Patients with an extraordinarily elevated serum ferritin: think of haemophagocytic lymphohistiocytosis
BACKGROUND: We retrospectively analysed charts of patients with blood ferritin level >5000 µg/l. The aim of the study was to look for the likelihood of haemophagocytic lymphohistiocytosis (HLH) in these patients. METHODS: Forty-two patients demonstrated hyperferritinaemia and could be...
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| Format: | Article |
| Language: | English |
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SMW supporting association (Trägerverein Swiss Medical Weekly SMW)
2015-06-01
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| Series: | Swiss Medical Weekly |
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| Online Access: | https://www.smw.ch/index.php/smw/article/view/2039 |
| _version_ | 1811194188178391040 |
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| author | Marc Schweizer Jeroen S. Goede Verena Briner |
| author_facet | Marc Schweizer Jeroen S. Goede Verena Briner |
| author_sort | Marc Schweizer |
| collection | DOAJ |
| description |
BACKGROUND: We retrospectively analysed charts of patients with blood ferritin level >5000 µg/l. The aim of the study was to look for the likelihood of haemophagocytic lymphohistiocytosis (HLH) in these patients.
METHODS: Forty-two patients demonstrated hyperferritinaemia and could be evaluated. The diagnosis of HLH was based on a recently published HScore and an earlier diagnostic algorithm.
RESULTS: According to the algorithm, 20 patients fulfilled the criteria for a diagnosis of HLH. However, patients with Still’s disease have macrophage activation and, in this context, a rise in ferritin without having HLH. Fourteen patients with carcinoma, haematological malignancies or infection and hyperferritinaemia remained. Signs and symptoms were: systemic inflammatory response syndrome (SIRS 100%), fever (95%), cytopenia of ≥2 lines (70%), immunosuppression (61.5%), splenomegaly (50%), elevated liver enzymes (45%), lymphadenopathy (35%), hepatomegaly (30%). These are nonspecific parameters. Therefore HLH may be overdiagnosed. Using the HScore, only 10 patients had >80% probability of having HLH. Patients demonstrating cytopenia of ≥2 cell lines had a >60% mortality rate. Time to death was 13.8 days; death was most often due to multiorgan failure.
CONCLUSION: HScore reflects a higher specificity than the algorithm for diagnosing HLH. The discrepancy may indicate the difficulty that a specific marker still is missing. Hyperferritinaemia was strongly associated with HLH in patients with haematological or oncological malignancies. HLH may be underdiagnosed because the majority of these patients suffer from a severe underlying disease, which easily might suggest a flare or infection. In this population, hyperferritinaemia and SIRS should rise suspicion because mortality in HLH is high.
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| first_indexed | 2024-04-12T00:21:49Z |
| format | Article |
| id | doaj.art-ef9437b3c7d143cebd7cf9e78ddb118f |
| institution | Directory Open Access Journal |
| issn | 1424-3997 |
| language | English |
| last_indexed | 2024-04-12T00:21:49Z |
| publishDate | 2015-06-01 |
| publisher | SMW supporting association (Trägerverein Swiss Medical Weekly SMW) |
| record_format | Article |
| series | Swiss Medical Weekly |
| spelling | doaj.art-ef9437b3c7d143cebd7cf9e78ddb118f2022-12-22T03:55:42ZengSMW supporting association (Trägerverein Swiss Medical Weekly SMW)Swiss Medical Weekly1424-39972015-06-01145252610.4414/smw.2015.14152Patients with an extraordinarily elevated serum ferritin: think of haemophagocytic lymphohistiocytosisMarc SchweizerJeroen S. GoedeVerena Briner BACKGROUND: We retrospectively analysed charts of patients with blood ferritin level >5000 µg/l. The aim of the study was to look for the likelihood of haemophagocytic lymphohistiocytosis (HLH) in these patients. METHODS: Forty-two patients demonstrated hyperferritinaemia and could be evaluated. The diagnosis of HLH was based on a recently published HScore and an earlier diagnostic algorithm. RESULTS: According to the algorithm, 20 patients fulfilled the criteria for a diagnosis of HLH. However, patients with Still’s disease have macrophage activation and, in this context, a rise in ferritin without having HLH. Fourteen patients with carcinoma, haematological malignancies or infection and hyperferritinaemia remained. Signs and symptoms were: systemic inflammatory response syndrome (SIRS 100%), fever (95%), cytopenia of ≥2 lines (70%), immunosuppression (61.5%), splenomegaly (50%), elevated liver enzymes (45%), lymphadenopathy (35%), hepatomegaly (30%). These are nonspecific parameters. Therefore HLH may be overdiagnosed. Using the HScore, only 10 patients had >80% probability of having HLH. Patients demonstrating cytopenia of ≥2 cell lines had a >60% mortality rate. Time to death was 13.8 days; death was most often due to multiorgan failure. CONCLUSION: HScore reflects a higher specificity than the algorithm for diagnosing HLH. The discrepancy may indicate the difficulty that a specific marker still is missing. Hyperferritinaemia was strongly associated with HLH in patients with haematological or oncological malignancies. HLH may be underdiagnosed because the majority of these patients suffer from a severe underlying disease, which easily might suggest a flare or infection. In this population, hyperferritinaemia and SIRS should rise suspicion because mortality in HLH is high. https://www.smw.ch/index.php/smw/article/view/2039Hyperferritinemiasystemic inflammatory response syndromeStill’s diseaseHLHhaematological and solid neoplasm |
| spellingShingle | Marc Schweizer Jeroen S. Goede Verena Briner Patients with an extraordinarily elevated serum ferritin: think of haemophagocytic lymphohistiocytosis Swiss Medical Weekly Hyperferritinemia systemic inflammatory response syndrome Still’s disease HLH haematological and solid neoplasm |
| title | Patients with an extraordinarily elevated serum ferritin: think of haemophagocytic lymphohistiocytosis |
| title_full | Patients with an extraordinarily elevated serum ferritin: think of haemophagocytic lymphohistiocytosis |
| title_fullStr | Patients with an extraordinarily elevated serum ferritin: think of haemophagocytic lymphohistiocytosis |
| title_full_unstemmed | Patients with an extraordinarily elevated serum ferritin: think of haemophagocytic lymphohistiocytosis |
| title_short | Patients with an extraordinarily elevated serum ferritin: think of haemophagocytic lymphohistiocytosis |
| title_sort | patients with an extraordinarily elevated serum ferritin think of haemophagocytic lymphohistiocytosis |
| topic | Hyperferritinemia systemic inflammatory response syndrome Still’s disease HLH haematological and solid neoplasm |
| url | https://www.smw.ch/index.php/smw/article/view/2039 |
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