Incidence and clinical characteristics of engraftment syndrome after syngeneic hematopoietic stem cell transplantation in patients with hematological diseases / 中华血液学杂志

Objective: To explore the incidence and clinical characteristics of engraftment syndrome (ES) after syngeneic hematopoietic stem cell transplantation (syn-HSCT) in patients with hematological diseases. Methods: The clinical data of 21 patients who received syn-HSCT at People's Hospital of Peking University from January 1994 to May 2018 were retrospectively analyzed. Results: Seven (33.3% ) of 21 patients developed ES. The onset of ES symptoms occurred at a median of 8 (range: 5-13) days after HSCT, and the diagnosis of ES occurred at a median of 10 (range: 7-14) days after HSCT. Steroids were administered immediately after the diagnosis of ES, the median time of symptom continuance was 2 (range: 1-5) days, and all patients showed complete resolution of ES symptoms. In the multivariate analysis, patients with acute myeloid leukemia and faster neutrophil reconstitution were the risk factors for ES (HR=15.298, 95% CI 1.486-157.501, P=0.022, and HR=17.459, 95% CI 1.776-171.687, P=0.014) . Meanwhile, there was no significant difference in the overall survival and disease-free survival between patients with ES and those without ES. Conclusion: A high incidence of ES was observed in syn-HSCT recipients. Moreover, the prognosis of ES was excellent.

Engraftment syndrome / 中国小儿急救医学

Engraftment syndrome (ES) is a clinical syndrome that occurs after hematopoietic stem cell transplantation and during the recovery process of neutrophils.The main clinical manifestations include non-infectious fever, rash, capillary leakage and non-cardiogenic pulmonary edema, which may be similar with many early complications after transplantation.Therefore, it is sometimes difficult to be diagnosed and differentiated among different kinds of complications.Typical ES is self-limiting and has good response to steroids.However, patients with ES may result in encephalopathy and multi-organ failure if it is untreated without notice.In this review, we discussed the pathophysiological mechanisms, clinical manifestations, diagnosis and differential diagnosis, risk factors, treatment and prognosis of ES, aiming to provide guidance and reference for clinicians.

Engraftment syndrome after allogeneic hematopoietic stem cell transplantation: a case report and literature review / 白血病·淋巴瘤

Objective To study the engraftment syndrome (ES) and the possible etiology after haploidentical allognneic hematopoietic stem cell transplantation (al]o-HSCT). Methods The clinical manifestation, treatment and outcome of a chronic myeloid leukemia patient who after allo-HSCT were presented. Results The patient started to cough on day 2 after allo-HSCT, dry cough at beginning, white foam sputum, non-infectious fever, fluid retention, weight gain, transient encephalopathy on day 4. The clinical symptoms were improved after treatment with corticosteroids, in which ES was diagnosed. Conclusion ES is a series of clinical syndromes following transplantation which is different from acute graft versus host disease. It occurred after HSCT, and corticosteroids are often effective. Therefore early recognition and accurate diagnosis is very important.

Pre-engraftment Syndrome in Hematopoietic Stem Cell Transplantation

The clinical findings of fever and skin rash with or without evidence of fluid retention, which mimic engraftment syndrome, have been observed during the pre-engraftment period in patients undergoing hematopoietic stem cell transplantation. In order to characterize this newly observed clinical syndrome called pre-engraftment syndrome (pES), we retrospectively analyzed the clinical records of 50 patients. Three out of 14 patients (23.1%) who underwent cord blood stem cell transplantation developed non-infectious fever, skin rash, and tachypnea 4-15 days prior to neutrophil engraftment. Two patients spontaneously recovered with fluid restriction and oxygen inhalation. One patient died of a complicated pulmonary hemorrhage in spite of aggressive supportive therapy and steroid treatment. Four out of 23 patients (17.4%) who underwent allogeneic bone marrow transplantation developed non-infectious fever and skin rash 4 to 5 days prior to neutrophil engraftment. All four of these patients recovered with only steroid treatment. These characteristic findings were not observed in patients who had undergone autologous peripheral blood stem cell transplantation. Interestingly, the speed of neutrophil engraftment was significantly faster for the patients suffering from pre-engraftment syndrome. The close observation and further pathophysiological research are required to better understand this syndrome.

Pre-engraftment Syndrome in Allogeneic Hematopoietic Stem Cell Transplantation / 대한소아혈액종양학회지

PURPOSE: Characteristic clinical findings of fever, skin rash with or without the evidence of fluid retention, mimicking engraftment syndrome (ES), have been observed during the pre-engraftment period in patients with hematopoietic stem cell transplantation (HSCT). We described these findings as pre-engraftment syndrome (pES) and analyzed the incidence and risk factors of pES in the pediatric patients who received HSCT with various stem cell sources. METHODS: Among 53 patients who received HSCT at Dong-A University Medical Center from Sep. 1997 to Mar. 2004, 37 patients with allogeneic HSCT were analyzed retrospectively to characterize the clinical syndrome. RESULTS: In 3 (21.4%) out of 14 patients with cord blood stem cell transplantation, non-infectious fever, skin rash and tachypnea developed on 4~15 days prior to neutrophil engraftment. Two of them spontaneously recovered just with fluid restriction and oxygen inhalation, however, one patient died of complicated pulmonary hemorrhage in spite of aggressive supportive therapy and steroid treatment. In 4 (17.4%) out of 23 patients with allogeneic bone marrow transplantation (BMT), non-infectious fever and skin rash developed on 4~5 days prior to neutrophil engraftment. All of them recovered with steroid treatment only. We could not find any risk factors for this syndrome, however, the speed of neutrophil engraftment was significantly faster in the patients with pES. CONCLUSION: We established a distinctive clinical syndrome during pre-engraftment period, which is very similar but different in occurrence timing from ES. The pES may be associated with enhanced engraftment, but has no impact on the other clinical outcomes.

Early Inflammatory Syndrome following Cord Blood Stem Cell Transplantation / 대한혈액학회지

PURPOSE: We experienced the early toxicities in preengraftment period following cord blood stem cell transplantation (CBSCT), mimicking engraftment syndrome, characterized by fever, skin rash and fluid retention. We report these toxicities here, first in Korea, as an early inflammatory syndrome (EIS). METHODS: We retrospectively reviewed the clinical data of 13 patients who received CBSCT with conventional conditioning chemotherapy for hematologic malignancies and immunodeficiency at Dong-A University Medical Center from 1998 to 2003. We defined the criteria for EIS as follows: noninfectious fever, skin rashes mimicking acute graft versus host disease and the evidence of fluid retention. These clinical signs should be appeared before neutrophil engraftment. RESULTS: We experienced 3 cases of EIS in patients received the conventional dosage of conditioning chemotherapy for CBSCT, which developed on post-transplant day 7~10 and persisted for 2~7 days. Two out of 3 patients were recovered from EIS spontaneously or by supportive treatment, except 1 patient who expired due to complicated pulmonary hemorrhage. CONCLUSION: The definition of EIS is yet obscure, thus more close observations for clinical course of CBSCT and pathophysiological research would be required.