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 Table of Contents  
CASE REPORT
Year : 2021  |  Volume : 7  |  Issue : 1  |  Page : 63-65

Severe hypertriglyceridemia with recurrent pancreatitis treated with therapeutic plasma exchange


Department of Cardiology, Sri Aggarsain International Hospital, New Delhi, India

Date of Submission07-Oct-2020
Date of Decision01-Mar-2021
Date of Acceptance12-Mar-2021
Date of Web Publication24-Apr-2021

Correspondence Address:
Anshul Kumar Jain
Department of Cardiology, Sri Aggarsain International Hospital, New Delhi
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jpcs.jpcs_99_20

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  Abstract 


Severe hypertriglyceridemia (HTG) is associated with an increased risk of cardiovascular disease and acute pancreatitis. We report the case of a 34-year-old female admitted with complaints of recurrent pancreatitis and triglyceride (TG) levels above 995 mg/dl. The patient was started on therapeutic plasma exchange (TPE) to achieve a rapid fall (78%) in serum TG levels. We conclude that TPE is a safe and quick therapeutic treatment modality for patients with HTG-induced recurrent acute pancreatitis who fail to respond to conventional therapy.

Keywords: Acute pancreatitis, dyslipidemia, hypertriglyceridemia, therapeutic plasma exchange


How to cite this article:
Jain AK, Bedi N, Garg A. Severe hypertriglyceridemia with recurrent pancreatitis treated with therapeutic plasma exchange. J Pract Cardiovasc Sci 2021;7:63-5

How to cite this URL:
Jain AK, Bedi N, Garg A. Severe hypertriglyceridemia with recurrent pancreatitis treated with therapeutic plasma exchange. J Pract Cardiovasc Sci [serial online] 2021 [cited 2021 Jun 22];7:63-5. Available from: https://www.j-pcs.org/text.asp?2021/7/1/63/314487




  Introduction Top


Severe hypertriglyceridemia (HTG) (>1000 mg/dl) is associated with an increased risk of atherosclerotic cardiovascular disease and pancreatitis.[1],[2] HTG results from overproduction and impaired clearance of triglyceride (TG)-rich lipoprotein and chylomicrons. The usual treatment regimen for HTG includes dietary restriction and lipid-lowering drugs such as fibrates, nicotinic acid, omega-3 fatty acid, and medium-chain TGs, but these have a slow and modest effect on reducing the TG levels, especially in severe HTG. Current data suggest that therapeutic plasma exchange (TPE) is an option to treat severe HTG with recurrent acute pancreatitis in selected patients.


  Case Report Top


A 34-year-old female, diabetic and obese patient, was brought to the emergency of the hospital with excruciating pain in the epigastric region, recurrent vomiting, and fever for the past 3 days. She had a past history of recurrent pancreatitis attributed to very high serum TG levels (above 1000 mg/dl; maximum levels 6789 mg/dl in past), requiring multiple admissions. She was given atorvastatin 20 mg and fenofibrate 145 mg to treat the same in addition to dietary restriction and diabetic control. She had no history of cholelithiasis or alcohol intake. Physical examination was unremarkable except for sinus tachycardia, fever, and epigastric tenderness. Her blood sugar was 299 mg/dl and ketones were absent. She was kept nil orally and managed with intravenous fluids, insulin infusion, antibiotics, and other supportive drugs. She had raised serum amylase and lipase and had TG levels of 995 mg/dl on admission [Table 1]. Contrast-enhanced computed tomography scan of the abdomen confirmed the diagnosis of multiple foci of necrosis in the body of the pancreas [Figure 1] and [Figure 2]. Echocardiography revealed a normal ejection fraction of 64%.
Figure 1: Contrast-enhanced computed tomography (axial section). Arrow indicates nonenhancing areas (necrosis) in the body of the pancreas with surrounding peripancreatic fat stranding, suggestive of acute necrotizing pancreatitis.

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Figure 2: Contrast-enhanced computed tomography (coronal reformat section). Arrow indicates acute necrotizing pancreatitis with surrounding peri-pancreatic fluid collections in left anterior pararenal space.

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Table 1: Lipid profile at the end of each session of therapeutic plasma exchange

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Owing to the recurrent history of pancreatitis and previous history of poor response of the TG levels to drug intake, we decided to proceed with TPE to achieve a rapid decrease in TG levels. Alternate-day TPE was planned. Three sessions of centrifugal TPE were performed on the ComTec cell separator (Fresenius Kabi, Germany). In each session, 1.5 total plasma volume was replaced with albumin solution and fresh frozen plasma. Following the first plasma exchange, the TG levels showed a marked 66% decrease from 995 to 334 mg/dl and the patient started improving clinically. However, the next day (without TPE), the TG levels rose to 579 mg/dl. On day 3, the second session was performed and the TG levels fell to 323 mg/dl. In view of the rapid rise of TG levels in the alternate-day regime, we changed over to the consecutive day TPE. In addition, drugs such as atorvastatin, fenofibrate, and high-dose omega-3 fatty acids were added to the patient's regime. The third session done on the next day resulted in a lowering of TG levels to 214 mg/dl. Further planned session of TPE had to be canceled due to derangement of the patient's coagulation profile (rise in activated partial thromboplastin time more than three times the control – 71.9 s) No other complications were reported in any of the TPE sessions. Overall 78% decrease was seen in the TG levels at the end of three sessions. TG levels of the patient maintained a constant level in the following days and were 310 mg/dl at the time of discharge. Thirteen days after her TPE, her TG levels had increased to 488 mg/dl. She was then counseled for intensive diabetic control and diet modification. After 4 months of dedicated therapy, her TG levels are down to 269 mg/dl. She has had no further episodes of pancreatitis for the past 4 months.


