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Hyperhemolysis Syndrome in a Patient with Sickle Cell Disease: A Case Report
INTRODUCTION: Hyperhemolysis syndrome (HHS) is a rare complication of repeat blood transfusions in sickle cell disease (SCD). This can occur acutely or have a delayed presentation and often goes unrecognized in the emergency department (ED) due to its rapid progression and similarity to acute chest...
Autores principales: | , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
University of California Irvine, Department of Emergency Medicine publishing Western Journal of Emergency Medicine
2021
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7872616/ https://www.ncbi.nlm.nih.gov/pubmed/33560964 http://dx.doi.org/10.5811/cpcem.2020.12.50349 |
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author | Kalter, Joshua A. Gupta, Ranju Greenberg, Marna Rayl Miller, Andrew J. Allen, Jamie |
author_facet | Kalter, Joshua A. Gupta, Ranju Greenberg, Marna Rayl Miller, Andrew J. Allen, Jamie |
author_sort | Kalter, Joshua A. |
collection | PubMed |
description | INTRODUCTION: Hyperhemolysis syndrome (HHS) is a rare complication of repeat blood transfusions in sickle cell disease (SCD). This can occur acutely or have a delayed presentation and often goes unrecognized in the emergency department (ED) due to its rapid progression and similarity to acute chest syndrome and other common complications of SCD. CASE REPORT: We present a case of a 20-year-old male with SCD who presented to the ED with pain and tenderness in his lower extremities one day after discharge for a crisis. Unbeknownst to the ED team, during his admission he had received a blood transfusion. On presentation he was noted to have hyperkalemia, hyperbilirubinemia, anemia, and uncontrolled pain, and was admitted for sickle cell pain crisis. Over the next 36 hours, his hemoglobin dropped precipitously from 8.9 grams per deciliter (g/dL) to 4.2 g/dL (reference range: 11.5–14.5 g/dL), reticulocyte count from 11.7 % to 3.8% (0.4–2.2%), and platelets from 318,000 per cubic centimeter (K/cm(3)) to 65 K/cm(3) (140–350 K/cm(3)). He also developed a fever, hypoxia, transaminitis, a deteriorating mental status, and severe lactic acidosis. Hematology was consulted and he was treated with methylprednisolone, intravenous immunoglobulin, two units of antigen-matched red blood cells, fresh frozen plasma, and cryoprecipitate. He was transferred to an outside hospital for exchange transfusion and remained hospitalized for 26 days with acute liver failure, bone marrow necrosis, and a fever of unknown origin. CONCLUSION: Because of the untoward outcomes associated with delay in HHS diagnosis and the need for early initiation of steroids, it is important for emergency providers to screen patients with hemoglobinopathies for recent transfusion at ED presentation. Asking the simple question about when a patient’s last transfusion occurred can lead an emergency physician to include HHS in the differential and work-up of patients with sickle cell disease complications. |
format | Online Article Text |
id | pubmed-7872616 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2021 |
publisher | University of California Irvine, Department of Emergency Medicine publishing Western Journal of Emergency Medicine |
record_format | MEDLINE/PubMed |
spelling | pubmed-78726162021-02-12 Hyperhemolysis Syndrome in a Patient with Sickle Cell Disease: A Case Report Kalter, Joshua A. Gupta, Ranju Greenberg, Marna Rayl Miller, Andrew J. Allen, Jamie Clin Pract Cases Emerg Med Case Report INTRODUCTION: Hyperhemolysis syndrome (HHS) is a rare complication of repeat blood transfusions in sickle cell disease (SCD). This can occur acutely or have a delayed presentation and often goes unrecognized in the emergency department (ED) due to its rapid progression and similarity to acute chest syndrome and other common complications of SCD. CASE REPORT: We present a case of a 20-year-old male with SCD who presented to the ED with pain and tenderness in his lower extremities one day after discharge for a crisis. Unbeknownst to the ED team, during his admission he had received a blood transfusion. On presentation he was noted to have hyperkalemia, hyperbilirubinemia, anemia, and uncontrolled pain, and was admitted for sickle cell pain crisis. Over the next 36 hours, his hemoglobin dropped precipitously from 8.9 grams per deciliter (g/dL) to 4.2 g/dL (reference range: 11.5–14.5 g/dL), reticulocyte count from 11.7 % to 3.8% (0.4–2.2%), and platelets from 318,000 per cubic centimeter (K/cm(3)) to 65 K/cm(3) (140–350 K/cm(3)). He also developed a fever, hypoxia, transaminitis, a deteriorating mental status, and severe lactic acidosis. Hematology was consulted and he was treated with methylprednisolone, intravenous immunoglobulin, two units of antigen-matched red blood cells, fresh frozen plasma, and cryoprecipitate. He was transferred to an outside hospital for exchange transfusion and remained hospitalized for 26 days with acute liver failure, bone marrow necrosis, and a fever of unknown origin. CONCLUSION: Because of the untoward outcomes associated with delay in HHS diagnosis and the need for early initiation of steroids, it is important for emergency providers to screen patients with hemoglobinopathies for recent transfusion at ED presentation. Asking the simple question about when a patient’s last transfusion occurred can lead an emergency physician to include HHS in the differential and work-up of patients with sickle cell disease complications. University of California Irvine, Department of Emergency Medicine publishing Western Journal of Emergency Medicine 2021-01-26 /pmc/articles/PMC7872616/ /pubmed/33560964 http://dx.doi.org/10.5811/cpcem.2020.12.50349 Text en Copyright: © 2021 Kalter et al. http://creativecommons.org/licenses/by/4.0/ This is an open access article distributed in accordance with the terms of the Creative Commons Attribution (CC BY 4.0) License. See: http://creativecommons.org/licenses/by/4.0/ |
spellingShingle | Case Report Kalter, Joshua A. Gupta, Ranju Greenberg, Marna Rayl Miller, Andrew J. Allen, Jamie Hyperhemolysis Syndrome in a Patient with Sickle Cell Disease: A Case Report |
title | Hyperhemolysis Syndrome in a Patient with Sickle Cell Disease: A Case Report |
title_full | Hyperhemolysis Syndrome in a Patient with Sickle Cell Disease: A Case Report |
title_fullStr | Hyperhemolysis Syndrome in a Patient with Sickle Cell Disease: A Case Report |
title_full_unstemmed | Hyperhemolysis Syndrome in a Patient with Sickle Cell Disease: A Case Report |
title_short | Hyperhemolysis Syndrome in a Patient with Sickle Cell Disease: A Case Report |
title_sort | hyperhemolysis syndrome in a patient with sickle cell disease: a case report |
topic | Case Report |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7872616/ https://www.ncbi.nlm.nih.gov/pubmed/33560964 http://dx.doi.org/10.5811/cpcem.2020.12.50349 |
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