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HEMOLYTIC UREMIC SYNDROME

Quite often massive destruction of erythrocytes with the subsequent increase of the
content of free hemoglobin in blood is combined with development of a severe renal
failure up to an anury a hemolytic uraemic syndrome. Thus application only of a
hemodialysis isn't able to stop this complication quickly enough. The best results are
received after use of a plasma exchange, especially at the phenomena of not stopped
intoxication, the proceeding hemolysis and high level of average weight molecules in
blood [Davin J.C. et al., 2008, 2009; Forzley B.R. et al., 2008; Ariceta G. et al., 2009;
Gomes A.M. et al., 2009; Clark W.F., 2012; Sengul Samanci N. et al., 2014]. In 1-2 days
after the beginning of a plasma exchange there was a decrease in level of average
weight molecules, and the period of an anury was stopped within 11 days against 15
days when treatment was carried out without plasma exchange [Davin J.C. et al., 2008,
2009]. Bambauer R. et al. (2011) also considered a plasma exchange as a choice
method in treatment of a hemolytico-uraemic syndrome.
Allocate a form of the atypical hemolytic-uraemic syndrome resulting from
activation of system of the complement leading to a thrombotic microangiopathy with
the chronic progressing current, leading to a renal failure, a stroke, pulmonary
bleedings, vessels thrombosis of a eyes retina [Noris M. et al., 2009]. Within a year 65%
of patients therefore immediate treatment with attraction of a plasma exchange is
required perish [Loirat C., Fremeaux-Bacchi V., 2011; Nakanishi T. et al., 2014].
According to the conclusion of advisory council of European Group the plasma
exchange is more effective, than transfusion of donor plasma. After a renal
transplantation there is a high risk (over 60%) of rejection of a transplant that also
demands application of a plasma exchange [Sanchez A.P., Ward D.M., 2012].

Hemolytic crises can arise also at deficiency of G-6-FD enzyme when at influence
of a number of provocative factors (some drugs, foodstuff, infections, etc.) can arise
severe intra-vascular hemolysis. By means of a plasma exchange with removal from
500 to 1500 ml of plasma for a session (for a course from 2500 to 5000 ml) it is possible
to liquidate quicker clinical manifestations of a disease and to restore laboratory
indicators [Gadzhiyev D.B., et al., 2005].

Hemolytic uremic syndrome 2


Intestinal infections has a definite value enterosorption preventing absorption
from the intestinal lumen enterogenous and bacterial toxins. Severe poisoning was
observed in some countries of Western Europe in the summer of 2011 caused by
serotype O104:H4 Escherichia coli. Secreted with Shiga-toxin it causes severe
enterocolitis with the development of hemolytic uremic syndrome, accompanied by
high mortality. Antibiotics were powerless in this case or, on the contrary, contributed
rise of endotoxemia. It became clear by the end of the epidemic, which forced to
abandon antibiotics. Using plasma exchange the early stages of disease contributed to
the rapid healing [Colic E. et al., 2011; Kreig L.S. et al., 2012; Ulrich S. et al., 2013 ; Van
Gemert L.M. et al., 2014]. J.T.Kielstein et al. (2012), summarizing the experience of
treating 631 patients in 84 hospitals in Germany, Sweden and the Netherlands came to
the same conclusion. I. Yildrim et al. (2010) by means of plasma exchange also have
been successful in the treatment of multiple organ failure on background
thrombocytopenia induced Salmonella enterica.

Colic E., Dieperink H., Titlestad K., Tepel M. Management of an acute outbreak of
diarrhoea-associated haemolytic uraemic syndrome with early plasma exchange in
adults from southern Denmark: an observational study // Lancet. 2011. Vol. 378,
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Kielstein J.T., Beutel G., Fieig S. et al. Best supportive care and therapeutic plasma
exchange with or without eculizumab in Shiga-toxin-producing E. coli O104:H4 induced
haemolytic-uraemic syndrome: an analysis of the German STEC-HUS registry //
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Kreig L.S., Marks S.D., Kim J.J. Shigatoxin-associated hemolytic uremic syndrome:
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Van Gemert L.M., Montemayor-Garcia C., Rose W.N. Dramatic improvement
immediately after plasma exchange in a patient with Shiga toxin-producing Escherichia
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Yildirim I., Ceyhan M., Bayrakci B. et al. A case report of thrombocytopenia-
associated multiple organ failure secondary to Salmonella enterica serotype Typhy
infection in a pediatric patient: successful treatment with plasma exchange // Ther.
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Ariceta G., Besbas N., Jonson S. et al. Guideline for the investigation and initial
therapy of diarrhea-negative hemolytic syndrome // Pediatr. Nephrol. 2009. Vol. 24,
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Bambauer R., Latza R., Schiel R. Therapeutic apheresis in the treatment of
hemolytic uremic syndrome in view of pathophysiological aspects // Ther. Apher. Dial.
2011. Vol. 15, 1. P. 10-19.
Clark W.F. Thrombotic microangiopathy: current knowledge and outcomes with
plasma exchange // Semin. Dial. 2012. Vol 25, 2. P. 214-219.
Davin J.C., Strain L., Goodship T.H.J. Plasma therapy in atypical haemolytic
syndrome: lessons from a family with a factor H mutation // Pediatr. Nephrol. 2008.
Vol. 23, 9. P. 1517-1521.
Davin J.C., Buter N., Groothoff J. et al. Prophylactic plasma exchange in CD46-
associated atypical haemolytic uremic syndrome // Pediatr. Nephrol. 2009. Vol. 24,
9. P. 1557-1560.
Forzley B.R., Sontrop J.M., Macnab J.J. et al. Treating TTP/HUS with plasma
exchange: a single centres 25-year experience // Brit. J. Haematol. 2008. Vol,143.
P. 100-106.
Gadzhiyev D.B. [The modes of carrying out a plasma exchange at haemolytic crises
at patients with hereditary deficiency of G-6-FD enzyme // Efferent Therapy (Rus).
2005. Vol. 11, No. 4. P. 68-71.
Gomes A.M., Ventura A., Almeida C. Hemolytic uremic syndrome as a primary
manifestation of acute human immunodeficiency virus infection // Clin. Nephrol. 2009.
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Loirat C., Fremeaux-Bacchi V. Atypical hemolytic uremic syndrome // Orphanet. J.
Rare dis. 2011. Vol. 6. P. 60-66.
Nakanishi T., Suzuki N., Kuragano T. et al. Current topics in therapeutic
plasmapheresis // Clin. Exp. Nephrol. 2014. Vol. 18, 1. P. 41-49.
Noris M., Remuzzi G. Atypical hemolytic-uremic syndrome // N. Engl. J. Med.
2009. Vol. 361. P. 1676-1687.
Sanchez J., Ward D.M. Therapeutic apheresis for renal disorders // Sem. Dial.
2012. Vol. 25. P. 119-131.
Sengul Samanci N., Ayer M., Gursu M. et al. Patrients treated with therapeutic
plasma exchange: a single center experience // Transfus. Apher. Sci. 2014. Vol. 51,
3. P. 83-89.

(Chapter from the book of Voinov V.A. Therapeutic apheresis, 2016, Bucuresti)

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