  Discussion Top


TG levels above 1000 mg/dl are a major risk factor for recurrent pancreatitis.[2] It is hypothesized that the presence of nongenetic stressors such as uncontrolled diabetes, thyroid disease, nephrotic syndrome, multiple myeloma, or some drugs is required to express the severe HTG phenotype in genetically susceptible individuals. The obstruction of the local capillary bed by the circulating chylomicrons leads to pancreatic ischemia. In addition, it is probable that the pancreatic lipase is mislocalized to plasma causing partial hydrolysis of TG to free fatty acids in the chylomicrons leading to cytotoxic injury and the release of inflammatory cytokines.[2],[3]

The management of acute episodes of pancreatitis consists of the usual strategy: keeping the patient nil orally, intravenous fluids, pain management, and antibiotics. For HTG, insulin and heparin infusion is supposed to be beneficial in addition to therapy with fenofibrates, omega-3–fatty acids, and niacin.[1],[2],[3],[4],[5] Plasmapheresis is reserved for the cases with familial chylomicronemia syndrome (FCS), pregnancy (where estrogen levels may further aggravate the HTG), or in conditions where the patient presents with recurrent pancreatitis nonresponsive to usual management strategies.[1],[5],[6],[7] Although TPE is a rapid and an efficient method of lowering TG and results in early resolution of symptoms due to interruption of the underlying pathogenic mechanism, the data about the long-term effectiveness of this therapy are still lacking (class III indication as per the American Society for Apheresis).[6] It should be performed till the TG levels decrease <500 mg/dl. The TG levels tend to rise again over variable periods depending on the individual patient if not given proper adjuvant medical therapy. In our case also, the daily TPE regime succeeded in maintaining lower TG levels. Plasma exchange may be associated with certain complications such as coagulopathy, allergic reaction, and infection. In our case, the derangement of coagulation profile forced us to abandon the further two planned cycles.

The prevention of the future episodes of pancreatitis requires strict lifestyle changes including adequate exercise, achievement of ideal weight, elimination of alcohol, minimization of sugar and fat intake, strict control of diabetes, and achievement of euthyroid state. In patients with FCS, it is advisable to substitute standard fats with medium-chain TG as these are not metabolized via chylomicrons.[8] Offending drugs must be changed. Long-term treatment of HTG should continue with combination of fenofibrates, high-dose omega-3 fatty acids, and niacin as per need. For those who fail to respond to the conventional therapy, certain experimental therapies are available which include the use of antisense oligonucleotide inhibitor of apolipoprotein C-III and monoclonal antibody against angiopoietin-like 3 with variable results.[9],[10]


  Conclusion Top


In very selected patients of HTG with recurrent pancreatitis, TPE appears to be a safe and useful therapeutic tool to achieve a rapid reduction in the TG levels and achieve marked clinical improvement.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Hegele RA, Ginsberg HN, Chapman MJ, Nordestgaard BG, Kuivenhoven JA, Averna M, et al. The polygenic nature of hypertriglyceridaemia: Implications for definition, diagnosis, and management. Lancet Diabetes Endocrinol 2014;2:655-66.  Back to cited text no. 1
    
2.
Carr RA, Rejowski BJ, Cote GA, Pitt HA, Zyromski NJ. Systematic review of hypertriglyceridemia-induced acute pancreatitis: A more virulent etiology? Pancreatology 2016;16:469-76.  Back to cited text no. 2
    
3.
Valdivielso P, Ramírez-Bueno A, Ewald N. Current knowledge of hypertriglyceridemic pancreatitis. Eur J Intern Med 2014;25:689-94.  Back to cited text no. 3
    
4.
Twilla JD, Mancell J. Hypertriglyceridemia-induced acute pancreatitis treated with insulin and heparin. Am J Health Syst Pharm 2012;69:213-6.  Back to cited text no. 4
    
5.
Berberich AJ, Ziada A, Zou GY, Hegele RA. Conservative management in hypertriglyceridemia-associated pancreatitis. J Intern Med 2019;286:644-50.  Back to cited text no. 5
    
6.
Padmanabhan A, Connelly-Smith L, Aqui N, Balogun RA, Klingel R, Meyer E, et al. Guidelines on the use of therapeutic apheresis in clinical practice-Evidence-based approach from the writing committee of the American Society for Apheresis: The eighth special issue. J Clin Apher 2019;34:171-354.  Back to cited text no. 6
    
7.
Kadikoylu G, Yukselen V, Yavasoglu I, Coşkun A, Karaoglu AO, Bolaman Z. Emergent therapy with therapeutic plasma exchange in acute recurrent pancreatitis due to severe hypertriglyceridemia. Transfus Apher Sci 2010;43:285-9.  Back to cited text no. 7
    
8.
Mizushima T, Ochi K, Matsumura N, Ichimura M, Ishibashi T, Tsuboi K, et al. Prevention of hyperlipidemic acute pancreatitis during pregnancy with medium-chain triglyceride nutritional support. Int J Pancreatol 1998;23:187-92.  Back to cited text no. 8
    
9.
Wierzbicki AS, Viljoen A. Anti-sense oligonucleotide therapies for the treatment of hyperlipidaemia. Expert Opin Biol Ther 2016;16:1125-34.  Back to cited text no. 9
    
10.
Yang X, Lee SR, Choi YS, Alexander VJ, Digenio A, Yang Q, et al. Reduction in lipoprotein-associated apoC-III levels following volanesorsen therapy: Phase 2 randomized trial results. J Lipid Res 2016;57:706-13.  Back to cited text no. 10
    


    Figures

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