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Central nervous system immune reconstitution inflammatory syndrome (IRIS) after hematopoietic SCT

Bone Marrow Transplantation volume 45, pages 593–596 (2010)Cite this article

Immune reconstitution inflammatory syndrome (IRIS) is an increasingly recognized clinical entity emerging in the context of the restoration of the immune system after significant immunosuppression. As the patient's immune system regains its potency, IRIS may lead to a paradoxical clinical deterioration when intense inflammatory infiltrates are formed to eradicate an underlying subclinical infection.1 The vast majority of the patients with IRIS are HIV-infected subjects, whose immune system recovers after the initiation of highly active antiretroviral therapy.2 Infections associated with IRIS include those caused by Mycobacterium tuberculosis, Mycobacterium Avium-Intracellulare(MAI), Cryptococcus neoformans, CMV, JC virus and Candida.1, 3 We report a case of IRIS in a non-HIV-positive patient, who developed a severe form of CNS IRIS after treatment of CML with allogeneic hematopoietic SCT (HSCT).

A 31-year-old previously healthy man was diagnosed with CML at the end of 2002. Allogeneic HSCT with peripheral blood from a matched unrelated donor was performed in May 2003. Standard conditioning with high-dose CY, TBI (12 Gy in five fractions) and antithymocyte globulin was given and immunosuppression thereafter consisted of short MTX and CYA. Grade 2 skin GVHD was treated with methylprednisolone; otherwise, he recovered without complications. Immunosuppression was tapered 1 year post transplant and stopped entirely in August 2004. In September 2004, he had a severe pneumocystis pneumonia. The cell counts of lymphocyte subtypes recovered into the normal ranges by December 2004 (Table 1).

Table 1 Reconstitution of blood lymphocyte counts ( × 106/l) after allogeneic HSCT in May 2003
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Authors and Affiliations

  1. Department of Neurology, Turku University Central Hospital, Turku, Finland

    L Airas & M Päivärinta

  2. Department of Pathology, Turku University Central Hospital, Turku, Finland

    M Röyttä

  3. Department of Radiology, Turku University Central Hospital, Turku, Finland

    J Karhu

  4. Department of Internal Medicine, Turku University Central Hospital, Turku, Finland

    M Kauppila, M Itälä-Remes & K Remes

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Correspondence to L Airas.

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Airas, L., Päivärinta, M., Röyttä, M. et al. Central nervous system immune reconstitution inflammatory syndrome (IRIS) after hematopoietic SCT. Bone Marrow Transplant 45, 593–596 (2010). https://doi.org/10.1038/bmt.2009.186

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