eMedicine Specialties > Gastroenterology > Colon

Neutropenic Enterocolitis

Author: Rajeev Vasudeva, MD, FACG, Clinical Professor of Medicine, Consultants in Gastroenterology, University of South Carolina School of Medicine
Contributor Information and Disclosures

Updated: Dec 8, 2006

Introduction

Background

Neutropenic enterocolitis is an acute life-threatening condition characterized by transmural inflammation of the small and large bowel in patients who are severely myelosuppressed and immunosuppressed.

The clinical presentation can be dramatic, and the outcome may be devastating. Mortality rates are high, and treatment is controversial, with options varying from conservative medical management to surgical intervention. Early recognition of the condition is paramount to a potentially good outcome.

Over the past 3 decades, this condition has increasingly been reported in adults with a variety of myeloproliferative disorders and solid malignant tumors and in adults who have had solid organ and bone marrow transplantation. Some cases in adults are due to the increasing use of myelotoxic chemotherapeutic regimens.

Pathophysiology

Although the exact etiology and progression are unknown, profound neutropenia appears to be the common denominator. Many factors have been described that may potentially play a role in the pathogenesis and include the following:

  • Mucosal injury is caused by cytotoxic drugs. However, mucosal injury can occur in the absence of cytotoxic drug therapy, and neutropenia itself can cause mucosal ulcerations.
  • Cecal distention, whether primary or secondary to vinca alkaloids, may compromise the blood supply, leading to further mucosal damage.
  • The use of antibiotics and steroids may contribute to an altered enteric bacterial flora and overgrowth of fungi.
  • Bacterial invasion of the impaired bowel wall may result in transmural inflammation, leading to perforation and peritonitis. Bacteremia, often recurrent, is a frequent complication.

The pathologic process appears to involve the cecum alone, or it may extend to the ileum, ascending colon, or both. It is felt that cecal distensibility and limited blood supply may predispose the cecum to injury more often than other areas.

Frequency

United States

The exact incidence and prevalence rates are unknown because many patients survive and are never diagnosed. An autopsy study in children reported a prevalence rate of 24%, while a cohort study in children treated for acute myelogenous leukemia reported a frequency rate of 33%. Data regarding neutropenic enterocolitis in adults are sparse. In one systematic review, a 5.3% pooled incidence rate has been reported in adults.

International

An even greater paucity of information regarding the international incidence and prevalence rates of neutropenic enterocolitis exists in the published literature. A recent study from India performed by Jain et al (2000) has reported a frequency rate of 6.1% in 180 children undergoing chemotherapy for acute lymphocytic leukemia. A retrospective study from Turkey performed by Buyukasik et al (1997) reported an incidence rate of 6.5% for neutropenic enterocolitis in acute myeloid leukemia and 4.6% for neutropenic enterocolitis in acute lymphoblastic leukemia in adults.

Mortality/Morbidity

  • Mortality rates of 5-100% have been reported during conservative management, with an average of about 40-50%.
  • In a collective review of 178 published cases, the mortality rate was reported at 48% for conservative management and 21% for surgical management; however, these numbers cannot be compared with each other because of selection bias.

Race

No predilection for any specific race is reported in the literature.

Sex

No sex predilection is reported in the literature.

Age

  • No known frequency differences in age groups exist based on the published literature.
  • Although neutropenic enterocolitis initially was described in children, it is increasingly reported in adults.

Clinical

History

Most patients who are affected with neutropenic enterocolitis are receiving antineoplastic drugs and are profoundly neutropenic (ie, <1000 cells/mm3).

  • Symptoms usually occur within 10-14 days after initiation of cytotoxic chemotherapy.
  • The typical presentation mimics acute appendicitis.
  • Symptoms include the following:
    • Right lower quadrant abdominal pain - May be cramping and intermittent or a continuous dull ache
    • Fever
    • Watery or bloody diarrhea - Occurs in about 25-45% of patients
    • Nausea
    • Vomiting
    • Abdominal distention
  • Oral and pharyngeal mucositis may manifest prior to the onset of colonic symptoms.
  • The time course and severity of the clinical presentation is variable.

