Hyperhemolysis Syndrome in a Patient with Sickle Cell Disease: A Case Report

Kalter, Joshua A; Gupta, Ranju; Greenberg, Marna Rayl; Miller, A.; Allen, Jamie

doi:10.5811/cpcem.2020.12.50349

Cited by 5 publications

(5 citation statements)

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“…Simultaneously, the physiological protective mechanisms against free heme become fulminant and the complement system, activated through the classical or alternative pathways, generates extreme hemolysis via the membrane attack complex. As a result, the red blood cells are destroyed as they are not only sensitive to oxidative stress but also to the non-specific binding of activated complement factors [11,[18][19][20][21].…”

Section: Discussionmentioning

confidence: 99%

A Case Report of Hyperhemolytic Syndrome in Sickle Cell Disease, with a Special Focus on Avoiding the Use of Transfusions

Obajed Al-Ali,

Pfliegler,

Magyari

et al. 2024

Thalassemia Reports

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In patients with sickle cell disease (SCD), transfusions pose risks like delayed hemolytic transfusion reaction (DHTR) and hyperhemolytic syndrome (HHS). We present the case of a 61-year-old Nigerian male patient with SCD, developing hyperhemolytic syndrome (HHS) post-orthopedic surgery due to alloimmunization from blood transfusions. Surgery induced massive hemorrhage, requiring RBC transfusions. Postoperatively, he developed HHS with jaundice, hemoglobinuria, and fever. Despite additional transfusions, his condition worsened, leading to hematological consultation on postoperative day +9. Laboratory findings showed positive DAT and multiple alloantibodies. The diagnosis of HHS was established and treatment involved high-dose methylprednisolone, intravenous immunoglobulin (IVIG), and erythropoietin. The patient was discharged on postoperative day +24 with stable hemoglobin levels, tapering doses of methylprednisolone, and continuous administration of hydroxyurea prescribed. HHS pathogenesis involves extensive intravascular hemolysis, exacerbated by alloimmunization. Diagnostic challenges and therapy selection complexity underscore the need for cautious transfusion strategies in HHS, reserving them for hemodynamic instability or hypoxia. This case highlights promptly recognizing and managing HHS in SCD for improved outcomes and avoiding unnecessary transfusions.

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Section: Discussionmentioning

confidence: 99%

A Case Report of Hyperhemolytic Syndrome in Sickle Cell Disease, with a Special Focus on Avoiding the Use of Transfusions

Obajed Al-Ali,

Pfliegler,

Magyari

et al. 2024

Thalassemia Reports

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“…HHS was diagnosed in a pregnant patient with compound-heterozygous SCD/β 0 -thalassemia due to symptomatic hemolysis 3 weeks after transfusion of a PRBC and with formation of new erythrocyte alloantibodies [5,12,20,26]. Due to the greater presence of hemolysis due to the underlying hemoglobinopathy in patients affected with HHS, it is frequently misdiagnosed, followed by an increased risk of potentially fatal complications occurring due to a delayed diagnosis [17]. Especially in patients with hemoglobinopathies and a history of transfusions, HHS should be considered, when presenting with posttransfusion hemolysis.…”

Section: Discussionmentioning

confidence: 99%

“…The direct antiglobulin test (DAT) is often negative, but in some cases, it can show weakly positive IgG and C3d, and new alloantibodies are often also present [12][13][14][15][16]. The clinical presentation varies from fever to jaundice to pain and can be difficult to differentiate from a VOC in a SCD patient [17].…”

Section: Introductionmentioning

confidence: 99%

“Don’t Add Fuel to the Fire”– Hyperhemolysis Syndrome in a Pregnant Woman with Compound Sickle Cell Disease/β<sup>0</sup>-Thalassemia: Case Report and Review of the Literature

Rihsling,

Simeunovic,

Sanchez

et al. 2023

Acta Haematol

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Hyperhemolysis syndrome (HHS) is a rare and severe posttransfusion complication characterized by the destruction of both recipient and donor red blood cells. The underlying mechanism of HHS is not fully understood and proper management can be difficult. Furthermore, there are few reports regarding HHS in pregnancy. We report on the development and management of HHS in a pregnant woman with known compound sickle cell disease (SCD)/β0-thalassemia and alloimmunization after transfusion of packed red blood cells (PRBC). We aim to raise awareness on this diagnostically challenging and life-threatening type of hemolysis with this report, and to stress the need to consider the diagnosis of HHS in SCD patients with progressive anemia despite PRBC administration.

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“…The liver has an important role in the regeneration of red blood cells [26][27][28]. According to Eissa and Zidan [29][30], reduction in red blood cell counts could result from the failure of the liver to supply the blood circulation with cells from haemohepatic tissues and the possible destructive effect on red blood cells by anti-nutrientive factors. Since PCV levels reflect the extent and efficiency of oxygen uptake and transfer to tissues [31][32], the low values in the test groups may signify a reduction in the body's metabolic activity [33][34][35].…”

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confidence: 99%

Effects of Biopolymers from Cassava Implanted with Rodent Bait on Wistar Rats

Okareh¹,

JC-UkA²,

Oyagbemi³

et al. 2021

Pol. J. Environ. Stud.

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The increase in the use of plastic-based materials has resulted in the generation of large volumes of plastic wastes that are non-biodegradable, and this is of public health concern. Hence, there is a need to adopt an eco-friendly technology that is capable of producing plastics that are biodegradable, and less hazardous to man and the environment. The study focuses on the conversion of biopolymer to bioplastic rodent bait and its toxicological effects. The toxicological effect of the product was tested on forty-five male Wistar rats randomly distributed into 9 groups after acclimatization period of two weeks. The groups include: Group 1 (Bioplastic alone); Group 2 (Bait meal + 1.5 g zinc phosphide); Group 3 (Bait meal + 3 g zinc phosphide), and Group 4 (Bait meal + 6 g zinc phosphide). Rats were fed ad libitum for 5 days in which their body weights, morphological changes, and time of death were recorded daily. After exposure experimental to diets, histopathological, biochemical, and haematological parameters were determined. Morphological observations such as reduced activities, fur loss in toxicant fed rats in comparison to the control were observed. The results showed that serum Aspartate Transaminase (AST) and Alanine Transferase (ALT) activities were highest in test groups compared to the control. The histopathological analysis revealed high levels of inflammatory cells, necrosis and haemorrhage within the test groups when compared with the control. Bioplastics produced from cassava residues as rodent bait were highly effective. These bioplastics can be used for the control of household rodents. Therefore, this technology can be adopted for domestic eradication of rodents.

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Hyperhemolysis Syndrome in a Patient with Sickle Cell Disease: A Case Report

Cited by 5 publications

References 0 publications

A Case Report of Hyperhemolytic Syndrome in Sickle Cell Disease, with a Special Focus on Avoiding the Use of Transfusions

A Case Report of Hyperhemolytic Syndrome in Sickle Cell Disease, with a Special Focus on Avoiding the Use of Transfusions

“Don’t Add Fuel to the Fire”– Hyperhemolysis Syndrome in a Pregnant Woman with Compound Sickle Cell Disease/β<sup>0</sup>-Thalassemia: Case Report and Review of the Literature

Effects of Biopolymers from Cassava Implanted with Rodent Bait on Wistar Rats

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