the B6.Aec1/2 Mouse Model of Sjögren’s Syndrome

Influence of Maternal Hyperglycemia on IL-10 and TNF-α Production: The Relationship with Perinatal Outcomes