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Clinical Features of Cutaneous Pre-Engraftment Syndrome in Patients Receiving Umbilical Cord Blood Stem Cell Transplantation
Umbilical cord blood (UCB) is a stem cell source for patients without matched sibling or unrelated donors undergoing hematopoietic stem cell transplantation (HSCT). Pre-engraftment syndrome (PES) is characterized by unexplained fever and/or skin rash occurring at or prior to neutrophil recovery in UCB HSCT patients. Few studies have described the clinical features of PES and outcomes related to development of acute graft-versus-host disease (aGVHD) and overall survival. Though skin rash occurs in the majority of patients with PES, little is known regarding the morphology, anatomic location, or histology of cutaneous PES. We aimed to identify these features in a series of patients with PES.
Methods:
We retrospectively examined a series of UCB HSCT patients who developed PES, defined as non-infectious fever and skin rash occurring before neutrophil engraftment. Data collected included skin lesion morphology and anatomic location, skin biopsy histopathology, concomitant clinical features, and outcome.
Results:
Four cases were reviewed and analyzed. 3 patients were female and 1 was male with mean age of 42 years (range 25-65). Conditions requiring HSCT included peripheral T-cell lymphoma, hepatosplenic T-cell lymphoma, myelodysplastic syndrome, and acute myeloid leukemia.
Skin rash onset ranged from day +10 to +15 post-HSCT with absolute neutrophil count of 0 to 100 cells/mm3. Lesion morphology was described as morbilliform with follicular accentuation (2/4), confluent erythema with follicular accentuation (1/4), and patchy erythema (1/4). Affected body surface area ranged from 7% to 60%. Histopathology demonstrated interface dermatitis with necrotic keratinocytes in 3/4 patients and mild spongiosis with a superficial perivascular lymphohistiocytic infiltrate in 1 patient.
Concurrent diarrhea was present in 3/4 patients (75%) and elevated liver transaminases occurred in 1/4 patients (25%). 3/4 patients (75%) were treated with systemic corticosteroids at doses between 0.5 to 1 mg/kg/day. All patients were diagnosed with aGVHD (3 intestinal, 1 cutaneous) between day +20 to +34 post-HSCT, and all have survived without disease relapse.
Conclusion:
In our PES case series, 3/4 patients developed a morbilliform skin eruption with follicular accentuation, and the predominant histologic description was interface dermatitis with necrotic keratinocytes. These are the same clinical and histologic features of cutaneous aGVHD. Systemic corticosteroids were administered in 3/4 cases, however all patients developed aGVHD and achieved 90 day relapse-free survival. Larger, prospective studies are needed to better describe the morphologic and histologic features of cutaneous PES to determine the clinical and prognostic significance of this condition, and how they differ from aGVHD. The necessity of treating PES with systemic steroids needs to be further delineated.