INTRODUCTION
Severe neutropenia and immunodeficiency, as either sequelae of
hematologic malignancies or direct consequences of chemotherapy or
hematopoietic cell transplant (HCT), increase the risk of infectious
complications. Gastrointestinal infections observed in this patient
population account for approximately 30% of neutropenic infections
[1]. Mortality secondary to gastrointestinal infections in
neutropenic patients has only been reported in two single-institution
reviews but is consistent at 13% and 14% [1, 2]. The incidence of
acute appendicitis in the pediatric population with acute leukemia or
lymphoma is approximately 1.5% [3], however, the incidence is
unknown for adults with hematologic malignancies or HCT patients. Acute
appendicitis is, indeed, scarcely mentioned in hematopoietic cell
transplant literature [1, 4, 5]. Though uncommon, appendiceal
disease is a challenging diagnostic and therapeutic problem. These
patients may be afflicted with alternative diseases such as mucositis,
acute GVHD of the gastrointestinal tract, neutropenic enterocolitis
(typhlitis), or infectious colitis, which confounds the diagnosis.
Additionally, patients may be in various stages of hematologic recovery
either in the pre-transplant or pre-engraftment period and may be on
additional immunosuppression for prophylaxis or treatment of
graft-vs-host disease (GVHD). This can lead to atypical, non-localized
pain and/or lack of peritoneal signs which may delay diagnosis. The
attenuated clinical findings were apparent in one pediatric study and
resulted in a 37.5% error rate in accurate diagnosis of appendicitis
[6]. These factors make it difficult to pursue invasive management
given higher risk of surgical complications. Medical treatment and
surgical intervention have been described in hematologic malignancies
[3, 6] and hematopoietic cell transplant [2, 4, 5] but the lack
of evidence makes management challenging. We report here on five adult
hematopoietic cell transplant patients treated in our institution who
developed appendiceal disease at various times in their clinical course.