Sandeep Rao Kordcal, Sarthak Kain, Amandeep Singh, Manish Soneja, Naval K Vikram, Hem Pandey, Tulika Seth
{"title":"输血后反常的血红蛋白下降:伴有巨噬细胞激活综合征的地中海贫血的高溶血。","authors":"Sandeep Rao Kordcal, Sarthak Kain, Amandeep Singh, Manish Soneja, Naval K Vikram, Hem Pandey, Tulika Seth","doi":"10.1093/qjmed/hcaf168","DOIUrl":null,"url":null,"abstract":"<p><p>Hyperhaemolysis syndrome (HHS) is a rare, severe complication of transfusion characterised by a paradoxical drop in haemoglobin (Hb) levels post-transfusion, often below pre-transfusion values. Most reported cases are in patients with sickle cell disease and it has been rarely reported in other haemoglobinopathies. We report a case of HHS in a newly diagnosed delta-beta thalassaemia patient, manifesting as macrophage activation syndrome (MAS), successfully managed with intravenous immunoglobulin (IVIG), corticosteroids, rituximab, and erythropoietin. A 19-year-old female presented with fever, jaundice, and hepatosplenomegaly three weeks after a blood transfusion. Investigations revealed microcytic anaemia, elevated LDH and indirect bilirubin, and HPLC findings suggestive of delta-beta thalassaemia. She developed haemolysis and a paradoxical Hb drop after subsequent transfusions, along with reticulocytopenia. Alloimmunisation was initially absent but anti-E and anti-K antibodies were subsequently detected. Despite antigen-matched transfusions, haemolysis persisted, raising suspicion for HHS. She was managed with IVIG, methylprednisolone, erythropoietin, and rituximab, resulting in initial improvement in haemoglobin levels, which subsequently declined again. She then developed fever and leukocytosis without evidence of infection, and a high HScore raised suspicion for macrophage activation syndrome (MAS). Dexamethasone was initiated, leading to defervescence, normalisation of the reticulocyte count, and a significant rise in haemoglobin. She was discharged in stable condition and remained well at 6-month follow-up. This case highlights hyperhaemolysis syndrome (HHS) as a potential complication in patients with haemoglobinopathies. Although its exact pathogenesis remains unclear, macrophage activation appears to play an important role. Early recognition and timely initiation of appropriate immunotherapy can lead to favourable outcomes.</p>","PeriodicalId":20806,"journal":{"name":"QJM: An International Journal of Medicine","volume":" ","pages":""},"PeriodicalIF":6.4000,"publicationDate":"2025-07-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Paradoxical Hemoglobin Drop Post-Transfusion: Hyperhemolysis in Delta-Beta Thalassemia with Macrophage Activation Syndrome.\",\"authors\":\"Sandeep Rao Kordcal, Sarthak Kain, Amandeep Singh, Manish Soneja, Naval K Vikram, Hem Pandey, Tulika Seth\",\"doi\":\"10.1093/qjmed/hcaf168\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><p>Hyperhaemolysis syndrome (HHS) is a rare, severe complication of transfusion characterised by a paradoxical drop in haemoglobin (Hb) levels post-transfusion, often below pre-transfusion values. Most reported cases are in patients with sickle cell disease and it has been rarely reported in other haemoglobinopathies. We report a case of HHS in a newly diagnosed delta-beta thalassaemia patient, manifesting as macrophage activation syndrome (MAS), successfully managed with intravenous immunoglobulin (IVIG), corticosteroids, rituximab, and erythropoietin. A 19-year-old female presented with fever, jaundice, and hepatosplenomegaly three weeks after a blood transfusion. Investigations revealed microcytic anaemia, elevated LDH and indirect bilirubin, and HPLC findings suggestive of delta-beta thalassaemia. She developed haemolysis and a paradoxical Hb drop after subsequent transfusions, along with reticulocytopenia. Alloimmunisation was initially absent but anti-E and anti-K antibodies were subsequently detected. Despite antigen-matched transfusions, haemolysis persisted, raising suspicion for HHS. She was managed with IVIG, methylprednisolone, erythropoietin, and rituximab, resulting in initial improvement in haemoglobin levels, which subsequently declined again. She then developed fever and leukocytosis without evidence of infection, and a high HScore raised suspicion for macrophage activation syndrome (MAS). Dexamethasone was initiated, leading to defervescence, normalisation of the reticulocyte count, and a significant rise in haemoglobin. She was discharged in stable condition and remained well at 6-month follow-up. This case highlights hyperhaemolysis syndrome (HHS) as a potential complication in patients with haemoglobinopathies. Although its exact pathogenesis remains unclear, macrophage activation appears to play an important role. Early recognition and timely initiation of appropriate immunotherapy can lead to favourable outcomes.