09/2017
Use of CytoSorb in hemophagocytic lymphohistiocytosis (HLH)

Dr. med. Marcel Frimmel1, PD Dr. med Sebastian Fetscher2, PD Dr. med Friedhelm Sayk1 Sana Clinics L├╝beck GmbH 1Interdisciplinary Intensive Care Medicine 2Medical Clinic III - Hematology, Internal Oncology, Immunology und Palliative Care

Summary:

This case study reports on a 49-year-old male patient who was admitted to the hospital with signs of sepsis (high lactate, hypotension, leucopenia, high LDH and ferritin) after he had undergone hematological examination 2 weeks earlier due to repeated episodes of fever of unclear origin and without final diagnosis.

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Case presentation

  • Transfer to the intensive care unit and initiation of antibiotic therapy with piperacillin/tazobactam
  • Despite of extensive diagnostics, no focus could be identified
  • Quick stabilization of the patient and transfer to Intermediate Care
  • Readmission to ICU due to a marked coagulation deficiency, suggesting a disseminated intravascular coagulopathy, however ferritin plasma concentrations were significantly increased (81,393 ng/ml), which ultimately resulted in the suspected diagnosis of hemophagocytic lymphohistiocytosis (HLH-acquired form), escalation of antibiotic therapy to meropenem
  • Initiation of immunosuppressive therapy with cortisone and immunoglobulins i.v. as well as chemotherapy with etoposide
  • Due to persistent leucocytopenia and repeated fever, antibiotic therapy with meropenem was supplemented with vancomycin and voriconazole (without microbiological findings)
  • The patient was catecholamine-free at all times and hemodynamically stable with volume resuscitation
  • No improvement of the coagulation situation (fibrinogen 0.67 g/l, Quick 41%) even with substitution of fibrinogen concentrate (2x4g/day), persistent leucocytopenia and thrombocytopenia
  • Patient also showed markedly increased values of inflammatory mediators (IL-6> 700 pg/ml, sIL-2R 16,000 U/ml, CRP 114 mg/l) as well as an increase in lactate dehydrogenase up to 2,000 U/l
  • With the rationale of intervening with the cytokine storm and the anticipation of stabilizing the coagulopathy, decision was made to initiate CytoSorb as an adjunctive therapy together with CVVHD (without the indication for acute renal failure)

Treatment

  • Eleven consecutive treatments with CytoSorb (first two treatments for 12 hours each, treatment 3-11 for 24 hours each)
  • CytoSorb was used in conjunction with CRRT (Multifiltrate, Fresenius Medical Care) performed in CVVHD mode
  • Blood flow rate: 150 ml/min
  • Anticoagulation: citrate
  • CytoSorb adsorber position: pre-hemofilter

Measurements

  • Inflammatory parameters (CRP, leucocytes, IL-6, sIL-2R)
  • Hematologic parameters (ferritin, fibrinogen, Quick, LDH)
  • Renal function (creatinine)

Results

  • Significant improvement in coagulation (fibrinogen, Quick) during the course of treatments
  • Clear reduction in inflammatory parameters under CytoSorb therapy (CRP, IL-6)
  • Improvement in HLH-relevant parameters (LDH and ferritin)
  • No acute effect on leukocytes and sIL-2R

Patient Follow-Up

  • In the further course of treatment no microbiological source could be found
  • Further improvements in coagulation and slow normalization of the leukocyte count
  • Transfer of the patient to Intermediate Care, later to a normal ward and discharge to the home environment
  • Outpatient care under continued immunosuppressive therapy with cyclosporin, cyclophosphamide and dexamethasone

CONCLUSIONS

  • The patient presented here was treated in a polypragmatic fashion due to his exceptional disease pattern and although a causal link is difficult to prove, CytoSorb treatment was associated with a gradual stabilization in his coagulation as well as a containment of the cytokine storm
  • The installation of the absorber into the CVVH circuit as well as the application of CytoSorb itself was simple and safe