Physical

Physical findings vary depending on the severity of the disease and the presence or absence of complications.

  • Abdominal distention, absence of bowel sounds, and a tympanitic abdomen may suggest an ileus.
  • The abdomen may be markedly tender, especially in the right lower quadrant.
  • The cecum may be palpated as a boggy mass.
  • Rebound tenderness may suggest colonic perforation.
  • Sepsis may elicit shock.

Causes

Although cytotoxic chemotherapeutic agents account for most cases, other conditions that may predispose some patients to develop this condition exist.

  • The cytotoxic chemotherapeutic agents include cytosine arabinoside, vinca alkaloids, and doxorubicin.
  • Other drugs that have been implicated anecdotally include paclitaxel, docetaxel, procainamide, sulfasalazine, 5-fluorouracil, vinorelbine, carboplatin, cisplatin, gemcitabine, and leucovorin.
  • Other predisposing conditions include the following:
    • Myelodysplastic syndromes, multiple myeloma, and aplastic anemia
    • Solid organ and bone marrow transplantation
    • AIDS
    • Cyclic neutropenia
    • Solid malignant tumors
    • Lymphomas

More on Neutropenic Enterocolitis

Overview: Neutropenic Enterocolitis
Differential Diagnoses & Workup: Neutropenic Enterocolitis
Treatment & Medication: Neutropenic Enterocolitis
Follow-up: Neutropenic Enterocolitis
References
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References

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Further Reading

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Keywords

necrotizing enterocolitis, ileocecal syndrome, typhlitis, acute ileocecal enterocolitis, transmural inflammation of the small bowel and large bowel in myelosuppression and immunosuppression, profound neutropenia, cecum, ileum, ascending colon, typhlitis, cecitis, right lower quadrant pain

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Contributor Information and Disclosures

Author

Rajeev Vasudeva, MD, FACG, Clinical Professor of Medicine, Consultants in Gastroenterology, University of South Carolina School of Medicine
Rajeev Vasudeva, MD, FACG is a member of the following medical societies: American College of Gastroenterology, American Gastroenterological Association, American Society for Gastrointestinal Endoscopy, and South Carolina Medical Association
Disclosure: Nothing to disclose.

Medical Editor

Robert J Fingerote, MD, MSc, BSc, FRCPC, Consultant, Clinical Evaluation Division, Biologic and Gene Therapies, Directorate Health Canada; Consulting Staff, Department of Medicine, Division of Gastroenterology, York Central Hospital, Richmond Hill, Ontario
Robert J Fingerote, MD, MSc, BSc, FRCPC is a member of the following medical societies: American Association for the Study of Liver Diseases, American Gastroenterological Association, Canadian Medical Association, Ontario Medical Association, and Royal College of Physicians and Surgeons of Canada
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

Douglas M Heuman, MD, FACP, Director of Hepatology, McGuire Veterans Affairs Medical Center, Professor, Department of Internal Medicine, Division of Gastroenterology, Virginia Commonwealth University School of Medicine
Douglas M Heuman, MD, FACP is a member of the following medical societies: American Association for the Study of Liver Diseases, American College of Physicians, and American Gastroenterological Association
Disclosure: Nothing to disclose.

CME Editor

Alex J Mechaber, MD, FACP, Assistant Dean for Medical Curriculum, Associate Professor of Medicine, Division of General Internal Medicine, University of Miami Miller School of Medicine
Alex J Mechaber, MD, FACP is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians-American Society of Internal Medicine, and Society of General Internal Medicine
Disclosure: Nothing to disclose.

Chief Editor

Julian Katz, MD, Clinical Professor of Medicine, Drexel University College of Medicine; Consulting Staff, Department of Medicine, Section of Gastroenterology and Hepatology, Hospital of the Medical College of Pennsylvania
Julian Katz, MD is a member of the following medical societies: American College of Gastroenterology, American College of Physicians, American Gastroenterological Association, American Geriatrics Society, American Medical Association, American Society for Gastrointestinal Endoscopy, American Society of Law Medicine and Ethics, American Trauma Society, Association of American Medical Colleges, and Physicians for Social Responsibility
Disclosure: Nothing to disclose.

 
 
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