DALI low-density lipoprotein apheresis in homozygous and heterozygous familial hypercholesterolemic patients using low-dose citrate anticoagulation.
The purpose of this study was to clarify the efficacy and safety of direct adsorption of lipoprotein low-density lipoprotein apheresis (DALI LDL apheresis) in patients with severe homozygous and heterozygous familial hypercholesterolemia who showed minor adverse effects during treatment with the usual DALI configuration (AC 1:20) through the use of a new system with low-dose citrate anticoagulation (AC 1:40) developed in order to minimize citrate-related adverse effects. Serum total cholesterol and LDL-cholesterol (LDL-C) showed a decrease of 57% to 61%, and 62% to 67%, respectively, in the 2 patients. Serum lipoprotein (a) (Lp[a]) was higher in the homozygous patient (Patient 1: MD) and within the normal range in the heterozygous patient (Patient 2: ES). In the former, Lp(a) was reduced by 52%. Serum high-density lipoprotein cholesterol (HDL-C) showed a statistically insignificant acute reduction: 15% to 19%. The observed reduction is mainly related to the well-known effect of hemodilution. The cardiovascular risk (total cholesterol/HDL-C) was reduced in both patients (46% to 54%) as expected. Serum triglycerides were reduced by 33% to 49%. The mean blood volume processed per session was 7,600 ml. Fifteen treatments for each patient have successfully been completed without the appearance of any clinically significant subjective and objective symptoms related to treatment with the new system.
Stefanutti
C
C
Dipartimento di Terapia Medica, Università degli Studi di Roma La Sapienza, Rome, Italy. plasmaferesi@tin.it
Di Giacomo
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S
Di Caro
M
M
Vivenzio
A
A
Musca
A
A
D000293:Adolescent; D000327:Adsorption / Q000187:drug effects*; D000328:Adult; D000925:Anticoagulants / Q000097:blood* / Q000627:therapeutic use*; D001781:Blood Component Removal / Q000009:adverse effects* / Q000379:methods; D019343:Citric Acid / Q000097:blood* / Q000627:therapeutic use*; D005260:Female; D006579:Heterozygote; D006720:Homozygote; D006801:Humans; D006938:Hyperlipoproteinemia Type II / Q000097:blood* / Q000628:therapy*; D008077:Lipoproteins, LDL / Q000097:blood*; D008297:Male; D016896:Treatment Outcome
D000925:Anticoagulants; D008077:Lipoproteins, LDL; D019343:Citric Acid