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Published in: Journal of Clinical Immunology 3/2014

01-04-2014 | Astute Clinician Report

Good’s Syndrome and Pure White Cell Aplasia Complicated by Cryptococcus Infection: A Case Report and Review of the Literature

Authors: K. Akinosoglou, M. Melachrinou, D. Siagris, E. Koletsis, M. Marangos, C. A. Gogos, E. E. Solomou

Published in: Journal of Clinical Immunology | Issue 3/2014

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Abstract

Thymomas can present with a variety of paraneoplastic manifestations, mostly of autoimmune origin, including Good’s syndrome when there is associated hypogammaglobulinemia. Although pure red cell aplasia is a recognised complication of thymoma, selective white cell aplasia is very rare, particularly in Good’s syndrome. Lethal opportunistic infections are a feature of Good’s syndrome, usually occurring in those patients with associated severe T lymphocyte defects. Although the cryptococcus is a recognised fungal pathogen in patients with other causes of CD4+ T cell lymphopenia, surprisingly this complication has not been reported in patients with Good’s syndrome. We now describe a 70 year old man with Good’s syndrome and pure white cell aplasia who presented with disseminated cryptococcosis, and provide an up-to-date review of the relevant literature. Despite meningeal involvement our patient recovered after combined treatment with intravenous globulin, granulocyte stimulating growth, corticosteroids and antifungal therapy.
Literature
1.
go back to reference 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 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
3.
go back to reference Degos L, Faille A, Housset M, Boumsell L, Rabian C, Parames T. Syndrome of neutrophil agranulocytosis, hypogammaglobulinemia, and thymoma. Blood. 1982;60(4):968–72.PubMed Degos L, Faille A, Housset M, Boumsell L, Rabian C, Parames T. Syndrome of neutrophil agranulocytosis, hypogammaglobulinemia, and thymoma. Blood. 1982;60(4):968–72.PubMed
4.
go back to reference Federico P, Imbimbo M, Buonerba C, Damiano V, Marciano R, Serpico D, et al. Is hypogammaglobulinemia a constant feature in Good’s syndrome? Int J Immunopathol Pharmacol. 2010;23(4):1275–9.PubMed Federico P, Imbimbo M, Buonerba C, Damiano V, Marciano R, Serpico D, et al. Is hypogammaglobulinemia a constant feature in Good’s syndrome? Int J Immunopathol Pharmacol. 2010;23(4):1275–9.PubMed
7.
go back to reference Jeandel C, Gastin I, Blain H, Jouanny P, Laurain MC, Penin F, et al. Thymoma with immunodeficiency (Good’s syndrome) associated with selective cobalamin malabsorption and benign IgM-kappa gammopathy. J Intern Med. 1994;235(2):179–82.PubMedCrossRef Jeandel C, Gastin I, Blain H, Jouanny P, Laurain MC, Penin F, et al. Thymoma with immunodeficiency (Good’s syndrome) associated with selective cobalamin malabsorption and benign IgM-kappa gammopathy. J Intern Med. 1994;235(2):179–82.PubMedCrossRef
9.
go back to reference Rogers BH, Manligod JR, Blazek WV. Thymoma associated with pancytopenia and hypogammaglobulinemia. Report of a case and review of the literature. Am J Med. 1968;44(1):154–64.PubMedCrossRef Rogers BH, Manligod JR, Blazek WV. Thymoma associated with pancytopenia and hypogammaglobulinemia. Report of a case and review of the literature. Am J Med. 1968;44(1):154–64.PubMedCrossRef
10.
go back to reference 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 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
12.
go back to reference Postiglione K, Ferris R, Jaffe JP, Stroncek D. Immune mediated agranulocytosis and anemia associated with thymoma. Am J Hematol. 1995;49(4):336–40.PubMedCrossRef Postiglione K, Ferris R, Jaffe JP, Stroncek D. Immune mediated agranulocytosis and anemia associated with thymoma. Am J Hematol. 1995;49(4):336–40.PubMedCrossRef
13.
go back to reference Thiele HG, Frenzel H. Immunoglobulin deficiency syndrome and agranulocytosis in thymoma [in German]. Schweiz Med Wochenschr. 1967;97:1606–11.PubMed Thiele HG, Frenzel H. Immunoglobulin deficiency syndrome and agranulocytosis in thymoma [in German]. Schweiz Med Wochenschr. 1967;97:1606–11.PubMed
14.
go back to reference Jacobson BM, Castleman B. Anaemia, granulocytopenia, and terminal sepsis in a 70-year-old woman. N Engl J Med. 1971;284:39–47.CrossRef Jacobson BM, Castleman B. Anaemia, granulocytopenia, and terminal sepsis in a 70-year-old woman. N Engl J Med. 1971;284:39–47.CrossRef
15.
go back to reference Young RH. Thymoma with agranulocytosis and hypogammaglobulinaemia. Ir J Med Sci. 1977;146(8):266–8.PubMedCrossRef Young RH. Thymoma with agranulocytosis and hypogammaglobulinaemia. Ir J Med Sci. 1977;146(8):266–8.PubMedCrossRef
16.
