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 Table of Contents  
CASE REPORT
Year : 2023  |  Volume : 10  |  Issue : 1  |  Page : 36-38

Acute pancreatitis due to massive hemolysis in a child with glucose-6-phosphate dehydrogenase deficiency: A case Report


1 Department of Critical Care Medicine, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India
2 Department of Microbiology, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India

Date of Submission05-Sep-2022
Date of Decision29-Nov-2022
Date of Acceptance11-Dec-2022
Date of Web Publication20-Jan-2023

Correspondence Address:
Prof. Banani Poddar
Department of Critical Care Medicine, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow - 226 014, Uttar Pradesh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jpcc.jpcc_76_22

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  Abstract 


The incidence of massive intravascular hemolysis causing pancreatitis is 25%; however, it is an often missed entity. Proinflammatory and immunoregulatory cytokines during hemolysis along with heme-induced neutrophil activation, chemoattraction, and disturbance in microcirculation are suggested as causative factors. We describe a case of glucose-6-phosphate dehydrogenase deficiency in a child which was latent through his childhood, triggering a hemolytic episode induced by Leptospira infection. In a patient with acute hemolysis, presenting with severe epigastric pain, one should therefore be aware of the possibility of the occurrence of pancreatitis.

Keywords: Acute pancreatitis, glucose-6-phosphate dehydrogenase deficiency, hemolysis, leptospirosis


How to cite this article:
Kayarat B, Gutte S, Pal S, Poddar B, Azim A, Gurjar M. Acute pancreatitis due to massive hemolysis in a child with glucose-6-phosphate dehydrogenase deficiency: A case Report. J Pediatr Crit Care 2023;10:36-8

How to cite this URL:
Kayarat B, Gutte S, Pal S, Poddar B, Azim A, Gurjar M. Acute pancreatitis due to massive hemolysis in a child with glucose-6-phosphate dehydrogenase deficiency: A case Report. J Pediatr Crit Care [serial online] 2023 [cited 2023 Feb 3];10:36-8. Available from: http://www.jpcc.org.in/text.asp?2023/10/1/36/368234




  Introduction Top


The etiology of acute pancreatitis, besides ethanol abuse, and biliary tract disease include other relatively rare causes such as drugs, infections, hyperparathyroidism, hyperlipidemia, shock, trauma, and pregnancy. Literature cites that massive intravascular hemolysis could lead to acute pancreatitis with a prevalence as high as 20%; however, it is an often missed and ignored entity.[1],[2],[3] We, therefore, report a case of acute pancreatitis as a consequence of massive intravascular hemolysis, complicated by leptospira infection in glucose-6-phosphate dehydrogenase (G6PD) deficient child and thereby dwell on the possible causal mechanisms.


  Case Report Top


A 9-year-old developmentally normal child had presented with complaints of high-grade fever along with vomiting for 1 week, following which he had a history of the passage of cola-colored urine. On admission to the intensive care unit, his complete blood count showed a hemoglobin level of 5.4 g/dl with elevated total white blood cell (WBC) counts (42,000/cu mm). The corrected total leukocyte count was 25,000/cu mm, 58 nucleated red blood cell (RBC)/WBC, differential count (neutrophils – 84%; lymphocytes – 10%), and platelets were within normal limits, but his reticulocyte count was 4.6%. Serum lactate dehydrogenase was raised (7094 U/L). Urinalysis showed the presence of hemoglobin in the urine. Serum electrolytes were normal. Total serum bilirubin was 10.2 mg/dl with indirect bilirubin of 6 mg/dl; however, liver enzymes were within normal limits. The blood urea nitrogen on admission was 98 mg/dl and serum creatinine was 5 mg/dl. His urine output was around 0.2–0.3 ml/kg/h.

The peripheral blood film showed the presence of polychromasia along with basophilic stippling. Hemoglobin analysis excluded hemoglobinopathies like thalassemia. Screening for G6PD deficiency was carried out. Enzyme levels were low – 1.94 U/g Hb (normal: 4.9–15 U/g Hb), suggestive of G6PD deficiency. The tropical fever (dengue, scrub typhus, malaria, and leptospira) workup showed the presence of IgM antibodies for leptospira infection. No other drugs that could precipitate G6PD deficiency or likely oxidative stress were administered in our patient. Hence, we postulated that G6PD deficiency could be the cause of the acute hemolysis, triggered by the high oxidative stress provoked by the Leptospira infection. The patient was managed with urgent blood transfusion of packed RBCs, appropriate hydration, and broad-spectrum antibiotics. Antipyretic and antiemetic medications were given symptomatically.

The next day he started experiencing escalating epigastric pain. Serum amylase was 900 U/L (normal – 16–108 U/L) and serum lipase was 3300 U/L (normal 10–150 U/L), consistent with acute pancreatitis. Ultrasound abdomen showed the presence of a bulky head of pancreas. The gallbladder was normal with no presence of gallstones. Serum calcium level was 7.4 mg/dL and triglycerides were 265 mg/dL. The patient was not on any medication known to cause pancreatitis. Hence, a diagnosis of hemolysis-induced pancreatitis was made. He was managed symptomatically with analgesia and there was a good resolution of symptoms.

