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. 2016 Nov;175(4):744-747.
doi: 10.1111/bjh.13884. Epub 2015 Dec 21.

Atypical haemolytic uraemic syndrome in a patient with sickle cell disease, successfully treated with eculizumab

Satheesh Chonat  1 , Shanmuganathan Chandrakasan  1 , Karen Ann Kalinyak  1 , David Ingala  1 , Ralph Gruppo  1 , Theodosia A Kalfa  1
Affiliations

Atypical haemolytic uraemic syndrome in a patient with sickle cell disease, successfully treated with eculizumab

Satheesh Chonat et al. Br J Haematol. 2016 Nov.
No abstract available

Keywords: atypical haemolytic uraemic syndrome; complement mutations; eculizumab; sickle cell disease; thrombotic microangiopathy.

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Conflict of interest statement

Competing interests

SC, ShC, KAK, DI and TAK have nothing to disclose and no competing interests. RG is a member of the speaker’s bureau for Alexion Pharmaceuticals, Inc., manufacturer of eculizumab.

Figures

Fig 1
Fig 1
The patient was diagnosed with acute chest syndrome and treated with erythrocytapheresis on day 0. Haemolysis [haemoglobin decrease by 30 g/l and lactate dehydrogenase (LDH) >4000 iu/l; upper limit of normal 920 iu/l], thrombocytopenia (platelet count down to 31 × 109/l) and renal failure (creatinine up to 548 μmol/l) developed by day 1, requiring dialysis. With the diagnosis of thrombotic microangiopathy on day 4, the patient was started on plasmapheresis (p/a) with slow improvement of her renal failure. Eculizumab was initiated on day 11 due to worsening respiratory status with plasma therapy; the patient had already been immunized against meningococcus according to sickle cell disease care guidelines. A remarkable clinical improvement was noted within a day after eculizumab infusion, while normalization of LDH, creatinine and platelet count followed in the ensuing 7 d. Retrospective evaluation of sC5b-9 plasma levels (MicrovueTM Enzyme Immunoassay, Quidel Corporation, San Diego, CA, USA) revealed that sC5b-9 was initially markedly elevated to >800 ng/ml (normal <244 ng/ml). The levels improved after plasmapheresis and eculizumab treatment; no plasma samples were available between day 14 and 73 to determine the sC5b-9 course in detail. We confirmed the return to normal levels when the patient had completely recovered and was seen in follow-up on days 73–77. During dialysis and plasma therapy, creatinine levels remained elevated between 230 and 539 μmol/l and decreased only after eculizumab was started, normalizing (below 84 μmol/l) by day 22. Dotted lines signify the higher level of normal range for SC5b-9, LDH and creatinine. Eculizumab therapy was cautiously discontinued after a 4-week course with close monitoring of the patient.
Fig 2
Fig 2
(A) Blood smear on day 4 showing schistocytes and helmet cells (arrows), along with paucity of platelets. (B) Blood smear after complete recovery, post-eculizumab, showing occasional sickle cells (arrowheads) and normal platelet count. Polychromasia of the red blood cells seen in this blood smear is normal at baseline for this patient with sickle cell disease.
See this image and copyright information in PMC

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