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Journal of Thoracic Oncology:
doi: 10.1097/01.JTO.0000263717.92191.f7
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Thymoma with Paraneoplastic Syndromes, Good's Syndrome, and Pure Red Cell Aplasia

van der Marel, Jacolien MD; Pahlplatz, Paul V.M. MD; Steup, Willem-Hans MD, PhD; Hendriks, Eduard R. MD

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HagaHospital, Department of Surgery, The Hague, The Netherlands.

Disclosure: The authors declare no conflict of interest.

Address for correspondence: Eduard R. Hendriks, MD, HagaHospital, Department of Surgery, Leyweg 275, 2545CH The Hague, The Netherlands. E-mail: e.hendriks@hagaziekenhuis.nl.

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Case Report

A 56-year-old man was examined for a 1.5-year history of recurrent airway infections and sinusitis. He had also been diagnosed with pure red cell aplasia (PRCA), hemoglobin concentration 4.7 mmol/l, for which he was treated with corticosteroids (prednisone 1 mg/kg/day) for 1 year and blood transfusions.1 This treatment stabilized his hemoglobin concentration at 9.5 mmol/l. The recurrent airway infections were first thought to be treatment-related complications by corticosteroids and were treated with antibiotics. Sputum cultures grew Haemophilus influenzae. A computed tomographic scan to exclude bronchiectasis revealed a non-invasive thymoma measuring 11 × 5 cm (Figure 1).

Figure 1
Figure 1
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Because the sinopulmonary infections did not diminish, additional tests were performed to exclude an immunodeficiency that can occur in the presence of a thymoma. The immunoglobulin levels were very low (IgG 3.2 g/l; IgA and IgM non-detectable), and the patient was diagnosed with Good's syndrome.2 Treatment with intravenous gamma globulin was started every 6 weeks, which increased the IgG level to 10.2 g/l and led to less frequent pulmonary infections. The noninvasive and encapsulated thymoma was radically resected by median sternotomy3 (Figure 2). Postoperative recovery was uneventful. Histology confirmed the diagnosis of thymoma, type A/B. As expected, the hypogammaglobulinaemia did not diminish. Therefore, intravenous gamma globulin therapy was continued every 6 weeks. The hematopoiesis, however, completely recovered after thymectomy.

Figure 2
Figure 2
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Thymomas are associated with different paraneoplastic syndromes; myasthenia gravis, Lambert-Eaton myasthenic syndrome, pemphigus, subacute sensory neuronopathy, pure red cell aplasia, and immunodeficiency. Most of these are autoimmune or endocrine-related. The most clinically important are myasthenia gravis, PRCA, and hypogammaglobulinaemia. In the presence of a thymoma, PRCA occurs in 5% to 15% and hypogammaglobulinaemia in less than 5% of the cases. Treatment of PRCA consists of thymectomy. However, this may result in normalization of the bone marrow in only 30% to 40%. A recent case series of 12 patients with thymoma and PRCA reported no remission of anemia after thymectomy.4 In contrast to other paraneoplastic disorders, hypogammaglobulinaemia is not influenced by thymectomy.

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REFERENCES

1. Murakawa T, Nakajima J, Sato H, et al. Thymoma associated with pure red-cell aplasia: clinical features and prognosis. Asian Cardiovasc Thorac Ann 2002;10:150–154.

2. Miyakis S, Pefanis A, Passam FH, et al. Thymoma with immunodeficiency (Good's syndrome): review of the literature apropos three cases. Scand J Infect Dis 2006;38:314–319.

3. Wright CD, Kessler KA. Surgical treatment of thymic tumors. Semin Thorac Cardiovasc Surg 2005;17:20–26.

4. Thompson CA, Steensma DP. Pure red cell aplasia associated with thymoma: clinical insights from a 50-year single-institution experience. Br J Haematol 2006;135:405–407.

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© 2007International Association for the Study of Lung Cancer

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