A severe case of atypical hemolytic uremic syndrome associated with pneumococcal infection and T activation treated successfully with plasma exchange

BACKGROUND: A severe nondiarrheal form of hemolytic uremic syndrome in children is associated with pneumococcal infection (pHUS). Neuraminidase released by the pneumococci may cleave N‐acetylneuraminic acid residues on red blood cells (RBCs), leading to the exposure of the T cryptantigen and polyagg...

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Bibliographic Details
Published in:Transfusion (Philadelphia, Pa.) Pa.), 2008-11, Vol.48 (11), p.2448-2452
Main Authors: Hopkins, Courtney K., Yuan, Shan, Lu, Qun, Ziman, Alyssa, Goldfinger, Dennis
Format: Article
Language:English
Subjects:
Anesthesia. Intensive care medicine. Transfusions. Cell therapy and gene therapy
Anti-Bacterial Agents - therapeutic use
Antigens, Tumor-Associated, Carbohydrate - analysis
Bacterial Proteins - metabolism
Biological and medical sciences
Blood. Blood and plasma substitutes. Blood products. Blood cells. Blood typing. Plasmapheresis. Apheresis
Child, Preschool
Combined Modality Therapy
Coombs Test
Drug toxicity and drugs side effects treatment
Erythrocyte Aggregation
Erythrocyte Transfusion
Hemagglutination Tests
Hemolytic-Uremic Syndrome - blood
Hemolytic-Uremic Syndrome - etiology
Hemolytic-Uremic Syndrome - therapy
Humans
Male
Medical sciences
Neuraminidase - metabolism
Pharmacology. Drug treatments
Plasma Exchange
Pneumococcal Infections - blood
Pneumococcal Infections - complications
Pneumococcal Infections - drug therapy
Remission Induction
Renal Dialysis
Respiration, Artificial
Serum Albumin - administration & dosage
Streptococcus pneumoniae
Streptococcus pneumoniae - enzymology
Toxicity: blood
Transfusions. Complications. Transfusion reactions. Cell and gene therapy
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Summary:BACKGROUND: A severe nondiarrheal form of hemolytic uremic syndrome in children is associated with pneumococcal infection (pHUS). Neuraminidase released by the pneumococci may cleave N‐acetylneuraminic acid residues on red blood cells (RBCs), leading to the exposure of the T cryptantigen and polyagglutinability of RBCs, a process known as T activation. Data suggest a pathogenic role of exposed T antigens on glomeruli interacting with naturally occurring anti‐T in the development of renal dysfunction in pHUS. By reducing the levels of anti‐T and neuraminidase, plasma exchange (PE) may have a role in the treatment of severe cases of pHUS. CASE REPORT: A previously healthy 2‐year‐old boy presented with acute renal failure, thrombocytopenia, microangiopathic hemolytic anemia, pneumococcal infection, and T activation of RBCs. A diagnosis of pHUS was made. Due to rapid clinical decline, daily single‐volume PE with 5 percent albumin replacement was initiated. Infusion of additional plasma was avoided by using only saline‐washed RBCs for transfusion. He made a full recovery after 13 PEs and remained well at follow‐up 7 months later. RESULTS: Polyagglutinability of RBCs was shown by mixing patient RBCs with five normal donor sera. The agglutination assays with a panel of lectins confirmed the specificity of exposed T antigen as the cause of polyagglutinability. CONCLUSION: The dramatic response seen in this patient suggests that PE utilizing albumin replacement may benefit patients with severe pHUS.
ISSN:0041-1132
1537-2995
DOI:10.1111/j.1537-2995.2008.01871.x