</p>\",\"PeriodicalId\":20806,\"journal\":{\"name\":\"QJM: An International Journal of Medicine\",\"volume\":\" \",\"pages\":\"\"},\"PeriodicalIF\":6.4000,\"publicationDate\":\"2025-07-31\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"QJM: An International Journal of Medicine\",\"FirstCategoryId\":\"3\",\"ListUrlMain\":\"https://doi.org/10.1093/qjmed/hcaf168\",\"RegionNum\":4,\"RegionCategory\":\"医学\",\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"Q1\",\"JCRName\":\"MEDICINE, GENERAL & INTERNAL\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"QJM: An International Journal of Medicine","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.1093/qjmed/hcaf168","RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q1","JCRName":"MEDICINE, GENERAL & INTERNAL","Score":null,"Total":0}
Paradoxical Hemoglobin Drop Post-Transfusion: Hyperhemolysis in Delta-Beta Thalassemia with Macrophage Activation Syndrome.
Hyperhaemolysis syndrome (HHS) is a rare, severe complication of transfusion characterised by a paradoxical drop in haemoglobin (Hb) levels post-transfusion, often below pre-transfusion values. Most reported cases are in patients with sickle cell disease and it has been rarely reported in other haemoglobinopathies. We report a case of HHS in a newly diagnosed delta-beta thalassaemia patient, manifesting as macrophage activation syndrome (MAS), successfully managed with intravenous immunoglobulin (IVIG), corticosteroids, rituximab, and erythropoietin. A 19-year-old female presented with fever, jaundice, and hepatosplenomegaly three weeks after a blood transfusion. Investigations revealed microcytic anaemia, elevated LDH and indirect bilirubin, and HPLC findings suggestive of delta-beta thalassaemia. She developed haemolysis and a paradoxical Hb drop after subsequent transfusions, along with reticulocytopenia. Alloimmunisation was initially absent but anti-E and anti-K antibodies were subsequently detected. Despite antigen-matched transfusions, haemolysis persisted, raising suspicion for HHS. She was managed with IVIG, methylprednisolone, erythropoietin, and rituximab, resulting in initial improvement in haemoglobin levels, which subsequently declined again. She then developed fever and leukocytosis without evidence of infection, and a high HScore raised suspicion for macrophage activation syndrome (MAS). Dexamethasone was initiated, leading to defervescence, normalisation of the reticulocyte count, and a significant rise in haemoglobin. She was discharged in stable condition and remained well at 6-month follow-up. This case highlights hyperhaemolysis syndrome (HHS) as a potential complication in patients with haemoglobinopathies. Although its exact pathogenesis remains unclear, macrophage activation appears to play an important role. Early recognition and timely initiation of appropriate immunotherapy can lead to favourable outcomes.
期刊介绍:
QJM, a renowned and reputable general medical journal, has been a prominent source of knowledge in the field of internal medicine. With a steadfast commitment to advancing medical science and practice, it features a selection of rigorously reviewed articles.
Released on a monthly basis, QJM encompasses a wide range of article types. These include original papers that contribute innovative research, editorials that offer expert opinions, and reviews that provide comprehensive analyses of specific topics. The journal also presents commentary papers aimed at initiating discussions on controversial subjects and allocates a dedicated section for reader correspondence.
In summary, QJM's reputable standing stems from its enduring presence in the medical community, consistent publication schedule, and diverse range of content designed to inform and engage readers.