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Occult T-Cell Acute Lymphoblastic Leukemia Presenting as Obstructive Pancreatitis

Kraynik, Jessica*; Noel, James M.; Bluefeather, Sara C.

Journal of Pediatric Gastroenterology and Nutrition: May 2015 - Volume 60 - Issue 5 - p e43–e45
doi: 10.1097/MPG.0000000000000222
Case Reports

*Pediatric Residency Program, University of Colorado, Denver

Department of Pediatric Gastroenterology

Department of Pediatrics, University of New Mexico, Albuquerque.

Address correspondence and reprint requests to Jessica Kraynik, MD, Pediatrics Residency, 13123 E 16th Ave, #B158, Aurora, CO 80045-7106 (e-mail: jessica.kraynik@childrenscolorado.org).

Received 30 June, 2013

Accepted 17 October, 2013

The authors report no conflicts of interest.

The incidence of pancreatitis is 3.6 to 13.2 per 100,000 children (1). Acute pancreatitis is often diagnosed when a child exhibits 2 of the following 3 criteria: epigastric pain, elevated serum amylase or lipase, or cross-sectional abdominal imaging findings indicating pancreatic inflammation (2). In pediatrics, the most common causes of acute pancreatitis are biliary obstruction, medications, idiopathic etiologies, systemic diseases, and trauma (3). Less common etiologies include infection, metabolic diseases, and hereditary pancreatitis (3,4). In addition, mechanical obstruction of the sphincter of Oddi, pancreatic ducts, or ampulla of Vater can cause pancreatitis (5). Etiologies of mechanical obstruction include vascular/ischemic injury; inflammatory conditions such as Crohn disease and sarcoidosis; infections such as tuberculosis, Whipple disease, and funguses; and neoplasms such as cholangiocarcinoma, metastatic disease, and leukemia/lymphoma (5). Mechanical obstruction by a primary malignancy of the blood, such as leukemia/lymphoma, is rare in pediatric population. Obstructive lymphoma has been reported in 5 adults, but only 1 child, whose pancreatic and biliary outflow tracts were obstructed by non-Hodgkin lymphoma (6). Pancreatic outflow obstruction by T-cell acute lymphoblastic leukemia (ALL) has never been reported. We report a case of an 11-year-old boy with obstructive pancreatitis as a result of occult T-cell ALL.

An 11-year-old developmentally delayed boy with a 3-week history of abdominal pain, emesis, and constipation was transferred to our tertiary care center for treatment of acute pancreatitis. The patient had intermittent, nonradiating epigastric pain, and nonbloody, nonbilious emesis, with no aggravating or alleviating factors. Associated symptoms included fevers, night sweats, and anorexia. The patient had history of encopresis and a family history positive for gallstones. The referring hospital laboratory studies revealed elevated lipase (7000 U/L, 146–200), amylase (420 U/L, 30–110), and a hypokalemic, hypochloremic metabolic alkalosis with cholestasis. The referring hospital's abdominal ultrasound was negative for gallstones.

The patient's only remarkable physical examination finding was palpable loops of bowel. Repeat laboratory studies revealed markedly elevated gammaglutamyltransferase (173, 7–30), total bilirubin (3.1, 0.3–1.0), direct bilirubin (2.5, 0.1–0.4), amylase (1007 U/L, <106), and lipase (16,911 U/L, 146–200). Triglycerides were within normal limits and a negative purified protein derivative test ruled out tuberculosis. The patient's cholestasis prompted concern that obstructive gallstones had been missed on ultrasound, and magnetic resonance cholangiopancreatography (MRCP) was performed for more detailed visualization of the biliary tree. The MRCP revealed diffuse thickening of the second, third, and fourth portions of the duodenum, areas of marked small bowel thickening in the jejunum and ileum, and biliary and pancreatic duct dilation (Fig. 1). Esophagogastroduodenoscopy (EGD) was performed for biopsy collection, and revealed gastric distension, an esophageal ulcer, and grape-like clusters intruding into the intestinal lumen, completely obstructing the second portion of the duodenum (Fig. 2). The ampulla of Vater was not visible (Fig. 3).

