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Sezary Syndrome: A Clinico-biological Study of 5 Cases

Received: 12 March 2022    Accepted: 9 April 2022    Published: 20 April 2022
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Abstract

Background: Sézary syndrome (SS) is a rare erythrodermic and leukemic variant of cutaneous T-cell lymphoma (CTCL) that belongs to the group of non-Hodgkin's lymphomas (NHL) resulting from malignant proliferation of skin-homing T cells. We report through a series of 5 cases, the experience of the hematology laboratory in the diagnosis of the syndrome of Sézary. Methods: This is a retrospective study of 5 cases of Sezary syndrome collected in the dermatology department of Marrakech. Results: five patients were identified with the clinicopathological criteria of SS. At the time of diagnosis, all 5 patients had erythroderma and generalized lymphadenopathy in both superficial and deep stations. The white blood cell count was elevated (>10,000 WBC/mm3) in all 5 patients with a mean value of 18,120 WBC/mm3. The blood smear showed the presence of 75% (27 G/l) of small to medium-sized cells with a high nucleocytoplasmic ratio and cerebriform nuclei typical of Sezary cells and suggests the diagnosis of SS. Conclusions: The diagnosis of SS remains a challenge in many situations, the pathophysiology and definition of SS have evolved significantly over the past decades.

Published in American Journal of Biomedical and Life Sciences (Volume 10, Issue 2)
DOI 10.11648/j.ajbls.20221002.16
Page(s) 42-44
Creative Commons

This is an Open Access article, distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution and reproduction in any medium or format, provided the original work is properly cited.

Copyright

Copyright © The Author(s), 2024. Published by Science Publishing Group

Keywords

Sezary Syndrome, Lymphoma, T-cell, Blood Smear

References
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  • APA Style

    Rania Rada, Hajar Saffour, Mahjouba Baiya, Hicham Yahyaoui, Mustapha Ait Ameur, et al. (2022). Sezary Syndrome: A Clinico-biological Study of 5 Cases. American Journal of Biomedical and Life Sciences, 10(2), 42-44. https://doi.org/10.11648/j.ajbls.20221002.16

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    ACS Style

    Rania Rada; Hajar Saffour; Mahjouba Baiya; Hicham Yahyaoui; Mustapha Ait Ameur, et al. Sezary Syndrome: A Clinico-biological Study of 5 Cases. Am. J. Biomed. Life Sci. 2022, 10(2), 42-44. doi: 10.11648/j.ajbls.20221002.16

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    AMA Style

    Rania Rada, Hajar Saffour, Mahjouba Baiya, Hicham Yahyaoui, Mustapha Ait Ameur, et al. Sezary Syndrome: A Clinico-biological Study of 5 Cases. Am J Biomed Life Sci. 2022;10(2):42-44. doi: 10.11648/j.ajbls.20221002.16

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  • @article{10.11648/j.ajbls.20221002.16,
      author = {Rania Rada and Hajar Saffour and Mahjouba Baiya and Hicham Yahyaoui and Mustapha Ait Ameur and Mohamed Chakour},
      title = {Sezary Syndrome: A Clinico-biological Study of 5 Cases},
      journal = {American Journal of Biomedical and Life Sciences},
      volume = {10},
      number = {2},
      pages = {42-44},
      doi = {10.11648/j.ajbls.20221002.16},
      url = {https://doi.org/10.11648/j.ajbls.20221002.16},
      eprint = {https://article.sciencepublishinggroup.com/pdf/10.11648.j.ajbls.20221002.16},
      abstract = {Background: Sézary syndrome (SS) is a rare erythrodermic and leukemic variant of cutaneous T-cell lymphoma (CTCL) that belongs to the group of non-Hodgkin's lymphomas (NHL) resulting from malignant proliferation of skin-homing T cells. We report through a series of 5 cases, the experience of the hematology laboratory in the diagnosis of the syndrome of Sézary. Methods: This is a retrospective study of 5 cases of Sezary syndrome collected in the dermatology department of Marrakech. Results: five patients were identified with the clinicopathological criteria of SS. At the time of diagnosis, all 5 patients had erythroderma and generalized lymphadenopathy in both superficial and deep stations. The white blood cell count was elevated (>10,000 WBC/mm3) in all 5 patients with a mean value of 18,120 WBC/mm3. The blood smear showed the presence of 75% (27 G/l) of small to medium-sized cells with a high nucleocytoplasmic ratio and cerebriform nuclei typical of Sezary cells and suggests the diagnosis of SS. Conclusions: The diagnosis of SS remains a challenge in many situations, the pathophysiology and definition of SS have evolved significantly over the past decades.},
     year = {2022}
    }
    

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  • TY  - JOUR
    T1  - Sezary Syndrome: A Clinico-biological Study of 5 Cases
    AU  - Rania Rada
    AU  - Hajar Saffour
    AU  - Mahjouba Baiya
    AU  - Hicham Yahyaoui
    AU  - Mustapha Ait Ameur
    AU  - Mohamed Chakour
    Y1  - 2022/04/20
    PY  - 2022
    N1  - https://doi.org/10.11648/j.ajbls.20221002.16
    DO  - 10.11648/j.ajbls.20221002.16
    T2  - American Journal of Biomedical and Life Sciences
    JF  - American Journal of Biomedical and Life Sciences
    JO  - American Journal of Biomedical and Life Sciences
    SP  - 42
    EP  - 44
    PB  - Science Publishing Group
    SN  - 2330-880X
    UR  - https://doi.org/10.11648/j.ajbls.20221002.16
    AB  - Background: Sézary syndrome (SS) is a rare erythrodermic and leukemic variant of cutaneous T-cell lymphoma (CTCL) that belongs to the group of non-Hodgkin's lymphomas (NHL) resulting from malignant proliferation of skin-homing T cells. We report through a series of 5 cases, the experience of the hematology laboratory in the diagnosis of the syndrome of Sézary. Methods: This is a retrospective study of 5 cases of Sezary syndrome collected in the dermatology department of Marrakech. Results: five patients were identified with the clinicopathological criteria of SS. At the time of diagnosis, all 5 patients had erythroderma and generalized lymphadenopathy in both superficial and deep stations. The white blood cell count was elevated (>10,000 WBC/mm3) in all 5 patients with a mean value of 18,120 WBC/mm3. The blood smear showed the presence of 75% (27 G/l) of small to medium-sized cells with a high nucleocytoplasmic ratio and cerebriform nuclei typical of Sezary cells and suggests the diagnosis of SS. Conclusions: The diagnosis of SS remains a challenge in many situations, the pathophysiology and definition of SS have evolved significantly over the past decades.
    VL  - 10
    IS  - 2
    ER  - 

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Author Information
  • Laboratory of Hematology, Avicenne Military Hospital, Marrakech, Morocco

  • Laboratory of Hematology, Avicenne Military Hospital, Marrakech, Morocco

  • Laboratory of Hematology, Avicenne Military Hospital, Marrakech, Morocco

  • Laboratory of Hematology, Avicenne Military Hospital, Marrakech, Morocco

  • Laboratory of Hematology, Avicenne Military Hospital, Marrakech, Morocco

  • Laboratory of Hematology, Avicenne Military Hospital, Marrakech, Morocco

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