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Systemic lupus erythematosus complicated with thymoma and pure red cell aplasia (PCRA). CR of both complications following thymectomy and allogeneic haematopoietic SCT (HSCT), but persistence of antinuclear antibodies (ANA)

Bone Marrow Transplantation volume 49, pages 982–983 (2014)Cite this article

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The association of systemic lupus erythematosus (SLE) with thymoma occurs with significant frequency.1 An autoimmune disorder of the blood, pure red cell aplasia (PRCA), may also be associated with thymoma2 and SLE.3 However, the occurrence of all three disorders, SLE, PRCA and thymoma, in the same patient is extremely rare. We are aware of one single case in which PRCA following thymectomy supervened in a patient with SLE.4 Here we report the case of a patient who suffered from all three disorders, was thymectomised with a short-lived remission of PRCA and finally attained full remission of the same conditions following allogeneic haematopoietic SCT (allo-HSCT). However antinuclear antibodies (ANA) of the homogeneous subtype are still present at a significant titre over 1 year after transplant.

Our female patient, now aged 55 years, started suffering from polyarthralgia at the age of 45. ANA with the titre of 1:320 (type unspecified) were present, as well as a high ESR and reduced C3/C4. Further laboratory examinations showed strong positivity of ANA of the homogeneous subtype, anti-ds-DNA antibodies and lupus anticoagulant (LAC). A subsequent severe reticulocytopenic anaemia, with BM aspirates and biopsy sections consistent with PRCA, had a good response to antilymphocytic serum (ATG). However, she relapsed, and was subsequently treated with regular blood transfusions. Three years later a diagnosis of thymoma was made. Thymectomy was performed on 29 November 2010, and was followed by a favourable response of Hb (13 g/dL). The thymoma was of nodular epithelial nature. Because of the thymic residue, she was irradiated transcutaneously on the mediastinum with a linear accelerator. However, severe anaemia recurred, and she was referred to our Institution. The patient was conditioned with a combination of fludarabine (30 mg/m2 × 4 days) and CY (30 mg/kg × 4 days), followed by TBI of 2 Gy. On 11 January 2013, she received a BM transplant from an HLA-identical sister, consisting of 3.1 × 108/kg nucleated cells. Erythroblastic repopulation of the BM was ascertained on day +20. Because of incomplete donor chimerism on day +64, she received two gradually incremental DLI. In January 2014, her haematological data were good (Hb 127.0 g/L, RBC 4.0 × 1012/L, haematocrit 42.5, reticulocytes 1000 × 100 RBC, WBC 6.24 × 109/L, plts 158 × 109/L). Full donor chimerism is present in peripheral CD3+ lymphocytes (100% donor), whereas in the BM it is 78%. ANA are present at the titre of 1:320, and still belong to the homogeneous subtype. Anti ds-DNA antibodies are absent.

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Acknowledgements

We thank Giorgia Conti for her technical assistance.

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  1. Division of Haematology and Haematopoietic Stem Cell Transplantation, IRCCS Azienda Ospedaliera Universitaria San Martino—IST, Genoa, Italy

    A M Marmont, A Bacigalupo, F Gualandi, S Bregante, M T van Lint & S Geroldi

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  1. A M Marmont
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  2. A Bacigalupo
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  3. F Gualandi
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Correspondence to A M Marmont.

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Marmont, A., Bacigalupo, A., Gualandi, F. et al. Systemic lupus erythematosus complicated with thymoma and pure red cell aplasia (PCRA). CR of both complications following thymectomy and allogeneic haematopoietic SCT (HSCT), but persistence of antinuclear antibodies (ANA). Bone Marrow Transplant 49, 982–983 (2014). https://doi.org/10.1038/bmt.2014.68

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