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Year : 2016  |  Volume : 19  |  Issue : 2  |  Page : 101-103

Autoimmune pancreatitis with pancytopenia: A rare occurrence

MIOT Advanced Centre for Gastrointestinal and Liver Diseases, MIOT Hospitals, Chennai, Tamil Nadu, India

Date of Web Publication12-Jul-2016

Correspondence Address:
Arulprakash Sarangapani
MIOT Advanced Centre for Gastrointestinal and Liver Diseases, MIOT Hospitals, Chennai - 600 089, Tamil Nadu
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DOI: 10.4103/1118-8561.186033

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Autoimmune pancreatitis (AIP) is considered a distinct type of pancreatitis with an autoimmune pathogenesis. Patients with AIP often develop extrapancreatic lesions such as biliary lesions, sialadenitis, retroperitoneal fibrosis, swelling of lymph nodes, chronic thyroiditis, and interstitial nephritis, suggesting that AIP may be a systemic disease. Moreover, several cases of immune thrombocytopenia and few with neutropenia complicated with AIP have been described. The pathogenesis of thrombocytopenia associated with AIP is still unclear; however, autoimmune processes are suggested. On the other hand, cases of pancytopenia complicated by AIP have not yet been reported. We herein report a very rare case of pancytopenia associated with AIP.

Keywords: Autoimmune pancreatitis, IgG4 disese, pancytopenia

How to cite this article:
Sarangapani A, Samal SC, Sahu MK, Chandy GM. Autoimmune pancreatitis with pancytopenia: A rare occurrence. Sahel Med J 2016;19:101-3

How to cite this URL:
Sarangapani A, Samal SC, Sahu MK, Chandy GM. Autoimmune pancreatitis with pancytopenia: A rare occurrence. Sahel Med J [serial online] 2016 [cited 2021 Jan 15];19:101-3. Available from: https://www.smjonline.org/text.asp?2016/19/2/101/186033

  Introduction Top

Autoimmune pancreatitis (AIP) results from infiltration of the pancreas and other organs by IgG4+ plasma cell resulting in both pancreatic and extrapancreatic manifestations. Pain is not a dominant symptom and in the presence of extrapancreatic symptoms, the diagnosis may not be obvious. A high index of suspicion is required to diagnose this condition, which is important considering the good response to steroids in many patients. We report a patient where the presentation was like a lymphoproliferative disorder with organomegaly, lymphadenopathy, and pancytopenia.

  Case Report Top

A 48-year-old female from South India presented to us with complaints of the postprandial nonradiating epigastric pain of moderate intensity accompanied by nausea and vomiting for past 2 months. She had lost 5 kg of her weight during this period. There was no history of fever and jaundice, and she had no significant past illnesses. On physical exam, she was found to have pallor, left axillary lymphadenopathy, and hepatosplenomegaly. Laboratory investigations revealed pancytopenia (hemoglobin - 7.4 g%, total leukocyte count - 3100/mm 3, platelet count –97,000/mm 3). Liver function tests showed elevated levels of serum alkaline phosphatase (361 IU/mL) and alanine aminotransferase (89 IU/mL) while the total and direct bilirubin levels were within normal limits. Serum gamma-glutamyl transpeptidase level was 577 IU/mL. Serum lipase and amylase levels were moderately elevated (lipase 540 IU/mL, amylase 400 IU/mL). Serum anti-neutrophil antibodies were positive. Abdominal ultrasound showed intrahepatic biliary dilatation with dilatation of common bile duct (8 mm), hepatosplenomegaly, and bulky pancreas.

Contrast enhanced computed tomography (CECT) of the abdomen showed a sausage shaped bulky hypoechoic pancreas [Figure 1]a. Bone marrow biopsy was done for pancytopenia, which showed a hyperplastic marrow. Axillary lymph node biopsy and immunohistochemical staining (BCL2 and CD20) showed reactive lymph node with no evidence of lymphoma. Endoscopic retrograde cholangiopancreatography (ERCP) was done which showed distal common bile duct stricture, and the pancreatic duct was thin and beaded in appearance [Figure 1]b and [Figure 1]c. AIP was suspected, and serum IgG4 levels were found to be was >815 mg%. Based on established criteria (HISORT), the patient was diagnosed as having AIP and was started on oral steroids.
Figure 1: (a) Contrast enhanced computed tomography of the abdomen shows a sausage shaped bulky hypoechoic pancreas. (b and c) Endoscopic retrograde cholangiopancreatography was done, which shows distal common bile duct stricture and the pancreatic duct was thin and beaded in appearance. (d) Normal endoscopic retrograde cholangiopancreatography

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She showed a good response to therapy and after 4 weeks, investigations showed normalization of serum alkaline phosphatase (88 IU/mL), total leukocyte count (7400/mm 3), and platelet counts (188,000/mm 3). Hemoglobin improved from 7.4 to 9.1 g%. Repeat ERCP was normal [Figure 1]d. She has been asymptomatic for the past 2 years.

