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Hemophagocytic Lymphohistiocytosis in a Kidney Transplant Recipient: Case Report
María Araceli Povedano Medina1, Álvaro Arnau Prieto1, Aitor Parra Zurutuza1
1Nephrology, Hospital Universitario Donostia, San Sebastián, España.
Insights
Hemophagocytic lymphohistiocytosis (HLH) is a serious condition. In transplant patients, identifying triggers like infections is crucial for effective treatment and recovery.
Area of Science:
- Immunology
- Hematology
- Transplant Medicine
Background:
- Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening inflammatory syndrome.
- Diagnostic criteria for adult HLH are primarily derived from pediatric studies.
- Adult HLH is often triggered by infections, autoimmunity, or malignancy, necessitating identification of the underlying cause.
Purpose of the Study:
- To highlight the importance of considering HLH in adult transplant recipients.
- To present a case of HLH secondary to multiple infections in a kidney transplant patient.
- To emphasize the role of prompt diagnosis and targeted treatment in managing HLH.
Main Methods:
- Case report of a 74-year-old kidney transplant recipient.
- Diagnosis of HLH based on clinical presentation (fever, pancytopenia, splenomegaly).
- Identification of co-existing infections: Epstein-Barr virus, aspergillosis, and leishmania.
Main Results:
- The patient was diagnosed with HLH secondary to Epstein-Barr virus, aspergillosis, and leishmania.
- Targeted treatment addressing these infections led to the resolution of HLH.
- Successful recovery of the patient following treatment.
Conclusions:
- HLH is a critical consideration in transplant recipients presenting with unexplained fever and cytopenias.
- Early diagnosis and treatment of underlying triggers are essential for managing HLH in this vulnerable population.
- This case underscores the complexity of HLH in immunocompromised individuals and the importance of a comprehensive diagnostic approach.
Abstract:
Hemophagocytic lymphohistiocytosis is a potentially fatal multisystemic inflammatory syndrome that is better understood in the pediatric population. Consequently, the diagnostic criteria for adults still derives from studies conducted in the pediatric population. Several genetic mutations and secondary causes, including infections, autoimmunity, and malignancy, have been reported as significant actors in this condition, especially in adults. It is of the utmost importance to identify these triggers, as the treatment of this condition is largely dependent on addressing the underlying cause. Those who have undergone transplantation and whose immune response is already compromised are particularly susceptible to this condition. We present the case of a 74-year-old kidney transplant recipient who was admitted due to a persistent fever of unknown origin, pancytopenia, and splenomegaly. The patient was ultimately diagnosed with hemophagocytic lymphohistiocytosis in our hospital secondary to Epstein-Barr virus, aspergillosis, and leishmania infections. Targeted treatments for the aforementioned conditions led to the resolution of the syndrome and the recovery of the patient. Lymphohistiocytosis is a rare, albeit serious, condition that should be considered a differential diagnosis in the early stages of critical illness in transplant recipient patients. Doing so enables target treatments to be administered as soon as possible.