go back to reference Sugiura Y, Itoh K, Hakumei O, Asai T, Fujioka S, Kumagaya A. A case of pure red cell aplasia associated with thymoma, granulocytopenia, hypogammaglobulinemia, and hypothyroidism. Rinsho Ketsueki. 1982;23(5):700–7.PubMed Sugiura Y, Itoh K, Hakumei O, Asai T, Fujioka S, Kumagaya A. A case of pure red cell aplasia associated with thymoma, granulocytopenia, hypogammaglobulinemia, and hypothyroidism. Rinsho Ketsueki. 1982;23(5):700–7.PubMed
17.
go back to reference Ackland SP, Bur ME, Adler SS, Robertson M, Baron JM. White blood cell aplasia associated with thymoma. Am J Clin Pathol. 1988;89(2):260–3.PubMed Ackland SP, Bur ME, Adler SS, Robertson M, Baron JM. White blood cell aplasia associated with thymoma. Am J Clin Pathol. 1988;89(2):260–3.PubMed
18.
go back to reference Weir 3rd AB, Dow LW. Response of agranulocytosis to thymectomy in a patient with thymoma and chronic lymphocytic leukemia. Med Pediatr Oncol. 1989;17(1):58–61.PubMedCrossRef Weir 3rd AB, Dow LW. Response of agranulocytosis to thymectomy in a patient with thymoma and chronic lymphocytic leukemia. Med Pediatr Oncol. 1989;17(1):58–61.PubMedCrossRef
19.
go back to reference El-Galaly TC, Gregersen H, Bukh A. Good’s syndrome with lymphopenia and neutropenia. Ugeskr Laeger. 2011;173(4):280–1.PubMed El-Galaly TC, Gregersen H, Bukh A. Good’s syndrome with lymphopenia and neutropenia. Ugeskr Laeger. 2011;173(4):280–1.PubMed
20.
go back to reference Joven MH, Palalay MP, Sonido CY. Case report and literature review on Good’s syndrome, a form of acquired immunodeficiency associated with thymomas. Hawaii J Med Public Health. 2013;72(2):56–62.PubMedCentralPubMed Joven MH, Palalay MP, Sonido CY. Case report and literature review on Good’s syndrome, a form of acquired immunodeficiency associated with thymomas. Hawaii J Med Public Health. 2013;72(2):56–62.PubMedCentralPubMed
21.
go back to reference Tumours of the thymus in Travis W, Brambilla E, Muller-Hermelink HK, Harris CC. Pathology genetics: Tumours of the lung, pleura, thymus and heart. World Health Organization Classification of tumours. Lyon: IARC Pres; 2004. Tumours of the thymus in Travis W, Brambilla E, Muller-Hermelink HK, Harris CC. Pathology genetics: Tumours of the lung, pleura, thymus and heart. World Health Organization Classification of tumours. Lyon: IARC Pres; 2004.
22.
go back to reference Courville P, Favennec L, Viacroze C, Barrel A, Young P, Abboud P, et al. Co-existent cutaneous cryptococcosis of the forearm and cutaneous alternariosis of the leg in patient with metastatic thymoma. J Cutan Pathol. 2002;29(1):55–8.PubMedCrossRef Courville P, Favennec L, Viacroze C, Barrel A, Young P, Abboud P, et al. Co-existent cutaneous cryptococcosis of the forearm and cutaneous alternariosis of the leg in patient with metastatic thymoma. J Cutan Pathol. 2002;29(1):55–8.PubMedCrossRef
23.
go back to reference Kishi K, Homma S, Nakatani T, Nakata K. A clinical study of cryptococcal meningitis—sequential changes of cryptococcal antigen titers. Kansenshogaku Zasshi. 2003;77(3):150–7.PubMed Kishi K, Homma S, Nakatani T, Nakata K. A clinical study of cryptococcal meningitis—sequential changes of cryptococcal antigen titers. Kansenshogaku Zasshi. 2003;77(3):150–7.PubMed
25.
go back to reference Schmidt S, Padberg F. Late onset immunodeficiency in a patient with recurrent thymic carcinoma and myasthenia gravis. J Neurol Sci. 1998;157(2):201–5.PubMedCrossRef Schmidt S, Padberg F. Late onset immunodeficiency in a patient with recurrent thymic carcinoma and myasthenia gravis. J Neurol Sci. 1998;157(2):201–5.PubMedCrossRef
28.
go back to reference Bodem CR, Hamory BH, Taylor HM, Kleopfer L. Granulomatous bone marrow disease. A review of the literature and clinicopathologic analysis of 58 cases. Medicine (Baltimore). 1983;62(6):372–83.CrossRef Bodem CR, Hamory BH, Taylor HM, Kleopfer L. Granulomatous bone marrow disease. A review of the literature and clinicopathologic analysis of 58 cases. Medicine (Baltimore). 1983;62(6):372–83.CrossRef
30.
go back to reference Rosai J. In: Ackerman’s Surgical Pathology, editor. Bone marrow. Singapore: Harcourt Brace & Co Asia Pte Ltd; 1996. p. 1806–14. Rosai J. In: Ackerman’s Surgical Pathology, editor. Bone marrow. Singapore: Harcourt Brace & Co Asia Pte Ltd; 1996. p. 1806–14.
31.
32.
go back to reference Feldmesser M, Harris C, Reichberg S, Khan S, Casadevall A. Serum cryptococcal antigen in patients with AIDS. Clin Infect Dis. 1996;23(4):827–30.PubMedCrossRef Feldmesser M, Harris C, Reichberg S, Khan S, Casadevall A. Serum cryptococcal antigen in patients with AIDS. Clin Infect Dis. 1996;23(4):827–30.PubMedCrossRef
Metadata
Title
Good’s Syndrome and Pure White Cell Aplasia Complicated by Cryptococcus Infection: A Case Report and Review of the Literature
Authors
K. Akinosoglou
M. Melachrinou
D. Siagris
E. Koletsis
M. Marangos
C. A. Gogos
E. E. Solomou
Publication date
01-04-2014
Publisher
Springer US
Published in
Journal of Clinical Immunology / Issue 3/2014
Print ISSN: 0271-9142
Electronic ISSN: 1573-2592
DOI
https://doi.org/10.1007/s10875-014-0014-7

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