  Discussion Top

AIP is an uncommon cause of chronic pancreatitis and was first described, in 1961, by Sarles et al., in a patient with pancreatitis and hypergammaglobulinemia.[1],[2] In 1991, Kawaguchi et al.[3] gave the first detailed description and described it as “lymphoplasmacytic sclerosing pancreatitis.” In 1995, Yoshida et al., reported a large series from Japan, and coined the term AIP.[4] The characteristic findings are:[5],[6],[7] (i) Diffuse enlargement of the pancreas on imaging; (ii) increased levels of serum gamma globulin or IgG4; (iii) diffusely irregular narrowing of the main pancreatic duct on ERCP; (iv) fibrotic changes with lymphocyte infiltration on histology; (v) occasional association with other extrapancreatic lesions; (vi) dramatic response to steroid therapy. Japan pancreas society described the diagnostic criteria based on imaging and serologic or histological findings.[8] A recent audit from the Mayo Clinic [9] has suggested an algorithm (HISORT) for diagnosis of AIP, which has a strong emphasis on clinical, serological, and histological factors with less weighing of radiological results. Treatment with corticosteroids often leads to resolution of pancreatic and extrapancreatic manifestations within weeks along with radiological improvement.[10]

AIP is considered a multisystem disorder, associated with extrapancreatic lesions. In a report from Mayo Clinic, extrapancreatic manifestations were described in 49% of cases, which included biliary strictures, sclerosing cholangitis, hilar or abdominal lymphadenopathy, and interstitial nephritis. Our patient had a presentation resembling lymphoproliferative disorder with pancytopenia. Bone marrow examination did not show myelodysplastic syndrome (MDS). The findings on CECT abdomen roused suspicion of AIP and were subsequently confirmed by elevated IgG4 levels and rapid response to steroids.

To date, many extrapancreatic lesions including immune thrombocytopenia have been reported in patients with AIP although concurrent development of anemia, neutropenia, and AIP seems extremely rare. The criteria for autoimmune neutropenia diagnosis are as follows: A decrease in the circulating absolute neutrophil count to <1500 µL −1 caused by serum anti-neutrophil antibodies.[11] Secondary autoimmune neutropenia is usually seen in systemic autoimmune disorders, infectious diseases, solid or hematological neoplasms, neurological diseases, posttransplantation, and with the use of certain drugs. The present case is considered to represent secondary autoimmune neutropenia accompanied by AIP because anti-neutrophil antibodies diminished after steroid therapy with the improvement of AIP symptoms. Systemic autoimmune reactions in AIP may induce the production of various autoantibodies including anti-neutrophil antibodies. The mechanisms of neutrophil destruction is unclear; however, anti-neutrophil antibodies can reportedly bind to the surface of granulocytes, followed by phagocytosis of sensitized granulocytes by macrophages, and finally resulting in the peripheral depletion of neutrophils.[12] Unfortunately, we failed to find unequivocal evidence of neutrophil destruction in the bone marrow clot specimen. Additional investigation is needed to clarify the cause of this phenomenon. In conclusion, AIP can be accompanied by autoimmune neutropenia; therefore, anti-neutrophil antibodies should be examined when a low neutrophil count is detected in patients with AIP. Steroid therapy is effective for both pathogeneses. This may be the first report of AIP with pancytopenia in the absence of MDS from India. This emphasizes the need for wider awareness among clinicians on the existence and protean manifestations of AIP. This is also vital from the perspective of effective patient management.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Sarles H, Sarles JC, Muratore R, Guien C. Chronic inflammatory sclerosis of the pancreas – An autonomous pancreatic disease? Am J Dig Dis 1961;6:688-98.  Back to cited text no. 1
Deshpande V, Mino-Kenudson M, Brugge W, Lauwers GY. Autoimmune pancreatitis: More than just a pancreatic disease? A contemporary review of its pathology. Arch Pathol Lab Med 2005;129:1148-54.  Back to cited text no. 2
Kawaguchi K, Koike M, Tsuruta K, Okamoto A, Tabata I, Fujita N, et al. Lymphopasmacytic sclerosing pancreatitis with cholangitis sclerosing pancreatitis with cholangitis: Variant of primary sclerosing cholangitis extensively involving pancreas. Hum Pathol 1991;22:387-95.  Back to cited text no. 3
Yoshida K, Toki F, Takeuchi T, Watanabe S, Shiratori K, Hayashi N. Chronic pancreatitis caused by an autoimmune abnormality. Proposal of the concept of autoimmune pancreatitis. Dig Dis Sci 1995;40:1561-8.  Back to cited text no. 4
Hamano H, Kawa S. Are there any other organs in which autoimmune pancreatitis-associated lesions remain to be identified? Intern Med 2006;45:883-4.  Back to cited text no. 5
Hirano K, Kawabe T, Yamamoto N, Nakai Y, Sasahira N, Tsujino T, et al. Serum IgG4 concentrations in pancreatic and biliary diseases. Clin Chim Acta 2006;367:181-4.  Back to cited text no. 6
Uchida K, Okazaki K, Konishi Y, Ohana M, Takakuwa H, Hajiro K, et al. Clinical analysis of autoimmune-related pancreatitis. Am J Gastroenterol 2000;95:2788-94.  Back to cited text no. 7
Okazaki K, Kawa S, Kamisawa T, Naruse S, Tanaka S, Nishimori I, et al. Clinical diagnostic criteria of autoimmune pancreatitis: Revised proposal. J Gastroenterol 2006;41:626-31.  Back to cited text no. 8
Chari ST, Smyrk TC, Levy MJ, Topazian MD, Takahashi N, Zhang L, et al. Diagnosis of autoimmune pancreatitis: The Mayo Clinic experience. Clin Gastroenterol Hepatol 2006;4:1010-6.  Back to cited text no. 9
Kojima E, Kimura K, Noda Y, Kobayashi G, Itoh K, Fujita N. Autoimmune pancreatitis and multiple bile duct strictures treated effectively with steroid. J Gastroenterol 2003;38:603-7.  Back to cited text no. 10
Bux J, Kissel K, Nowak K, Spengel U, Mueller-Eckhardt C. Autoimmune neutropenia: Clinical and laboratory studies in 143 patients. Ann Hematol 1991;63:249-52.  Back to cited text no. 11
Shastri KA, Logue GL. Autoimmune neutropenia. Blood 1993;81:1984-95.  Back to cited text no. 12


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