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Case Report
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| Human herpesvirus-8 related hemophagocytic lymphohistiocytosis with Kaposi sarcoma in an immunocompetent HIV negative adult | ||||||
| Talal Alnabelsi1, Cecilia Jang1, Ramzi Mulki1, Amin Benyounes1, Manju Balasubramanian2, Mark Morginstin3 | ||||||
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1MD, Resident, Internal Medicine, Einstein Medical Center, Philadelphia, PA.
2MD, Attending physician, Pathology, Einstein Medical Center, Philadelphia, PA. 3DO, Attending physician, Hematology and Oncology, Einstein Medical Center, Philadelphia, PA. | ||||||
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| Alnabelsi T, Jang C, Mulki R, Benyounes A, Balasubramanian M, Morginstin M. Human herpesvirus-8 related hemophagocytic lymphohistiocytosis with Kaposi sarcoma in an immunocompetent HIV negative adult. Int J Case Rep Images 2016;7(8):546–550. |
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Abstract
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Introduction:
Hemophagocytic lympho-histiocytosis (HLH) is an aggressive and life-threatening disorder characterized by proliferation of the mononuclear phagocytic system resulting in marked hemophagocytosis. While HLH may occur as a consequence of a herpes viral infection, this usually arises in immunocompromised individuals. We report a rare case of HLH with Kaposi sarcoma triggered by human herpesvirus-8 (HHV-8) infection in an immunocompetent adult.
Case Report: Our patient is a 58-year-old male with a recent diagnosis of Kaposi sarcoma of his lower extremities hospitalized for fever, chills and night sweats. Evaluation revealed pancytopenia, splenomegaly, diffuse lymphadenopathy and Kaposi sarcoma on lymph node biopsy in the absence of HIV infection. Investigations also revealed hemophagocytosis on bone marrow examination. A diagnosis of secondary Hemophagocytic Lymphohistiocytosis was made and the patient was initiated on etoposide and steroids with marked improvement of symptoms and blood counts. One month later, he was hospitalized for high grade fevers and was again found to be pancytopenic and septic with multi-organ failure. The patient's condition rapidly deteriorated and he succumbed to his illness five days later. Conclusion: Hemophagocytic lymphohistiocytosis is a rare and devastating condition typically arising in immunocompromised individuals. Our patient had non-specific symptoms in addition to pancytopenia and lymphadenopathy in the setting of Kaposi sarcoma. While this may seldom be compatible with an HHV-8 infection, a thorough search revealed associated HLH. Although extremely rare, HLH should be considered in immunocompetent patients with HHV-8 related manifestations particularly in the presence of pancytopenia. | |
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Keywords:
Hemophagocytosis, Kaposi sarcoma, Immunocompetent, Human herpesvirus-8
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Author Contributions
Talal Alnabelsi – Substantial contributions to conception and design, Acquisition of data, Analysis and interpretation of data, Drafting the article, Revising it critically for important intellectual content, Final approval of the version to be published Cecilia Jang – Substantial contributions to conception and design, Revising it critically for important intellectual content, Final approval of the version to be published Ramzi Mulki – Substantial contributions to conception and design, Revising it critically for important intellectual content, Final approval of the version to be published Amin Benyounes – Substantial contributions to conception and design, Revising it critically for important intellectual content, Final approval of the version to be published Manju Balasubramanian – Substantial contributions to conception and design, Revising it critically for important intellectual content, Final approval of the version to be published Mark Morginstin – Substantial contributions to conception and design, Revising it critically for important intellectual content, Final approval of the version to be published |
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Guarantor of submission
The corresponding author is the guarantor of submission. |
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Source of support
None |
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Conflict of interest
Authors declare no conflict of interest. |
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Copyright
© 2016 Talal Alnabelsi et al. This article is distributed under the terms of Creative Commons Attribution License which permits unrestricted use, distribution and reproduction in any medium provided the original author(s) and original publisher are properly credited. Please see the copyright policy on the journal website for more information. |
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