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A Case Report on Idiopathic-Multi-centric Castleman’s Disease Associated POEMS- Syndrome

Received: 13 January 2022     Accepted: 3 February 2022     Published: 16 February 2022
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Abstract

Castleman’s Disease is a rare lymphoproliferative disorder of idiopathic etio-pathogenesis and was first described by Dr Benjamin Castleman in 1954. It is a rather rare disease entity in clinical practice. However, the incidence cannot be ignored. It may affect a single lymph node (Unicentric CD) or multiple lymph nodes throughout the body (Multicentric CD). Management depends upon the type and severity of the disease and early recognition of the condition. Our case report is that of a 60-year-old female patient with underlying comorbidities including type 2 diabetes mellitus, hypothyroidism who presented with complaints of generalized weakness, weight loss, hair loss, non-resolving muco-purulent rhinorrhea, bilateral pedal edema and multiple, non-tender, firm and mobile left sided Level I and II ‘AJCC’ cervical lymphadenopathy of >2cm which was later biopsied and diagnosed to be Castleman’s disease associated POEMS syndrome. The aim of this report is to consider CD as a differential diagnosis in patients presenting with similar clinical picture and early identification and treatment may provide a better prognosis. We would also like to highlight 3 take home points: 1) The significance of histology in differentiating it from other lymph proliferative conditions. 2) LN excision of the bothersome LN can be diagnostic and may be considered therapeutic to some extend even in MCD. 3) Steroid therapy has beneficial effects to control progression of the disease.

Published in International Journal of Clinical Oncology and Cancer Research (Volume 7, Issue 1)
DOI 10.11648/j.ijcocr.20220701.13
Page(s) 14-16
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), 2022. Published by Science Publishing Group

Keywords

Castleman’s Disease, POEMS Syndrome, Lymph Node Hyperplasia, HIV, Paraproteinemia

References
[1] Castleman, B.; Iverson, L.; Menendez, V. P. (July 1956). "Localized mediastinal lymphnode hyperplasia resembling thymoma". Cancer. 9 (4): 822–830.
[2] Van Rhee F, Munshi NC. Castleman Disease. Hematol Oncol Clin North Am. 2018 Feb. 32 (1): xiii-xiv. [Medline].
[3] Haap M, Wiefels J, Horger M, Hoyer A, Müssig K. Clinical, laboratory and imaging findings in Castleman's disease - The subtype decides. Blood Rev. May 2018. 32 (3): 225-234. [Medline].
[4] Chronowski GM, Ha CS, Wilder RB, Cabanillas F, Manning J, Cox JD. Treatment of unicentric and multicentric Castleman disease and the role of radiotherapy. Cancer. 2001; 92 (3): 670–6.
[5] Talat N, Belgaumkar AP, Schulte KM. Surgery in Castleman's disease: a systematic review of 404 published cases. Ann Surg. 2012 Apr. 255 (4): 677-84. [Medline].
[6] Iyer, S., Bhatti, M. I., & Halliday, M. (2010). Castleman's disease-A case report. International journal of surgery case reports, 1 (3), 25–26. https://doi.org/10.1016/j.ijscr.2010.06.005.
[7] Yu L, Tu M, Cortes J, et al. Clinical and pathological characteristics of HIV- and HHV-8-negative Castleman disease. Blood. 2017; 129 (12): 1658-1668.
[8] Yoshizaki K, Matsuda T, Nishimoto N, et al. Pathogenic significance of interleukin-6 (IL-6/BSF-2) in Castleman’s disease. Blood. 1989; 74 (4): 1360-1367.
[9] Bowne WB, Lewis JJ, Filippa DA, Niesvizky R, Brooks AD, Burt ME, et al. The management of unicentric and multicentric Castleman’s disease. Cancer. 1999; 85: 706–17.
[10] Van Rhee, Frits et al. “International, evidence-based consensus treatment guidelines for idiopathic multicentric Castleman disease.” Blood vol. 132, 20 (2018): 2115-2124. doi: 10.1182/blood-2018-07-862334.
[11] Dispenzieri A. POEMS Syndrome: 2019 Update on diagnosis, risk-stratification, and management. Am J Hematol. 2019 Jul; 94 (7): 812-827. doi: 10.1002/ajh.25495. Epub 2019 May 23. PMID: 31012139.
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  • APA Style

    Amritha Radhakrishnan Nair, Changaramkumarath Gichin, Deepa Pramod Raj Santhi, Moothiringode Chithrabhanu Savithri. (2022). A Case Report on Idiopathic-Multi-centric Castleman’s Disease Associated POEMS- Syndrome. International Journal of Clinical Oncology and Cancer Research, 7(1), 14-16. https://doi.org/10.11648/j.ijcocr.20220701.13

