alexa Plasmapheresis should be Considered in Addition to Standard Therapy in Patients with Familial Chylomicronemia Syndrome | Open Access Journals
ISSN: 2329-9126
Journal of General Practice
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Plasmapheresis should be Considered in Addition to Standard Therapy in Patients with Familial Chylomicronemia Syndrome

Suleyman Baldane1*, Banu Bozkurt2 and Levent Kebapcilar1
1Department of Internal Medicine, Division of Endocrinology and Metabolism, Faculty of Medicine, Selcuk University, Konya, Turkey
2Department of Opthalmology, Faculty of Medicine, Selcuk University, Konya, Turkey
Corresponding Author : Suleyman Baldane MD
Department of Internal Medicine
Selcuk University, Konya, Postal code: 42075, Turkey
Tel: +903322244685
Fax: +903322412184
E-mail: [email protected]
Received May 19, 2015; Accepted June15, 2015; Published June 18, 2015
Citation: Baldane S, Bozkurt B, Kebapcilar L (2015) Plasmapheresis should be Considered in Addition to Standard Therapy in Patients with Familial Chylomicronemia Syndrome. J Gen Practice S1:i004. doi:10.4172/2329-9126.S1i001
Copyright: © 2015 Baldane S, et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
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Abstract

A 52-year-old man was referred to our clinic for management of severe hypertriglyceridemia diagnosed five years previously as a result of routine blood work. His medical history was also significant for type 2 diabetes mellitus. His hypertriglyceridemia had been poorly controlled, because of medication noncompliance. He did not use any medication for his severe hypertriglyceridemia since one year. The patient did not smoke or consume alcohol. He was asymptomatic and had no history of abdominal pain or pancreatitis. There was no evidence of abdominal distension, rebound tenderness or guarding. There was only family history of dyslipidemia. The patient's father and brother both were diagnosed with hyperlipidemia.

Keywords
Severe hypertriglyceridemia; Familial chylomicronemia; Plasmapheresis
Introduction
At the time of admission, the patient's body weight was 85 kg and height was 1.72 m (body mass index was 28.8 kg/m2) with a waist circumference of 96 cm. His blood pressure was 130/70 mmHg. No other abnormalities were noted on physical examination. During sampling, his blood was found to be highly viscous and when blood was drawn, it was noted to be milky (Figure 1).
Initial laboratory investigations were fasting serum total cholesterol 1126 mg/dl (Normal Range (NR): 130-200), HDL cholesterol 21 mg/dl (NR: 40-60), triglycerides 5452 mg/dl (NR: 40-200) and direct LDL cholesterol 277 mg/dl (NR: 50-100). Fasting glucose was 337 mg/dl (NR: 70-100) and glycosylated hemoglobin 13.6% (NR: 4-6). Thyroidstimulating hormone, liver transaminase, amylase, lipase, blood urea nitrogen and creatinine levels were all normal, and urine protein test was negative. Ophthalmological examination revealed lipemia retinalis. Due to extremely high triglyceride levels, the appearance of a milky white retinal arteries and veins, along with a salmon-colored retinal background a diagnosis of lipemia retinalis was made (Figure 2).
Patients with severe primary hypertriglyceridemi to (type V hyperlipoproteinemia; Mixed hypertriglyceridemia; Fredrickson's classification) was diagnosed. We started fat-restricted diet, fibrate, fish oil, insulin dextrose infusion and subcutaneous heparin treatment. Within five days the patient was asymptomatic and his triglyceride level had much more increased to 8912 mg/dl. Accordingly, the decision was made to initiate plasmapheresis to acutely lower the serum TG level. No complications developed during or after the procedures. After six days, triglyceride levels were modestly improved at 4726 mg/dl, and 10 days later his triglyceride was 1226 mg/dl. The plasmapheresis treatment was ceased after two sessions.
Fasting chylomicronemia (type V hyperlipoproteinemia; Mixed hypertriglyceridemia; Fredrickson's classification) is characterized by triglyceride levels above the 99th percentile in association with a creamy plasma supernatant and cloudy infranatant due to increases in chylomicrons and VLDL. Patients with severe hypertriglyceridemia (>1000 mg/dL) may develop chylomicronemia syndrome. These patients may manifest short-term memory loss, abdominal pain and/or pancreatitis, eruptive xanthoma and lipemia retinalis. Majority of the patients have secondary form including some other dyslipidemia types (e.g. familial hypertriglyceridemia due to partial lipoprotein lipase (LPL) deficiency) that are exacerbated by some factors such as poor controlled diabetes or utilization of drugs that elevate triglyceride levels. In addition there is primary form of type V hyperlipoproteinemia, in which there is not a deficiency of LPL, nor its ligand apo CII. The actual cause of this disorder is not clearly known; however, apo E4, which acts as a ligand for hepatic chylomicron and VLDL remnant receptors, may play role in this disorder.
Fasting chylomicronemia can be diagnosed by confirming the presence of chylomicrons and excess VLDL on agarose gel electrophoresis or ultracentrifugal analysis. A simple technique is to refrigerate plasma overnight and examine the specimen for a creamy supernatant from chylomicrons and a turbid VLDL-rich infranatant.
The initial treatment for severe hypertriglyceridemia should be lifestyle therapy; a combination of diet modification and drug therapy may also be considered. Standard therapeutic measures are based on the use of lipid-lowering agents (fenofibrate, gemfibrozil, niacin, W-3 fatty acids), and insulin in diabetic patients. In patients with severe hypertriglyceridemia, a fibrate should be used as a first-line agent. Other treatment options include insulin and heparin. Insulin can decrease triglyceride levels by increasing LPL activity, whereas heparin can do it by increasing release of endothelial LPL.
However, when standard medical therapies have failed, nonpharmacological approaches based upon the removal of triglycerides with therapeutic plasmapheresis can also provide benefit to patients, with severe hypertriglyceridemia to reduce triglycerides. Plasmapheresis is the fastest way of decreasing triglyceride levels. There is not a current guideline that recommends initiation time of plasmapheresis, its frequency and target triglyceride levels; however, plasmepheresis has been shown to be clearly beneficial in patients with hypertriglyceridemic pancreatitis. Plasmapheresis treatment should be planned individually depending on patient’s clinical status. Since plasmapheresis remains the quickest method to reduce the triglyceride levels, early initiation results in improved tissue perfusion and can prevent end-organ complications. Therefore, in patients with severe hypertriglyceridemia such as familial chylomicronemia, plasmapheresis should be considered in addition to standard therapy.

 

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