Post‐hematopoietic stem cell transplant hemophagocytic lymphohistiocytosis or an impostor: Case report and review of literature

Abstract

HLH occurring after HSCT is a relatively rare disease. Many conditions may mimic or trigger HLH in post-HSCT period (eg, cytokine release syndrome, engraftment syndrome, graft rejection/failure, acute graft-vs-host disease, infections systemic inflammatory response syndrome/sepsis, and thrombotic microangiopathy). Moreover, this period is usually marked by febrile illness, cytopenia, and a "cytokine storm" leading to elevation of inflammatory biomarkers like ferritin and sCD25. These parameters overlap with the diagnostic criteria for HLH. Such confounding factors make the management of post-HSCT HLH quite challenging. We illustrate this critical issue with case report of a patient who was diagnosed with HLH after allogeneic HSCT for tAML. He received MP and CsA for HLH but VP-16 was not administered due to fear of severe myelosuppression. Fortunately, he responded well to treatment and remains in remission to date. We recommend caution while using HLH-94/HLH-2004 guidelines for the diagnosis and management of post-HSCT HLH. In this article, we pinpoint these issues with a brief review of all the pediatric cases and clinical studies of post-HSCT HLH along with a critical evaluation of its various diagnostic criteria. Finally, based on the limitations of current diagnostic criteria, we suggest a need for formulating disease-specific diagnostic criteria for post-HSCT HLH.

Overseas Chinese Returnees’ Swindler Syndrome and Their Entrepreneurial Education Under Psychological Resilience

“…Through the analysis and study of the psychological resilience of these individuals, their problems are explored. Because of these problems, students' psychologies are imperceptibly changed by carrying out the above contents, so that students can have a more accurate understanding of their abilities, face up to their own effort's skills, and weaken their performance of swindler syndrome (Anant et al, 2018;Wang, 2021).…”

Post-transplant Hemophagocytic Lymphohistiocytosis in Benign Hematological Disorders: Experience of 4 Cases with Review of Literature

“…The largest case series on HLH was published by Ishii et al, in which the incidence of post transplant HLH was 0.019% [5]. It can be either early-onset (B 30 days post transplant) or late-onset ([ 30 days post transplant) [3]. All the above-illustrated cases had an early-onset post transplant HLH presenting within 30 days of transplant.…”

“…While late onset post transplant HLH is caused by infective etiology, the causes for early onset variety are still obscure. The early onset variety overlaps with hyperacute GVHD, engraftment syndrome [2], sepsis, capillary leak syndrome, and cytokine release syndrome [3]. In a case series by Asano et al, on posttransplant HLH in children, it was shown that early onset HLH was commoner and presence of respiratory symptoms, elevated bilirubin and triglyceride levels were associated with poorer prognosis.…”

“…Post transplant HLH is a form of secondary HLH that can be either early-onset (\ 30 days post transplant) or lateonset (C 30 days post transplant) [2]. The incidence of post-transplant HLH in previous case series has been estimated from 0.3 to 16.8% [3]. It is seen commonly post transplant in both benign and malignant conditions.…”

Secondary hemophagocytic syndrome after autologous hematopoietic cell transplant and immune therapy for neuroblastoma

“…Recognition of this risk may allow for improved patient counseling, earlier intervention, and the application of targeted treatments. One example is secondary hemophagocytic syndrome (HPS), a historically rare complication of hematopoietic cell transplant (HCT) . Definitions of posttransplant secondary HPS range from classical hemophagocytic lymphohistiocytosis (HLH) criteria to modified parameters that attempt to account for confounding factors in this population.…”