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CASE REPORT
Unexplained lymphadenopathies: autoimmune lymphoproliferative syndrome in an adult patient
  1. Fatima Leal-Seabra1,
  2. Gonçalo Sarmento Costa1,
  3. Henrique Pereira Coelho2,
  4. Agripino Oliveira1
  1. 1Department of Internal Medicine, Centro Hospitalar Vila Nova de Gaia/Espinho, Vila Nova Gaia, Porto, Portugal
  2. 2Hematology Service, Centro Hospitalar Vila Nova de Gaia/Espinho, Vila Nova Gaia, Porto, Portugal
  1. Correspondence to Dr Agripino Oliveira, agripino{at}netcabo.pt

Summary

Autoimmune lymphoproliferative syndrome (ALPS) is characterised by massive enlargement of the lymphoid organs, autoimmune cytopenias and a predisposition to develop lymphoid malignancies. The basic defect is a disturbance of the lymphocyte apoptosis, and a high number of circulating TCRab CD3+CD4−CD8− T-cells (double-negative T cells (DNT cells)). We describe a case of a 41-year-old man with fever, hepatosplenomegaly, multiple lymphadenopathy, autoimmune haemolytic anaemia and severe thrombocytopenia. Peripheral blood immunophenotyping revealed elevation of the characteristic DNT cells in 8% and high levels of interleukin 10. Histopathological analysis of lymph nodes showed lymphadenitis with paracortical hyperplasia. It was assumed as a probable diagnosis of ALPS, and the procedure was to medicate the patient with steroids. As a result, a significant clinical improvement was achieved, and he has been in remission for 2 years. To our knowledge, this is the first case reported in a Portuguese adult patient.

This is an Open Access article distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/

https://doi.org/10.1136/bcr-2016-216758

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    Footnotes

    • Contributors All authors had been involved in this patient's care. FLS and GSC contributed to the drafting of the manuscript. HPC contributed to the interpretation of data. AO revised the content and accepts responsibility for the overall content as a guarantor.

    • Competing interests None declared.

    • Patient consent Obtained.

    • Provenance and peer review Not commissioned; externally peer reviewed.