Cyclic neutropenia

A 19-month-old girl was brought to the gastrointestinal clinic with a history of poor feeding, persistent intermittent diarrhea, and poor weight gain. In addition, she had a history of numerous ear infections requiring treatment with multiple courses of antibiotics, placement of pressure-equalizing tympanostomy tubes, and bilateral adenoidectomies. She is currently being investigated for immunoglobulin A deficiency and cyclic neutropenia. She was delivered by cesarean section following a failed trial of labor, to a 23-year-old gravida 2, para 1 mother; she weighed 2866 g at birth.

An esophagogastroduodenoscopy revealed no mucosal abnormalities. Multiple biopsies were taken from the duodenum, the gastric antrum, and the distal esophagus. Microscopically, the biopsies from the esophagus and the duodenum were predominantly unremarkable. The gastric antral biopsy snowed an expansion of the lamina propria by edema and lymphoid aggregates (Figure 1; hematoxylin-eosin, original magnification ×20). Although the numbers of lymphocytes, plasma cells, and eosinophils did not appear to be increased above expected levels, occasional neutrophils were scattered in the lamina propria. A few neutrophils were also seen within the surface epithelium. In some of the gastric pits, the surface glycocalyx layer appeared fuzzy. Clusters of corkscrew-shaped, bacilliform organisms, consistent with Helicobacter heilmannii, were seen in these gastric pits (Figure 2, hematoxylin-eo, original magnification ×100).

Helicobacter heilmannii, formerly called Gastrospirillum liominis, is a urease-producing, gram-negative bacterium. It measures 4 to 10 µm in length and 0.5 to 0.89 µm in diameter. It has a corkscrew structure with 4 to 8 spirals and 6 to 10 tufts of bipolar flagella. The organism is more tightly coiled and larger than Helicobacter pylori and is readily distinguished from the latter in hematoxylin-eosin-stained sections of paraffin-embedded tissue. Helicobncter heilmannii has been difficult to culture in vitro, and its proper speciation requires amplification and sequencing of its 16S recombinant RNA genes. While it is found in a variety of animal hosts, including cats, dogs, pigs, cattle, and nonhuman primates, it occasionally infects humans. The prevalence of infection with H hcilniannii in humans who undergo gastric biopsies for dyspepsia-related complaints varies from up to 0.5% in Western countries to 6% in China and Thailand. Similar to H pylori, infection with H heilmannii has been associated with chronic gastritis, peptic ulcer disease, gastric adenocarcinoma, and mucosa-associated lymphoid tissue lymphoma. Treatment guidelines for H heilmannii infection are analogous to those for H pylori infection, and the therapy should include a gastric acid production blocker (histamine H2 blocker or a proton-pump inhibitor) along with at least 2 antibiotics (clarithromycin, metronidazole, or amoxicillin).1,2

References

1. Solnick JV, Schauer DB. Emergence of diverse Helicobacter species in the pathogenesis of gastric and cnterohepatic diseases, din Microhiol Rev. 2001 ;14: 59-97.

2. Solnick JV. Clinical significance of Helicobactcr species other than Helicobacter pylori. Clin infect Dis. 2003;36:349-354.

Dennis C. Wooten, MD, PhD; Dinesh Rakheja, MD; Charles F. Timmons, MD, PhD

Accepted for publication July 6, 2004.

From the Departments of Pathology, University of Texas Southwestern Medical Center, Dallas (Drs Wooten, Rakheja, andTimmons), and Children's Medical Center of Dallas, Dallas, Tex (Drs Rakheja and Timmons).

The authors have no relevant financial interest in the products or companies described in this article.

Reprints: Dinesh Rakheja, MD, Department of Pathology, Mail Code 9073, University of Texas Southwestern Medical Center, 5323 Harry Mines Blvd, Dallas, TX 75235 (e-mail: dinesh.rakheja@utsouthwestern. edu).

Copyright College of American Pathologists Dec 2004
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