An Atypical Presentation of Hemophagocytic Lymphohistiocytosis (HLH) Secondary to Occult Hodgkin Lymphoma
Hemophagocytic lymphohistiocytosis (HLH) is a rare and life-threatening syndrome of immune system dysregulation characterized by the phagocytosis of various cells by histiocytes in the bone marrow. HLH can present in one of the two ways: primary HLH, which is caused by mutations in genes essential t...
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| Format: | Article |
| Language: | English |
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Wiley
2021-01-01
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| Series: | Case Reports in Hematology |
| Online Access: | http://dx.doi.org/10.1155/2021/6672257 |
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| author | Justin Komisarof Kevin McGann Alissa Huston Hani Katerji Mary Anne Morgan |
| author_facet | Justin Komisarof Kevin McGann Alissa Huston Hani Katerji Mary Anne Morgan |
| author_sort | Justin Komisarof |
| collection | DOAJ |
| description | Hemophagocytic lymphohistiocytosis (HLH) is a rare and life-threatening syndrome of immune system dysregulation characterized by the phagocytosis of various cells by histiocytes in the bone marrow. HLH can present in one of the two ways: primary HLH, which is caused by mutations in genes essential to T and NK-cell function, and secondary HLH, typically caused by Epstein–Barr virus (EBV) infection or malignancy. Because of the rapid progression and high mortality of this disease, prompt diagnosis is essential to good outcomes. Here, we report the 2-month clinical course of a patient who presented with altered mental status and recurrent fever of unknown origin. Initially, he did not meet diagnostic criteria for HLH and had a negative bone marrow biopsy; however, he eventually progressed to full-blown HLH secondary to occult Hodgkin lymphoma. This case is unusual for the slow and smoldering course of the patient’s disease and highlights the importance of aggressively searching for potential malignancies to ensure the initiation of definitive therapy as soon as possible. |
| format | Article |
| id | doaj-art-307889f3abcd4e56808ff97b39576b64 |
| institution | OA Journals |
| issn | 2090-6560 2090-6579 |
| language | English |
| publishDate | 2021-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Case Reports in Hematology |
| spelling | doaj-art-307889f3abcd4e56808ff97b39576b642025-08-20T02:07:53ZengWileyCase Reports in Hematology2090-65602090-65792021-01-01202110.1155/2021/66722576672257An Atypical Presentation of Hemophagocytic Lymphohistiocytosis (HLH) Secondary to Occult Hodgkin LymphomaJustin Komisarof0Kevin McGann1Alissa Huston2Hani Katerji3Mary Anne Morgan4Department of Medicine, University of Rochester Medical Center, 601 Elmwood Avenue Box MED, Rochester, NY 14642, USADepartment of Medicine, University of Rochester Medical Center, 601 Elmwood Avenue Box MED, Rochester, NY 14642, USADepartment of Medicine, University of Rochester Medical Center, 601 Elmwood Avenue Box MED, Rochester, NY 14642, USADepartment of Pathology and Laboratory Medicine, University of Rochester Medical Center, 601 Elmwood Avenue Box 626, Rochester, NY 14642, USADepartment of Medicine, University of Rochester Medical Center, 601 Elmwood Avenue Box MED, Rochester, NY 14642, USAHemophagocytic lymphohistiocytosis (HLH) is a rare and life-threatening syndrome of immune system dysregulation characterized by the phagocytosis of various cells by histiocytes in the bone marrow. HLH can present in one of the two ways: primary HLH, which is caused by mutations in genes essential to T and NK-cell function, and secondary HLH, typically caused by Epstein–Barr virus (EBV) infection or malignancy. Because of the rapid progression and high mortality of this disease, prompt diagnosis is essential to good outcomes. Here, we report the 2-month clinical course of a patient who presented with altered mental status and recurrent fever of unknown origin. Initially, he did not meet diagnostic criteria for HLH and had a negative bone marrow biopsy; however, he eventually progressed to full-blown HLH secondary to occult Hodgkin lymphoma. This case is unusual for the slow and smoldering course of the patient’s disease and highlights the importance of aggressively searching for potential malignancies to ensure the initiation of definitive therapy as soon as possible.http://dx.doi.org/10.1155/2021/6672257 |
| spellingShingle | Justin Komisarof Kevin McGann Alissa Huston Hani Katerji Mary Anne Morgan An Atypical Presentation of Hemophagocytic Lymphohistiocytosis (HLH) Secondary to Occult Hodgkin Lymphoma Case Reports in Hematology |
| title | An Atypical Presentation of Hemophagocytic Lymphohistiocytosis (HLH) Secondary to Occult Hodgkin Lymphoma |
| title_full | An Atypical Presentation of Hemophagocytic Lymphohistiocytosis (HLH) Secondary to Occult Hodgkin Lymphoma |
| title_fullStr | An Atypical Presentation of Hemophagocytic Lymphohistiocytosis (HLH) Secondary to Occult Hodgkin Lymphoma |
| title_full_unstemmed | An Atypical Presentation of Hemophagocytic Lymphohistiocytosis (HLH) Secondary to Occult Hodgkin Lymphoma |
| title_short | An Atypical Presentation of Hemophagocytic Lymphohistiocytosis (HLH) Secondary to Occult Hodgkin Lymphoma |
| title_sort | atypical presentation of hemophagocytic lymphohistiocytosis hlh secondary to occult hodgkin lymphoma |
| url | http://dx.doi.org/10.1155/2021/6672257 |
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