Hemolytic Non-uremic Syndrome
Chen, K. S., Neuneright, C. E., Crary, S. E., & Buchanan, G. R. (2012). Hemolytic non-uremic syndrome. Pediatric blood & cancer, 59(1), 167–169. https://doi.org/10.1002/pbc.23264
View Original Source →Abstract
More than 50% of patients with non-Shiga toxin-associated hemolytic uremic syndrome (non-Stx-HUS) progress to ESRD. Kidney transplant failure for disease recurrence is common; hence, whether renal transplantation is appropriate in this clinical setting remains a debated issue. The aim of this study was to identify possible prognostic factors for renal transplant outcome by focusing on specific genetic abnormalities associated with the disease. All articles in literature that describe renal transplant outcome in patients with ESRD secondary to non-Stx-HUS, genotyped for CFH, MCP, and IF mutations, were reviewed, and data of patients who were referred to the International Registry of Recurrent and Familial HUS/TTP and data from the Newcastle cohort were examined. This study confirmed that the overall outcome of kidney transplantation in patients with non-Stx-HUS is poor, with disease recurring in 60% of patients, 91.6% of whom developed graft failure. No clinical prognostic factor that could identify patients who were at high risk for graft failure was found. The presence of a factor H (CFH) mutation was associated with a high incidence of graft failure (77.8 versus 54.9% in patients without CFH mutation). Similar results were seen in patients with a factor I (IF) mutation. In contrast, graft outcome was favorable in all patients who carried a membrane co-factor protein (MCP) mutation. Patients with non-Stx-HUS should undergo genotyping before renal transplantation to help predict the risk for graft failure. It is debatable whether a kidney transplant should be recommended for patients with CFH or IF mutation. Reasonably, patients with an MCP mutation can undergo a kidney transplant without risk for recurrence.
Case Details
Disease Location
Systemic
Personal Characteristics
A 13- months-old female
Clinical Characteristics
Was admitted with pallor and fatigue preceded by one day of non-bloody diarrhea 2 weeks ago. Hemoglobin concentration was 3.6 g/dl with a reticulocyte count of 0.8%, platelets of 98,000/mm3 and abundant schistocytes on the blood smear following a transfusion of packed red blood cells and fresh frozen plasma, her hemoglobin concentration rose to 7.2 g/dl, but the reticulocyte count did not increase. Bone marrow biopsy demonstrated erythroid and megakaryocytic hyperplasia. Viral pcr tests were negative for cmv, ebv, and parvovirus.
Remission Characteristics
Following a transfusion and plasma, her hemoglobin concentration rose to 7.2 g/dl, but the reticulocyte count did not increase. Bone marrow biopsy demonstrated erythroid and megakaryocytic hyperplasia. Viral pcr tests were negative for cmv, ebv, and parvovirus two weeks later, with one more transfusion, her hemoglobin concentration was 11.6 g/dl with a normal peripheral smear, and it remained stable over the next four months
Treatment & Mechanisms
Proposed Remission Mechanisms
The children reported here perhaps therefore represent a variant of hus which we have termed ‘‘hemolytic non-uremic syndrome’’. Its transient and limited nature implies that it, too, is toxin-mediated, perhaps by a novel mechanism not heretofore identified. Alternatively, host factors may possibly protect these children from developing overnight renal failure through down-regulation of renal gb3 receptors or a polymorphism that confers resistance to shiga toxin..
Clinical Treatment
Transfusion with red packed cells and fresh frozen plasma
Non-Clinical Treatment
The cases described above do not fit into these well-defined entities