FIGURE 1

FIGURE 1

FIGURE 2

FIGURE 2

FIGURE 3

FIGURE 3

The patient's initial treatment consisted of aggressive fluid therapy with close monitoring of serum electrolytes and urine output, as well as morphine for pain and ondansetron for nausea. A peripherally inserted central catheter was placed to administer parenteral nutrition. A chest radiograph to confirm catheter placement revealed widening of the patient's upper mediastinal silhouette, and a subsequent computed tomography scan of chest revealed a bulky anterior mediastinal mass.

Soon thereafter, intestinal biopsies revealed infiltration of the lamina propria by atypical precursor T cells, blastic in appearance, and suggestive of leukemia. Subsequent bone marrow biopsies confirmed the diagnosis of T-cell ALL. The pediatric ALL study protocol called for intervention with daunorubicin, vincristine, methylprednisolone, and intrathecal cytarabine. After initiation of chemotherapy, the patient's small bowel tumors receded and his pancreatitis resolved. At this time, enteral feeds were introduced and because biopsies of the patient's gastric antrum and body had also displayed chronic, mildly active Helicobacter pylori gastritis, oral triple therapy treatment was initiated.

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DISCUSSION

Based on the patient's cholestasis and family history of gallstones, our leading diagnosis was initially gallstone pancreatitis. The bowel wall thickening incidentally seen on his MRCP, combined with the patient's acute constipation and palpable loops of bowel on examination, redirected our focus from biliary obstruction to intestinal obstruction by duodenal hematoma versus malignancy. On EGD, the absence of vascular lesions, ischemic changes, and granulomatous inflammation decreased our suspicion for vascular/ischemic injury, inflammatory conditions, and infective processes. The obliterated bowel lumen and our inability to visualize the ampulla of Vater on EGD confirmed obstruction of pancreaticobiliary outflow. The intestinal grape-like masses and mediastinal mass on chest radiograph were consistent with a primary leukemia/lymphoma, and led to the diagnosis of obstructive pancreatitis secondary to T-cell ALL.

Concurrently, managing T-cell ALL and pancreatitis necessitated unique treatment. Our institution typically provides enteral feeding in acute pancreatitis—a protocol patterned after adult recommendations, and designed to avoid parenteral nutrition-related hyperglycemia and catheter sepsis (2). In our patient, duodenal obstruction prevented oral feeding or passage of a nasojejunal tube, and enteral nutrition was not possible; consequently, parenteral nutrition was administered. Second, the treatment for biliary obstruction because of primary blood malignancies is chemotherapy (7). Many chemotherapeutic agents—including cytarabine and methylprednisolone, which are part of the pediatric ALL treatment protocol—can cause pancreatitis, threatening patients with existing pancreatic injury. Fortunately, the protocol called for intrathecal cytarabine, which has less potential for pancreatic toxicity than intravenous cytarabine; therefore, the only protocol medication that posed a threat to the patient's pancreas was intravenous methylprednisolone. With this in mind, we treated the patient conventionally for a few days until his amylase and lipase trending downward and then administered methylprednisolone, with close monitoring of the patient's pancreatic enzymes. Finally, the patient's duodenal blockage prevented administration of oral triple therapy for his H pylori esophageal ulcer and gastritis, and caused accumulation of hydrochloric acid, which exacerbated his pain. To address this, we administered intravenous pantoprazole, drained the patient's stomach acid via nasogastric tube, and replaced these fluid losses with intravenous fluid until his obstruction had resolved.

The spectrum of pancreatitis etiologies is broad and includes serious malignancies; it is essential for health care providers to maintain an expansive differential throughout treatment. In our case of acute obstructive pancreatitis, the patient's unusual presentation provoked an extensive workup and led to diagnosis and treatment of pancreatitis and T-cell ALL.

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REFERENCES

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