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Uterine intravascular lymphoma as a cause of fever of unknown origin

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Abstract

Primary intravascular large B cell lymphoma (IVL) remains a diagnostic challenge because of non-specific clinical, laboratory and imaging findings. The aim of the study was to analyse the major characteristics of IVL with uterine involvement. We retrospectively collected features of IVL with uterine involvement that was proven histologically or demonstrated by significant 18FDG uptake on 18FDG-PET/CT. Findings were compared to a comprehensive literature review. Five patients were identified. All of them were admitted for fever of unknown origin (FUO), with haemophagocytic lymphohistiocytosis in three cases. None had gynaecological symptom, contrasting with the literature data. Structural imaging (including whole-body CT scan and pelvic RMI) failed to yield any diagnosis. 18FDG-PET/CT showed intense uterine uptake in all cases. Endometrial biopsy was performed in three cases and was positive in one. Diagnosis was obtained from coelioscopic iliac adenopathy biopsy in one case and from total hysterectomy in another. Punch biopsy of skin lesions led to diagnosis in the two remaining cases. Bone marrow biopsy was normal in all cases. Clinicians should be aware of potential isolated uterine involvement in IVL, especially in elderly women with FUO. Normal structural imaging does not rule out the diagnosis and 18FDG-TEP/CT should be performed to guide high-yielding biopsy.

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Author notes

  1. HN and EP equally contributed to this work.

Authors and Affiliations

  1. Clinical Immunology Department, Hôpital Saint Louis, Assistance Publique Hôpitaux de Paris, Université Paris Diderot, 1 Avenue Claude Vellefaux, Paris, France

    Jérôme Hadjadj, Claire Fieschi, Eric Oksenhendler, Lionel Galicier & David Boutboul

  2. Internal Medicine and Rheumatology Department, Percy Military Teaching Hospital, 101 Avenue Henri Barbusse, Clamart, France

    Hubert Nielly & Marc Aletti

  3. Nuclear Medicine Department, Hôpital Bichat-Claude Bernard, Université Paris Diderot, 46 Rue Henri Huchard, Paris, France

    Eve Piekarski & Khadija Benali

  4. Department of Cytogenetics, Hematology Laboratory, Hôpital Saint Louis, Université Paris Diderot, 1 Avenue Claude Vellefaux, Paris, France

    Wendy Cuccuini

  5. Hematology Department, Hôpital Cochin, Université Paris Descartes, 27 Rue du Faubourg Saint-Jacques, Paris, France

    Bénedicte Deau-Fischer

  6. Pathology Department, Hôpital Bichat-Claude Bernard, Université Paris Diderot, 46 Rue Henri Huchard, Paris, France

    Muriel Hourseau

  7. Internal Medicine Department, Hôpital Bichat-Claude Bernard, Université Paris Diderot, 46 Rue Henri Huchard, Paris, France

    Thomas Papo

Authors
  1. Jérôme Hadjadj
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  2. Hubert Nielly
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  3. Eve Piekarski
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  4. Wendy Cuccuini
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  5. Bénedicte Deau-Fischer
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  6. Muriel Hourseau
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  7. Khadija Benali
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  8. Claire Fieschi
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  9. Marc Aletti
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  10. Thomas Papo
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  11. Eric Oksenhendler
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  12. Lionel Galicier
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  13. David Boutboul
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Corresponding author

Correspondence to David Boutboul.

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This manuscript is original research and is not under consideration elsewhere. Informed consent was obtained from all individual participants included in the study. All of the authors contribute to the preparation of the manuscript.

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The authors declare that they have no conflict of interest.

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The opinions or assertions expressed here are the private views of the authors and are not to be considered as official or as reflecting the views of the French Military Health Service.

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Hadjadj, J., Nielly, H., Piekarski, E. et al. Uterine intravascular lymphoma as a cause of fever of unknown origin. Ann Hematol 96, 1891–1896 (2017). https://doi.org/10.1007/s00277-017-3117-4

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  • DOI: https://doi.org/10.1007/s00277-017-3117-4

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