Secondary Hemophagocytic Lymphohistiocytosis: A Challenging Diagnosis in a Patient with Autoimmune Hepatitis
Background. We describe a case of secondary Hemophagocytic Lymphohistiocytosis (HLH) from autoimmune hepatitis mimicking severe sepsis in a man admitted to the intensive care unit. Case Presentation. A 34-year-old Pakistani male with a prior history of biopsy-proven autoimmune hepatitis presented to...
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| Format: | Article |
| Language: | English |
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Wiley
2019-01-01
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| Series: | Case Reports in Critical Care |
| Online Access: | http://dx.doi.org/10.1155/2019/3580796 |
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| _version_ | 1832552647348977664 |
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| author | Colin Casault Juan G. Posadas-Calleja |
| author_facet | Colin Casault Juan G. Posadas-Calleja |
| author_sort | Colin Casault |
| collection | DOAJ |
| description | Background. We describe a case of secondary Hemophagocytic Lymphohistiocytosis (HLH) from autoimmune hepatitis mimicking severe sepsis in a man admitted to the intensive care unit. Case Presentation. A 34-year-old Pakistani male with a prior history of biopsy-proven autoimmune hepatitis presented to a regional hospital with severe fever, cytopenias, hyperferritinemia, hypertriglyceridemia, splenomegaly, and a bone marrow biopsy showing hemophagocytosis. After ruling out mimicking conditions, a diagnosis of HLH was made using the HLH-2004 diagnostic criteria. He was treated with dexamethasone and etoposide, without bone marrow transplantation (BMT) due to poor functional status. At one-year after follow-up, he had returned to his baseline functional status without recurrence. Conclusion. We describe a rare case of secondary HLH in the setting of autoimmune hepatitis. Broadly, this case report educates clinicians to consider this potentially missed diagnosis. This case also informs clinicians that treatment of secondary HLH with BMT may not be necessary for the management of secondary HLH due to autoimmune hepatitis. Finally, it provides a detailed description of the natural history of a single patient with secondary HLH due to autoimmune hepatitis. |
| format | Article |
| id | doaj-art-6c00db7a61fb4365b2ea949595d12b03 |
| institution | Kabale University |
| issn | 2090-6420 2090-6439 |
| language | English |
| publishDate | 2019-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Case Reports in Critical Care |
| spelling | doaj-art-6c00db7a61fb4365b2ea949595d12b032025-02-03T05:58:12ZengWileyCase Reports in Critical Care2090-64202090-64392019-01-01201910.1155/2019/35807963580796Secondary Hemophagocytic Lymphohistiocytosis: A Challenging Diagnosis in a Patient with Autoimmune HepatitisColin Casault0Juan G. Posadas-Calleja1Department of Critical Care Medicine, University of Calgary, Alberta, CanadaDepartment of Critical Care Medicine, University of Calgary, Alberta, CanadaBackground. We describe a case of secondary Hemophagocytic Lymphohistiocytosis (HLH) from autoimmune hepatitis mimicking severe sepsis in a man admitted to the intensive care unit. Case Presentation. A 34-year-old Pakistani male with a prior history of biopsy-proven autoimmune hepatitis presented to a regional hospital with severe fever, cytopenias, hyperferritinemia, hypertriglyceridemia, splenomegaly, and a bone marrow biopsy showing hemophagocytosis. After ruling out mimicking conditions, a diagnosis of HLH was made using the HLH-2004 diagnostic criteria. He was treated with dexamethasone and etoposide, without bone marrow transplantation (BMT) due to poor functional status. At one-year after follow-up, he had returned to his baseline functional status without recurrence. Conclusion. We describe a rare case of secondary HLH in the setting of autoimmune hepatitis. Broadly, this case report educates clinicians to consider this potentially missed diagnosis. This case also informs clinicians that treatment of secondary HLH with BMT may not be necessary for the management of secondary HLH due to autoimmune hepatitis. Finally, it provides a detailed description of the natural history of a single patient with secondary HLH due to autoimmune hepatitis.http://dx.doi.org/10.1155/2019/3580796 |
| spellingShingle | Colin Casault Juan G. Posadas-Calleja Secondary Hemophagocytic Lymphohistiocytosis: A Challenging Diagnosis in a Patient with Autoimmune Hepatitis Case Reports in Critical Care |
| title | Secondary Hemophagocytic Lymphohistiocytosis: A Challenging Diagnosis in a Patient with Autoimmune Hepatitis |
| title_full | Secondary Hemophagocytic Lymphohistiocytosis: A Challenging Diagnosis in a Patient with Autoimmune Hepatitis |
| title_fullStr | Secondary Hemophagocytic Lymphohistiocytosis: A Challenging Diagnosis in a Patient with Autoimmune Hepatitis |
| title_full_unstemmed | Secondary Hemophagocytic Lymphohistiocytosis: A Challenging Diagnosis in a Patient with Autoimmune Hepatitis |
| title_short | Secondary Hemophagocytic Lymphohistiocytosis: A Challenging Diagnosis in a Patient with Autoimmune Hepatitis |
| title_sort | secondary hemophagocytic lymphohistiocytosis a challenging diagnosis in a patient with autoimmune hepatitis |
| url | http://dx.doi.org/10.1155/2019/3580796 |
| work_keys_str_mv | AT colincasault secondaryhemophagocyticlymphohistiocytosisachallengingdiagnosisinapatientwithautoimmunehepatitis AT juangposadascalleja secondaryhemophagocyticlymphohistiocytosisachallengingdiagnosisinapatientwithautoimmunehepatitis |