title: Hemophagocytic syndrome following haploidentical peripheral blood stem cell transplantation with post-transplant cyclophosphamide.
abstract: Hemophagocytic syndrome (HPS) is a rare but serious complication after allogeneic transplantation which has been reported to be particularly high after unrelated cord blood transplantation. We report on the incidence, risk factors and outcome of HPS in 51 patients (age 2-64 years) after haploidentical peripheral blood stem cell (PBSC) transplantation with post-transplantation cyclophosphamide (PTCY). The incidence of HPS was 12.2 %, occurring at a median of 18 days. The non-relapse mortality in patients with HPS was 83.3 % compared to 11.6 % in patients without HPS. Complete donor chimerism was documented in all patients with HPS. Definite infective etiology was identified in two patients only. The others were refractory to multiple lines of treatment and 3 patients underwent a second transplant. Even though the symptoms and biochemical markers of HPS showed prompt response in 2/3 patients undergoing a second allograft, they succumbed to infections before haematological recovery. The others succumbed to multi-organ failure or infections. Age < 10 years, transplantation for non-malignant disease and high CD34 content of the graft were identified as risk factors for HPS. Considering the fact that post-transplant HPS is usually a refractory and fatal condition, we discuss further attempts at deciphering the pathogenesis, developing modalities to prevent this complication and improve the outcome.
authors.affiliation: Department of Blood and Marrow Transplantation and Hematology, Dharamshila Hospital and Research Centre, Vasundhara Enclave, New Delhi, 110096, India. drsaritaranij@gmail.com.|Manashi Chakrabarti Foundation, Kolkata, India. drsaritaranij@gmail.com.
authors.forename: Sarita Rani
authors.identifier: http://orcid.org/0000-0002-9988-3514
authors.initials: SR
authors.lastname: Jaiswal
authors.affiliation: Manashi Chakrabarti Foundation, Kolkata, India.
authors.forename: Aditi
authors.initials: A
authors.lastname: Chakrabarti
authors.affiliation: Manashi Chakrabarti Foundation, Kolkata, India.|Apollo Gleneagles Hospital, Kolkata, India.
authors.forename: Sumita
authors.initials: S
authors.lastname: Chatterjee
authors.affiliation: Department of Blood and Marrow Transplantation and Hematology, Dharamshila Hospital and Research Centre, Vasundhara Enclave, New Delhi, 110096, India.
authors.forename: Sneh
authors.initials: S
authors.lastname: Bhargava
authors.affiliation: CSIR-Indian Institute of Chemical Biology, Kolkata, India.|Academy of Scientific and Innovative Research, CSIR-CRRI, New Delhi, India.
authors.forename: Kunal
authors.initials: K
authors.lastname: Ray
authors.affiliation: Department of Blood and Marrow Transplantation and Hematology, Dharamshila Hospital and Research Centre, Vasundhara Enclave, New Delhi, 110096, India.|Manashi Chakrabarti Foundation, Kolkata, India.
authors.forename: Suparno
authors.initials: S
authors.lastname: Chakrabarti
mesh_terms: D000293:Adolescent; D000328:Adult; D064591:Allografts; D018952:Antigens, CD34; D002648:Child; D002675:Child, Preschool; D003520:Cyclophosphamide / Q000008:administration & dosage*; D005260:Female; D006238:Haploidy; D006801:Humans; D007166:Immunosuppressive Agents / Q000008:administration & dosage*; D015994:Incidence; D051359:Lymphohistiocytosis, Hemophagocytic / Q000453:epidemiology / Q000209:etiology* / Q000517:prevention & control; D008297:Male; D008875:Middle Aged; D036102:Peripheral Blood Stem Cell Transplantation / Q000009:adverse effects*; D011182:Postoperative Care; D012307:Risk Factors; D013997:Time Factors; D014019:Tissue Donors; D018183:Transplantation Chimera; D055815:Young Adult
chemical_list: D018952:Antigens, CD34; D007166:Immunosuppressive Agents; D003520:Cyclophosphamide
keywords: Haploidentical; Hemophagocytic syndrome; PBSC; Post-transplantation cyclophosphamide