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Abstract: SA-PO867

Hemophagocytic Lymphohistiocytosis Following Simultaneous Liver and Kidney Transplant

Session Information

Category: Transplantation

  • 2002 Transplantation: Clinical

Authors

  • Azeem, Zeeshan, Albert Einstein Medical Center, Philadelphia, Pennsylvania, United States
  • Shaikh, Zahir Ali, Albert Einstein Medical Center, Philadelphia, Pennsylvania, United States
  • Bradauskaite, Gitana, Albert Einstein Medical Center, Philadelphia, Pennsylvania, United States
  • Zaki, Radi, Albert Einstein Medical Center, Philadelphia, Pennsylvania, United States
  • Kaul, Hitesh, Albert Einstein Medical Center, Philadelphia, Pennsylvania, United States
  • Khanmoradi, Kamran, Albert Einstein Medical Center, Philadelphia, Pennsylvania, United States
  • Knorr, John P., Albert Einstein Medical Center, Philadelphia, Pennsylvania, United States
Introduction

Hemophagocytic lymphohistiocytosis (HLH) is a rare disease entity characterized by an inappropriate immune activation syndrome characterized by fever, hepatosplenomegaly, cytopenia and progressive multiple-organ failure. It is broadly classified as either primary or secondary form. Primary HLH is an autosomal recessive usually diagnosed in children less than 2 years of age. Secondary HLH is triggered by immune insults such as vaccinations, viral infections, autoimmune disorders or malignancies. HLH has also been reported after kidney, liver and hematopoietic stem cell transplants.
We present a case of HLH secondary to graft vs host disease (GVHD) in a patient who underwent simultaneous liver-kidney transplant (SLKT).

Case Description

A 74 year old male received SLKT due to NASH cirrhosis, from a 29 year old female deceased donor. He was re-admitted 4 weeks post-transplant with altered mental status, diarrhea, fever, rash and signs of sepsis. Following initial resuscitation, intubation and mechanical ventilation, the patient was started on broad spectrum antibiotics and anti-fungal medications. His cell counts, kidney function and liver tests were within normal range at admission but he later developed severe pancytopenia and a very high ferritin (15,910 ng/mL). Blood cultures showed P.aeruginosa and E. faecium. Abdominal imaging revealed splenomegaly. Patient’s white cell count did not improve despite maximum dose of G-CSF. A bone marrow biopsy revealed hemophagocytes (Fig. 1).A diagnosis of HLH was made and the patient was a started on etoposide and dexamethasone. A skin biopsy was suggestive of GVHD.Hemodynamic status continued to deteriorate during hospital course leading to death on 10th day of re-admission.

Discussion

HLH is a rare disease and should be suspected in patients with fever, cytopenias and ferritin >500 µg/L. Etoposide and dexamethasone are most commonly used therapies. Secondary HLH has a dismal prognosis and a very high fatality rate. Early diagnosis and treatment can lead to improved outcomes.
Keywords: Hemophagocytic lymphohistiocytosis (HLH), graft versus host disease (GVHD).