Hemophagocytic lymphohistiocytosis, an unclear nosologic entity: case report of an adult man with rising of amylase and lipase and spinal cord infiltration
Here we present the case of a 57-years old patient affected by hemophagocytic lymphohistiocytosis (HLH), a rare disease characterized by an uncontrolled immune activation, resulting in clinical and biochemical manifestations of extreme inflammation. In a previous hospitalization, the patient showed...
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| Format: | Article |
| Language: | English |
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MDPI AG
2017-03-01
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| Series: | Hematology Reports |
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| Online Access: | http://www.pagepress.org/journals/index.php/hr/article/view/6859 |
| _version_ | 1797936919319937024 |
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| author | Moris Sangineto Antonio Perrone Pasquale Agosti Viera Boccuti Anna Campobasso Carlo Sabbà |
| author_facet | Moris Sangineto Antonio Perrone Pasquale Agosti Viera Boccuti Anna Campobasso Carlo Sabbà |
| author_sort | Moris Sangineto |
| collection | DOAJ |
| description | Here we present the case of a 57-years old patient affected by hemophagocytic lymphohistiocytosis (HLH), a rare disease characterized by an uncontrolled immune activation, resulting in clinical and biochemical manifestations of extreme inflammation. In a previous hospitalization, the patient showed fever, hepato-splenomegaly, pancytopenia, hyperferrtitinemia, lymphadenopathy and cholestasis. No diagnosis was done, however, he totally recovered after splenectomy. Eight months later, he relapsed, showing also hypofibrinogenemia, hypertriglyceridemia, hemophagocytic signs in bone marrow, cholestatic jaundice, high LDH and high PT-INR. Interestingly, he presented increased levels of amylase and lipase in absence of radiologic signs of pancreatitis. He was treated with Dexamethasone and Cyclosporine according to HLH-2004 guidelines. The clinical and biochemical manifestations disappeared in a few weeks, but he was newly hospitalized for lower limbs hypotonia caused by a hemophagocytic lesion of the cauda equina and lumbar cord. The death occurred in a few days, despite the immunosuppressive treatment. |
| first_indexed | 2024-04-10T18:37:52Z |
| format | Article |
| id | doaj.art-ca1924c69862415b88801386df185d8f |
| institution | Directory Open Access Journal |
| issn | 2038-8322 2038-8330 |
| language | English |
| last_indexed | 2024-04-10T18:37:52Z |
| publishDate | 2017-03-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Hematology Reports |
| spelling | doaj.art-ca1924c69862415b88801386df185d8f2023-02-02T00:45:39ZengMDPI AGHematology Reports2038-83222038-83302017-03-019110.4081/hr.2017.68593641Hemophagocytic lymphohistiocytosis, an unclear nosologic entity: case report of an adult man with rising of amylase and lipase and spinal cord infiltrationMoris Sangineto0Antonio Perrone1Pasquale Agosti2Viera Boccuti3Anna Campobasso4Carlo Sabbà5Department of Interdisciplinary Medicine, University of Bari “Aldo Moro”Department of Interdisciplinary Medicine, University of Bari “Aldo Moro”Department of Interdisciplinary Medicine, University of Bari “Aldo Moro”Department of Interdisciplinary Medicine, University of Bari “Aldo Moro”Department of Interdisciplinary Medicine, University of Bari “Aldo Moro”Department of Interdisciplinary Medicine, University of Bari “Aldo Moro”Here we present the case of a 57-years old patient affected by hemophagocytic lymphohistiocytosis (HLH), a rare disease characterized by an uncontrolled immune activation, resulting in clinical and biochemical manifestations of extreme inflammation. In a previous hospitalization, the patient showed fever, hepato-splenomegaly, pancytopenia, hyperferrtitinemia, lymphadenopathy and cholestasis. No diagnosis was done, however, he totally recovered after splenectomy. Eight months later, he relapsed, showing also hypofibrinogenemia, hypertriglyceridemia, hemophagocytic signs in bone marrow, cholestatic jaundice, high LDH and high PT-INR. Interestingly, he presented increased levels of amylase and lipase in absence of radiologic signs of pancreatitis. He was treated with Dexamethasone and Cyclosporine according to HLH-2004 guidelines. The clinical and biochemical manifestations disappeared in a few weeks, but he was newly hospitalized for lower limbs hypotonia caused by a hemophagocytic lesion of the cauda equina and lumbar cord. The death occurred in a few days, despite the immunosuppressive treatment.http://www.pagepress.org/journals/index.php/hr/article/view/6859Hemophagocytic Lymphohistiocytosis, spinal cord, amylase and lipase, splenectomy |
| spellingShingle | Moris Sangineto Antonio Perrone Pasquale Agosti Viera Boccuti Anna Campobasso Carlo Sabbà Hemophagocytic lymphohistiocytosis, an unclear nosologic entity: case report of an adult man with rising of amylase and lipase and spinal cord infiltration Hematology Reports Hemophagocytic Lymphohistiocytosis, spinal cord, amylase and lipase, splenectomy |
| title | Hemophagocytic lymphohistiocytosis, an unclear nosologic entity: case report of an adult man with rising of amylase and lipase and spinal cord infiltration |
| title_full | Hemophagocytic lymphohistiocytosis, an unclear nosologic entity: case report of an adult man with rising of amylase and lipase and spinal cord infiltration |
| title_fullStr | Hemophagocytic lymphohistiocytosis, an unclear nosologic entity: case report of an adult man with rising of amylase and lipase and spinal cord infiltration |
| title_full_unstemmed | Hemophagocytic lymphohistiocytosis, an unclear nosologic entity: case report of an adult man with rising of amylase and lipase and spinal cord infiltration |
| title_short | Hemophagocytic lymphohistiocytosis, an unclear nosologic entity: case report of an adult man with rising of amylase and lipase and spinal cord infiltration |
| title_sort | hemophagocytic lymphohistiocytosis an unclear nosologic entity case report of an adult man with rising of amylase and lipase and spinal cord infiltration |
| topic | Hemophagocytic Lymphohistiocytosis, spinal cord, amylase and lipase, splenectomy |
| url | http://www.pagepress.org/journals/index.php/hr/article/view/6859 |
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