Lymphomatoid Granulomatosis as a Debut of Common Variable Immunodeficiency: A Case Report and Review of Literature
Affiliation(s)
1 Department of Internal Medicine, Hospital Marqués de Valdecilla-IDIVAL, University of Cantabria, RETICEF, Santander, Spain. 2 Department of Intensive Care, Hospital Marqués de Valdecilla, Santander, Spain. 3 Department of Anatomic Pathology, Hospital Marqués de Valdecilla, Santander, Spain.
1 Department of Internal Medicine, Hospital Marqués de Valdecilla-IDIVAL, University of Cantabria, RETICEF, Santander, Spain. 2 Department of Intensive Care, Hospital Marqués de Valdecilla, Santander, Spain. 3 Department of Anatomic Pathology, Hospital Marqués de Valdecilla, Santander, Spain.
ABSTRACT
Lymphomatoid granulomatosis, currently called as extranodal angiocentric and angiodestructive immunoproliferative disorder, is a rare entity of unclear etiology. It involves most frequently lungs, central nervous system and skin. The clinical course is variable, but mortality is high. Today, it represents a diagnostic challenge because it can emulate autoimmunity, infection, and malignancy processes. Optimal therapy scheme is still unknown. We report the case of a 20 year-old man presenting with fever, weight loss, sweating, multiple bilateral lung nodules on the chest X-ray and cutaneous involvement.
Lymphomatoid granulomatosis, currently called as extranodal angiocentric and angiodestructive immunoproliferative disorder, is a rare entity of unclear etiology. It involves most frequently lungs, central nervous system and skin. The clinical course is variable, but mortality is high. Today, it represents a diagnostic challenge because it can emulate autoimmunity, infection, and malignancy processes. Optimal therapy scheme is still unknown. We report the case of a 20 year-old man presenting with fever, weight loss, sweating, multiple bilateral lung nodules on the chest X-ray and cutaneous involvement.
Cite this paper
Fernández-García, M. , Zhuravetska, T. , Rodríguez, J. and Ots, E. (2014) Lymphomatoid Granulomatosis as a Debut of Common Variable Immunodeficiency: A Case Report and Review of Literature. Journal of Biosciences and Medicines, 2, 13-15. doi: 10.4236/jbm.2014.28002.
Fernández-García, M. , Zhuravetska, T. , Rodríguez, J. and Ots, E. (2014) Lymphomatoid Granulomatosis as a Debut of Common Variable Immunodeficiency: A Case Report and Review of Literature. Journal of Biosciences and Medicines, 2, 13-15. doi: 10.4236/jbm.2014.28002.
References
[1] Swerdlow, S., Campo, E., Lee Harris, N., Jaffe, E., Pileri, S., Stein, H., et al. (2008) WHO Classification of Tumors of Haematopoietic and Lymphoid Tissues. IARC Press, Lyon. [2] Inaba, M., Ushijim, S., Hirata, N., Saisyoji, T., Kitaoka, M. and Yoshinaga, T. (2011) Methotrexate-Related Lymphomatoid Granulomatosis in a Patient with Rheumatoid Arthritis. Nihon Kokyuki Gakkai Zasshi, 49, 597-601. [3] Wyen, C., Stenzel, W., Hoffmann, C., Lehmann, C., Deckert, M. and Fatkenheuer, G. (2007) Fatal Cerebral Lymphomatoid Granulomatosis in an HIV-1-Infected Patient. Journal of Infection, 54, 175-178.
http://dx.doi.org/10.1016/j.jinf.2006.11.003 [4] Cunningham-Rundles, C. and Bodian, C. (1999) Common Variable Immunodeficiency: Clinical and Immunological Features of 248 Patients. Clinical Immunology, 92, 34-48.
http://dx.doi.org/10.1006/clim.1999.4725 [5] Váróczy, L., Gergely, L., Szakáll, S. and Illés, A. (2002) Angiocentric lymphomatoid Granulomatosis and Severe Hypogammaglobulinaemia. Haematologia, 32, 535-541.
[1] Swerdlow, S., Campo, E., Lee Harris, N., Jaffe, E., Pileri, S., Stein, H., et al. (2008) WHO Classification of Tumors of Haematopoietic and Lymphoid Tissues. IARC Press, Lyon. [2] Inaba, M., Ushijim, S., Hirata, N., Saisyoji, T., Kitaoka, M. and Yoshinaga, T. (2011) Methotrexate-Related Lymphomatoid Granulomatosis in a Patient with Rheumatoid Arthritis. Nihon Kokyuki Gakkai Zasshi, 49, 597-601. [3] Wyen, C., Stenzel, W., Hoffmann, C., Lehmann, C., Deckert, M. and Fatkenheuer, G. (2007) Fatal Cerebral Lymphomatoid Granulomatosis in an HIV-1-Infected Patient. Journal of Infection, 54, 175-178.
http://dx.doi.org/10.1016/j.jinf.2006.11.003 [4] Cunningham-Rundles, C. and Bodian, C. (1999) Common Variable Immunodeficiency: Clinical and Immunological Features of 248 Patients. Clinical Immunology, 92, 34-48.
http://dx.doi.org/10.1006/clim.1999.4725 [5] Váróczy, L., Gergely, L., Szakáll, S. and Illés, A. (2002) Angiocentric lymphomatoid Granulomatosis and Severe Hypogammaglobulinaemia. Haematologia, 32, 535-541.