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Hemophagocytic lymphohistiocytosis in an adult kidney transplant recipient successfully treated by plasmapheresis: A case report and review of the literature.

Abstract Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening disease entity primarily described in children, but not less relevant in adults. It is characterized by a misdirected activation of the immune system, resulting in an uncontrolled cytokine release from macrophages and cytotoxic T-cells (CTLs). Primary HLH relies on a genetic predisposition, whereas secondary HLH develops in the context of infections, malignancies or autoimmune diseases. However, the awareness and therapeutic knowledge for HLH in adulthood is limited. Most therapy protocols are almost exclusively validated in pediatric cohorts and for primary HLH. Their transferability to adult individuals with mostly secondary HLH is doubtful. Especially the high liver and bone marrow toxicity of applied etoposide-based protocols is discussed controversially and connected to overwhelming infections and death.
PMID
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Authors

Mayor MeshTerms

Kidney Transplantation

Plasmapheresis

Keywords
Journal Title medicine
Publication Year Start




PMID- 29390386
OWN - NLM
STAT- MEDLINE
DCOM- 20180212
LR  - 20180212
IS  - 1536-5964 (Electronic)
IS  - 0025-7974 (Linking)
VI  - 96
IP  - 50
DP  - 2017 Dec
TI  - Hemophagocytic lymphohistiocytosis in an adult kidney transplant recipient
      successfully treated by plasmapheresis: A case report and review of the
      literature.
PG  - e9283
LID - 10.1097/MD.0000000000009283 [doi]
AB  - RATIONALE: Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening disease
      entity primarily described in children, but not less relevant in adults. It is
      characterized by a misdirected activation of the immune system, resulting in an
      uncontrolled cytokine release from macrophages and cytotoxic T-cells (CTLs).
      Primary HLH relies on a genetic predisposition, whereas secondary HLH develops in
      the context of infections, malignancies or autoimmune diseases. However, the
      awareness and therapeutic knowledge for HLH in adulthood is limited. Most therapy
      protocols are almost exclusively validated in pediatric cohorts and for primary
      HLH. Their transferability to adult individuals with mostly secondary HLH is
      doubtful. Especially the high liver and bone marrow toxicity of applied
      etoposide-based protocols is discussed controversially and connected to
      overwhelming infections and death. PATIENT CONCERN: A 51-year old, male, kidney
      transplant recipient was admitted to our center suffering from diarrhea, fever,
      nausea, hyponatremia, kidney graft failure, disorientation, progressive
      hemodynamic instability, and multiorgan failure. DIAGNOSES: Clinical and
      laboratory findings resembled those of a septic shock. Ferritin and soluble
      interleukin-2 receptor (sCD25) levels were disproportionally elevated. Only a
      mild hepatosplenomegaly was diagnosed in a CT scan. A T2-weighted,
      fluid-attenuated inversion recovery MRI showed marked, bilateral and
      periventricular white matter hyperintensities. The cerebrospinal fluid (CSF)
      analysis showed a moderately elevated protein content and cell count. There was
      no evidence of any bacterial, viral, or parasitic infection. The diagnosis of HLH
      was made. INTERVENTIONS & OUTCOMES: The patient was successfully treated by a
      combined approach consisting of plasma exchange (PE), corticosteroids, anakinra, 
      and cyclosporine (CsA). LESSONS: HLH is an important differential diagnosis in
      critically ill patients. Its unspecific clinical picture complicates an early
      diagnosis and may be misclassified as sepsis. A combination of plasma exchange
      (PE), corticosteroids, anakinra, and cyclosporine (CsA) may be a promising and
      less toxic approach for HLH therapy in adults.
CI  - Copyright (c) 2017 The Authors. Published by Wolters Kluwer Health, Inc. All
      rights reserved.
FAU - Nusshag, Christian
AU  - Nusshag C
AD  - Department of Nephrology.
FAU - Morath, Christian
AU  - Morath C
AD  - Department of Nephrology.
FAU - Zeier, Martin
AU  - Zeier M
AD  - Department of Nephrology.
FAU - Weigand, Markus A
AU  - Weigand MA
AD  - Department of Anesthesiology.
FAU - Merle, Uta
AU  - Merle U
AD  - Departement of Gastroenterology, Heidelberg University Hospital, Germany.
FAU - Brenner, Thorsten
AU  - Brenner T
AD  - Department of Anesthesiology.
LA  - eng
PT  - Case Reports
PT  - Journal Article
PT  - Review
PL  - United States
TA  - Medicine (Baltimore)
JT  - Medicine
JID - 2985248R
RN  - 0 (Adrenal Cortex Hormones)
RN  - 0 (Antirheumatic Agents)
RN  - 0 (Immunosuppressive Agents)
RN  - 0 (Interleukin 1 Receptor Antagonist Protein)
RN  - 83HN0GTJ6D (Cyclosporine)
SB  - AIM
SB  - IM
MH  - Adrenal Cortex Hormones/therapeutic use
MH  - Antirheumatic Agents/therapeutic use
MH  - Combined Modality Therapy
MH  - Cyclosporine/therapeutic use
MH  - Diagnosis, Differential
MH  - Diagnostic Imaging
MH  - Humans
MH  - Immunosuppressive Agents/therapeutic use
MH  - Interleukin 1 Receptor Antagonist Protein/therapeutic use
MH  - *Kidney Transplantation
MH  - Lymphohistiocytosis, Hemophagocytic/etiology/*therapy
MH  - Male
MH  - Middle Aged
MH  - *Plasmapheresis
EDAT- 2018/02/03 06:00
MHDA- 2018/02/13 06:00
CRDT- 2018/02/03 06:00
PHST- 2018/02/03 06:00 [entrez]
PHST- 2018/02/03 06:00 [pubmed]
PHST- 2018/02/13 06:00 [medline]
AID - 10.1097/MD.0000000000009283 [doi]
AID - 00005792-201712150-00136 [pii]
PST - ppublish
SO  - Medicine (Baltimore). 2017 Dec;96(50):e9283. doi: 10.1097/MD.0000000000009283.