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Harangi et al.                                                                                                                                                           LDL apheresis or PCSK9 inhibition, or both?































           Figure 3: LDL-C levels during the various treatment strategies. The oral treatment was continued during treatment with PCSK9 and LDL
           apheresis. LDL-C: low density lipoprotein cholesterol; PCSK9: proprotein convertase subtilisin/kexin type 9
           Lp(a) reduction. It must be noted that from the initiation   treatment may give hope for FH patients whose quality
           of the apheresis treatments regular carotid ultrasound   of life and life expectancies are limited because of the
           examinations did not show any progression and CAD   ineffective or not tolerated lipid lowering treatment.
           was not diagnosed.
                                                              Selective  LDL apheresis  or PCSK9 inhibition?
           One hundred  and forty mg biweekly  administrated   Sometimes we have to combine them.
           evolocumab can reduce  the LDL-C  level  by 66%.
                                                         [15]
           PCSK9  inhibition  also  results  in  significant  (18-  Authors’ contributions
           20%)  reductions  in  plasma  Lp(a).  Unique  efficacy  of   Concept, data analysis, and manuscript preparation:
           evolocumab  in LDL-C  reduction  could  be observed   M. Harangi
           in our case.  However,  LDL  apheresis treatment  is   Data acquisition, and literature search: L. Juhász,
           superior in Lp(a) reduction.                       B. Nádró
                                                              Manuscript  editing,  and  manuscript  review:  J.  Balla,
           So, which treatment strategy should we choose in our   G. Paragh
           severe heterozygous  FH patients? Would selective
           LDL apheresis or PCSK9 inhibition be better? Decades   Financial support and sponsorship
           of clinical experience,  results of long-term  follow-  This research was supported by a grant from
           up studies [16,17]   and  unique  Lp(a)  reducing  efficacy   the  National  Research,  Development  and
           supports selective  LDL apheresis.  Furthermore,   Innovation (NFKI) (OTKA 115723) and by the
           LDL  apheresis  treatment  significantly  improves  the   GINOP-2.3.2-15-2016-00005 Project.  The  project
           oxidative,  inflammatory,  rheological  and  hemostasis   is  co-financed  by  the  European  Union  under  the
           status of the treated patients.  Nevertheless, superior   European Regional Development Fund.
                                     [18]
           efficacy of PCSK9 inhibitors in LDL-C reduction is in
           its favor. Both treatment options are well tolerated and   Conflicts of interest
           safe, even in combination as was demonstrated in our   There are no conflicts of interest.
           case. It must be noted that further observations  are
           needed to assess long-term side effects of this therapy.  Patient consent
                                                              The patient gave her consent form.
           This  case  report  highlights  the  difficulties  of  the
           treatment of severe heterozygous FH and proves that   Ethics approval
           in some special cases LDL  apheresis and PCSK9     The work is conforming to the guiding principles of
           inhibition  can be combined  successfully and safely   the Declaration of Helsinki, and our patient gave
           resulting in extreme LDL-C reduction. Combined     informed consent.
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