Idiopathic hemophagocytic lymphohistiocytosis during pregnancy treated with steroids
| Autor: | Bachar Samra, Mohamad Yasmin, Sami Arnaout, Jacques Azzi |
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| Jazyk: | angličtina |
| Rok vydání: | 2015 |
| Předmět: | |
| Zdroj: | Hematology Reports, Vol 7, Iss 3 (2015) |
| Druh dokumentu: | article |
| ISSN: | 2038-8322 2038-8330 |
| DOI: | 10.4081/hr.2015.6100 |
| Popis: | Hemophagocytic lymphohistiocytosis (HLH) is a rare and severe clinical syndrome characterized by a dysregulated hyperinflammatory immune response. The diagnosis of HLH during pregnancy is especially challenging due to the rarity of this condition. The highly variable clinical presentation, laboratory findings, and associated diagnoses accompanying this syndrome further complicate the problem. A pronounced hyperferritinemia in the setting of systemic signs and symptoms along with a negative infectious and rheumatological workup should raise suspicions for HLH. While treatment ideally consists of immunosuppressive chemotherapy and hematopoietic stem cell transplant, the potential toxicity to both the pregnant woman and the fetus poses a challenging decision. We report the first case of idiopathic HLH presenting as fever of unknown origin in a pregnant woman successfully treated with steroids. |
| Databáze: | Directory of Open Access Journals |
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