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    ACS Style

    Amritha Radhakrishnan Nair; Changaramkumarath Gichin; Deepa Pramod Raj Santhi; Moothiringode Chithrabhanu Savithri. A Case Report on Idiopathic-Multi-centric Castleman’s Disease Associated POEMS- Syndrome. Int. J. Clin. Oncol. Cancer Res. 2022, 7(1), 14-16. doi: 10.11648/j.ijcocr.20220701.13

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    AMA Style

    Amritha Radhakrishnan Nair, Changaramkumarath Gichin, Deepa Pramod Raj Santhi, Moothiringode Chithrabhanu Savithri. A Case Report on Idiopathic-Multi-centric Castleman’s Disease Associated POEMS- Syndrome. Int J Clin Oncol Cancer Res. 2022;7(1):14-16. doi: 10.11648/j.ijcocr.20220701.13

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  • @article{10.11648/j.ijcocr.20220701.13,
      author = {Amritha Radhakrishnan Nair and Changaramkumarath Gichin and Deepa Pramod Raj Santhi and Moothiringode Chithrabhanu Savithri},
      title = {A Case Report on Idiopathic-Multi-centric Castleman’s Disease Associated POEMS- Syndrome},
      journal = {International Journal of Clinical Oncology and Cancer Research},
      volume = {7},
      number = {1},
      pages = {14-16},
      doi = {10.11648/j.ijcocr.20220701.13},
      url = {https://doi.org/10.11648/j.ijcocr.20220701.13},
      eprint = {https://article.sciencepublishinggroup.com/pdf/10.11648.j.ijcocr.20220701.13},
      abstract = {Castleman’s Disease is a rare lymphoproliferative disorder of idiopathic etio-pathogenesis and was first described by Dr Benjamin Castleman in 1954. It is a rather rare disease entity in clinical practice. However, the incidence cannot be ignored. It may affect a single lymph node (Unicentric CD) or multiple lymph nodes throughout the body (Multicentric CD). Management depends upon the type and severity of the disease and early recognition of the condition. Our case report is that of a 60-year-old female patient with underlying comorbidities including type 2 diabetes mellitus, hypothyroidism who presented with complaints of generalized weakness, weight loss, hair loss, non-resolving muco-purulent rhinorrhea, bilateral pedal edema and multiple, non-tender, firm and mobile left sided Level I and II ‘AJCC’ cervical lymphadenopathy of >2cm which was later biopsied and diagnosed to be Castleman’s disease associated POEMS syndrome. The aim of this report is to consider CD as a differential diagnosis in patients presenting with similar clinical picture and early identification and treatment may provide a better prognosis. We would also like to highlight 3 take home points: 1) The significance of histology in differentiating it from other lymph proliferative conditions. 2) LN excision of the bothersome LN can be diagnostic and may be considered therapeutic to some extend even in MCD. 3) Steroid therapy has beneficial effects to control progression of the disease.},
     year = {2022}
    }
    

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    JF  - International Journal of Clinical Oncology and Cancer Research
    JO  - International Journal of Clinical Oncology and Cancer Research
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    AB  - Castleman’s Disease is a rare lymphoproliferative disorder of idiopathic etio-pathogenesis and was first described by Dr Benjamin Castleman in 1954. It is a rather rare disease entity in clinical practice. However, the incidence cannot be ignored. It may affect a single lymph node (Unicentric CD) or multiple lymph nodes throughout the body (Multicentric CD). Management depends upon the type and severity of the disease and early recognition of the condition. Our case report is that of a 60-year-old female patient with underlying comorbidities including type 2 diabetes mellitus, hypothyroidism who presented with complaints of generalized weakness, weight loss, hair loss, non-resolving muco-purulent rhinorrhea, bilateral pedal edema and multiple, non-tender, firm and mobile left sided Level I and II ‘AJCC’ cervical lymphadenopathy of >2cm which was later biopsied and diagnosed to be Castleman’s disease associated POEMS syndrome. The aim of this report is to consider CD as a differential diagnosis in patients presenting with similar clinical picture and early identification and treatment may provide a better prognosis. We would also like to highlight 3 take home points: 1) The significance of histology in differentiating it from other lymph proliferative conditions. 2) LN excision of the bothersome LN can be diagnostic and may be considered therapeutic to some extend even in MCD. 3) Steroid therapy has beneficial effects to control progression of the disease.
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Author Information
  • Internal Medicine, Intern, General Hospital, Thrissur, India

  • Internal Medicine, Intern, General Hospital, Thrissur, India

  • Internal Medicine, General Hospital, Thrissur, India

  • Department of Pathology, Amala Institute of Medical Sciences, Thrissur, India

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