In view of persistent uremia (110 mg/dl) and likely developing uremic encephalopathy, the child required one session of hemodialysis. The patient responded well to the treatment. Follow-up investigations showed decreasing levels of serum bilirubin and creatinine. Urine examination showed no RBCs and absent hemoglobinuria with visible improvement of high-colored urine to normal straw color. The child was discharged in a stable condition


  Discussion Top


Acute pancreatitis has been reported in retrospective case studies in at least 25% of patients presenting with severe hemolysis caused by various etiologies such as microangiopathic hemolytic anemia, toxemia, G6PD deficiency, septic abortion, malaria, Wilson's disease, hypophosphatemia, or autoimmune hemolytic anemia.[2] In this case, the G6PD deficiency was latent through his childhood, and the intravenous hemolysis was possibly triggered by Leptospira infection. The episodic hemolysis that is characteristic of G6PD deficiency is usually caused by exposures that generate oxidant stress. Leptospirosis is one such infection known to increase reactive oxygen species levels and thereby affecting the oxidant/antioxidant balance.[4]

Pancreatitis is usually diagnosed in the presence of elevated serum amylase level with at least one of the following features: clinical signs (abdominal pain and distension and epigastric tenderness) and ultrasound findings (swelling of the pancreas). In our case, the patient had elevated amylase levels of 900 U/L with epigastric tenderness. Ultrasonography also revealed swelling of the head of the pancreas.

Animal models have demonstrated hemolysis as a cause of pancreatitis.[5] The precise mechanisms underlying the pathogenesis of hemolysis-induced acute pancreatitis have always remained unclear. It is the inflammatory cytokine response that is responsible for the mortality and morbidity associated with any acute pancreatitis. It has been demonstrated in mice that massive intravascular hemolysis leads to the release of large amounts of free heme, which sometimes exceeds the binding capacity of hemopexin overwhelming the heme aids in the formation of reactive oxygen radicals and causes leukocyte recruitment. Experimental animal models also demonstrated edema formation and intense accumulation of neutrophils even in the pleural cavities.[6] There are also studies demonstrating the protective effects of HO-1 against severe pancreatitis further supporting the postulated mechanism.[7]

Massive hemolysis also leads to activation of the coagulation cascade forming microthrombi and damaging the vascular integrity of pancreatic microvasculature. Clearance of fibrinogen-fibrin split products is also reduced during hemolysis due to the blockage of the reticuloendothelial system by cell detritus. It is also seen that free heme inhibits endothelium-derived relaxing factors, causing local vasoconstriction further promoting organ dysfunction.[8] Most of the cases described in the literature developed complications of acute renal failure and intravascular coagulation.[2],[3]

In our patient, in view of developing uremic encephalopathy, dialysis was initiated. It could be a part of the spectrum of Leptospirosis, but it is also a well-known complication of massive hemolysis. Acute pancreatitis associated with Leptospira infection usually presents in the 1st week of illness. In our case, pancreatitis developed in the 2nd week of illness.[9] Even though renal failure per se may cause a rise in serum amylase level, this rarely exceeds more than 2.5 times the upper limit of normal, and is usually not associated with clinical or ultrasound signs of pancreatitis. A combination of these and associated factors such as anemia and tissue hypoxia would have further contributed to the development of injury to the pancreas.

In a patient with acute hemolysis, presenting with severe epigastric pain, one should be aware of the possibility of the occurrence of pancreatitis. This association may not have been emphasized previously possible due to the low incidence of massive hemolysis.

Declaration of patient consent

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

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Saruc M, Yuceyar H, Turkel N, Ozutemiz O, Tuzcuoglu I, Yuce G, et al. An experimental model of hemolysis-induced acute pancreatitis. Braz J Med Biol Res 2003;36:879-86.  Back to cited text no. 1
    
2.
Druml W, Laggner AN, Lenz K, Grimm G, Schneeweiss B. Pancreatitis in acute hemolysis. Ann Hematol 1991;63:39-41.  Back to cited text no. 2
    
3.
Przybyłowski J, Kłos M, Kisiel E, Mierzwa-Slefarska H. Glucose-6-phosphate dehydrogenase deficiency in erythrocytes observed in two cases. Pol Arch Med Wewn 1993;90:72-7.  Back to cited text no. 3
    
4.
Araújo AM, Reis EA, Athanazio DA, Ribeiro GS, Hagan JE, Araujo GC, et al. Oxidative stress markers correlate with renal dysfunction and thrombocytopenia in severe leptospirosis. Am J Trop Med Hyg 2014;90:719-23.  Back to cited text no. 4
    
5.
Saruç M, Yuceyar H, Turkel N, Ozutemiz O, Tuzcuoglu I, Ayhan S, et al. The role of heme in hemolysis-induced acute pancreatitis. Med Sci Monit 2007;13:BR67-72.  Back to cited text no. 5
    
6.
Graça-Souza AV, Arruda MA, de Freitas MS, Barja-Fidalgo C, Oliveira PL. Neutrophil activation by heme: Implications for inflammatory processes. Blood 2002;99:4160-5.  Back to cited text no. 6
    
7.
Zhang FH, Sun YH, Fan KL, Dong XB, Han N, Zhao H, et al. Protective effects of heme oxygenase-1 against severe acute pancreatitis via inhibition of tumor necrosis factor-α and augmentation of interleukin-10. BMC Gastroenterol 2017;17:100.  Back to cited text no. 7
    
8.
Anand IS, Chandrashekhar Y, Wander GS, Chawla LS. Endothelium-derived relaxing factor is important in mediating the high output state in chronic severe anemia. J Am Coll Cardiol 1995;25:1402-7.  Back to cited text no. 8
    
9.
Maier A, Kaeser R, Thimme R, Boettler T. Acute pancreatitis and vasoplegic shock associated with leptospirosis – A case report and review of the literature. BMC Infect Dis 2019;19:395.  Back to cited text no. 9
    




 

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