Efficacy of cascade plasmapheresis in comparison with conventional therapeutic plasma exchange for relapsed atypical hemolytic uremic syndrome: A case report

Shastry, Shamee; Vaidya, Ashwinkumar; Polavarapu, Isha; Prabhu, RavindraAttur; Mohan, Ganesh; Prethika, PA

doi:10.4103/ajts.ajts_121_21

Cited by 2 publications

(3 citation statements)

References 11 publications

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“…This approach effectively eliminates dysregulated, non-functional proteins that regulate both the classical and alternative pathways of the complement system. If, even after five consecutive daily plasma exchanges, hemolysis persists or renal function does not show improvement, it signifies uncontrolled aHUS-this is the case even if the platelet count returns to normal levels (2,11,12).…”

Section: Discussionmentioning

confidence: 99%

Multifactorial etiology of atipical hemolytic uremic syndrome: Case report

Škorić,

Klačar-Uzelac,

Kostić

2024

Sanamed

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Introduction: Hemolytic uremic syndromes are characterized by the simultaneous occurrence of hemolytic anemia, microangiopathy, thrombocytopenia, and acute renal insufficiency. In terms of the clinical prodrome, they can be classified as typical, which is more common and occurs in 90% of cases, often preceded by diarrheal syndrome induced by enterohemorrhagic Escherichia coli. Alternatively, there is an atypical and rarer form associated with pneumococcal infection, dysregulation of the alternative complement pathway, and cases involving the use of cyclosporine. Hemolytic anemia is confirmed in laboratory analyses (presence of fragmented red blood cells, decreased hemoglobin, undetectable haptoglobin values, and elevated LDH values), along with thrombocytopenia and an increase in nitrogenous substances (urea and creatinine). Case report: The report details the case of an 18-month-old girl who experienced acute renal insufficiency subsequent to a respiratory infection. Ten days preceding admission, the patient exhibited nasal discharge, and during the seven days leading up to hospitalization, she presented with fever. Furthermore, two days prior to admission, the onset of persistent vomiting and abdominal pain occurred. Suspected of bowel intussusception, the patient underwent a surgical assessment where acute surgical pathology was ruled out. The absence of urination, coupled with heightened urea and creatinine levels, prompted consideration of hemolytic-uremic syndrome, later confirmed as atypical during hospitalization. This was grounded in the clinical presentation, devoid of diarrhea syndrome but marked by nasal discharge over the preceding ten days. The administration of fresh frozen plasma yielded no improvement, and there were decreased values of the C3 complement component, H factor, and reduced ADAMTS13 activity. The lack of verotoxins from enterohemorrhagic Escherichia coli further supported the diagnosis of atypical hemolytic-uremic syndrome. After the first dose of eculizumab, a terminal complement C5 component inhibitor, the girl recovered renal function and established diuresis. Conclusion: The prompt diagnosis of atypical hemolytic-uremic syndrome is challenging due to nonspecific symptoms like nasal discharge, vomiting, fatigue, and abdominal pain. Laboratory analyses, lacking specific criteria, make it difficult to conclusively identify aHUS at the disease's onset. In Serbia, pneumococcal immunization is recommended as a preventive measure, administered through a conjugated vaccine in three doses starting from the second month of life. Rapid and accurate differentiation between typical and atypical HUS is crucial for effective treatment and prognosis. Typical HUS requires hemodialysis and plasmapheresis, whereas atypical HUS is managed with plasmapheresis, immunosuppressive therapy, and eculizumab. Administering eculizumab heightens the risk of meningococcal infection by inhibiting the C5 complement component. Therefore, it is crucial not to disregard the importance of meningococcal immunization.

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Section: Discussionmentioning

confidence: 99%

Multifactorial etiology of atipical hemolytic uremic syndrome: Case report

Škorić,

Klačar-Uzelac,

Kostić

2024

Sanamed

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“…Atypical HUS is a rare and life‐threatening form of TMA, associated with high mortality and morbidity. The incidence of aHUS is approximately 1‐2 per million per year 6 . Atypical HUS has a poor prognosis, with 50% of cases progressing to ESKD and mortality rates as high as 10%‐15% 7 .…”

Section: Discussionmentioning

confidence: 99%

“…The incidence of aHUS is approximately 1-2 per million per year. 6 Atypical HUS has a poor prognosis, with 50% of cases progressing to ESKD and mortality rates as high as 10%-15%. 7 Regardless of the underlying disease, aHUS results from vascular endothelial damage leading to platelet activation, micro thrombus formation, microangiopathic hemolytic anemia, activation of the complement system, and multiple end-organ injuries, including renal and extrarenal organ and system involvements such as neurologic, cardiac, gastrointestinal, and respiratory systems.…”

Section: On Admissionmentioning

confidence: 99%

Why has plasma exchange failed in TRACK syndrome? Lessons from a new variant of the atypical hemolytic uremic syndrome

Durak¹,

Sahin²,

Can³

et al. 2023

J of Clinical Apheresis

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Atypical hemolytic uremic syndrome (aHUS) is a rare and life‐threatening form of thrombotic microangiopathy, associated with high mortality and morbidity. Most cases present with hemolytic anemia, thrombocytopenia, and renal insufficiency. However, it can have unusual multiple end‐organ injuries including extrarenal organ and system involvements such as neurologic, cardiac, gastrointestinal, and respiratory systems. We describe a 4‐year‐old girl who developed aHUS due to the TSEN2 mutation and had cardiac involvement. She did not benefit from plasma exchange, as stated in previous cases. It should be kept in mind that therapeutic plasma exchange may not be beneficial in some cases of aHUS, especially due to genetic mutations.

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Efficacy of cascade plasmapheresis in comparison with conventional therapeutic plasma exchange for relapsed atypical hemolytic uremic syndrome: A case report

Cited by 2 publications

References 11 publications

Multifactorial etiology of atipical hemolytic uremic syndrome: Case report

Multifactorial etiology of atipical hemolytic uremic syndrome: Case report

Why has plasma exchange failed in TRACK syndrome? Lessons from a new variant of the atypical hemolytic uremic syndrome

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