Anicteric veno-occlusive disease after hematopoietic stem cell transplantation in children

Naples, Jeffrey; Skeens, Micah; Auletta, Jeffery J.; Rangarajan, Hemalatha G.; Abu‐Arja, Rolla; Horwitz, Edwin M.; Stanek, Joseph; Bajwa, Rajinder

doi:10.1038/bmt.2015.208

Cited by 26 publications

(19 citation statements)

References 12 publications

(16 reference statements)

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“…The main difference between the two diagnostic systems is hyperbilirubinemia being mandatory in the Baltimore criteria, which implies longer time waiting for its development or intrinsically more aggressive forms. Up to 30% of children with VOD/SOS was anicteric (7,45,46) compared to 12% of adults. The clinical scenario can be variable, in particular in children where anicteric forms are not rare (13,47) and dynamically changing.…”

Section: Clinical Presentation and Diagnosismentioning

confidence: 99%

Diagnosis and Treatment of VOD/SOS After Allogeneic Hematopoietic Stem Cell Transplantation

et al. 2020

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Hepatic veno-occlusive disease (VOD) or sinusoidal obstruction syndrome (SOS) is a rare complication characterized by hepatomegaly, right-upper quadrant pain, jaundice, and ascites, occurring after high-dose chemotherapy, hematopoietic stem cell transplantation (HSCT) and, less commonly, other conditions. We review pathogenesis, clinical appearance and diagnostic criteria, risk factors, prophylaxis, and treatment of the VOD occurring post-HSCT. The injury of the sinusoidal endothelial cells with loss of wall integrity and sinusoidal obstruction is the basis of development of postsinusoidal portal hypertension responsible for clinical syndrome. Risk factors associated with the onset of VOD and diagnostic tools have been recently updated both in the pediatric and adult settings and here are reported. Treatment includes supportive care, intensive management, and specific drug therapy with defibrotide. Because of its severity, particularly in VOD with associated multiorgan disease, prophylaxis approaches are under investigation. During the last years, decreased mortality associated to VOD/SOS has been reported being it attributable to a better intensive and multidisciplinary approach.

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Section: Clinical Presentation and Diagnosismentioning

confidence: 99%

Diagnosis and Treatment of VOD/SOS After Allogeneic Hematopoietic Stem Cell Transplantation

et al. 2020

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“…Demonstration of reversal of portal venous blood flow (RPVF) by ultrasonography is suggestive of VOD but is not pathognomonic because as flow can remain normal in the presence of VOD [8] or be reversed in other clinical settings. Moreover, RPVF is not included in either the Seattle or Baltimore diagnostic criteria.…”

Section: Introductionmentioning

confidence: 99%

“…Liver biopsy can provide a definitive diagnosis; however, because of thrombocytopenia and coagulopathy, biopsies are rarely performed. Anicteric VOD (total bilirubin < 2 mg at diagnosis and remains below 2 mg/ dL throughout the course of the disease) is well described in the pediatric literature [8,9] but is not captured using the Baltimore criteria, for which hyperbilirubinemia is a mandatory criteria. Some providers and/or institutions use the Baltimore criteria, whereas others use the Seattle criteria to diagnose VOD, further complicating management and study of this syndrome.…”

Section: Introductionmentioning

confidence: 99%

High Variability in the Reported Management of Hepatic Veno-Occlusive Disease in Children after Hematopoietic Stem Cell Transplantation

Skeens

McArthur

Cheifetz

et al. 2016

Biology of Blood and Marrow Transplantation

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Veno-occlusive disease (VOD) is a potentially fatal complication of hematopoietic stem cell transplantation (HSCT). Patients with VOD are often critically ill and require close collaboration between transplant physicians and intensivists. We surveyed members of a consortium of pediatric intensive care unit (PICU) and transplant physicians to assess variability in the self-reported approach to the diagnosis and management of VOD. An internet-based self-administered survey was sent to pediatric HSCT and PICU providers from September 2014 to February 2015. The survey contained questions relating to the diagnosis and treatment of VOD. The response rate was 41% of 382 providers surveyed. We found significant variability in the diagnostic and management approaches to VOD in children. Even though ultrasound is not part of the diagnostic criteria, providers reported using reversal of portal venous flow seen on abdominal ultrasound in addition to Seattle criteria (70%) or Baltimore criteria to make the diagnosis of VOD. Almost 40% of respondents did not diagnose VOD in anicteric patients (bilirubin < 2 mg/dL). Most providers (75%) initiated treatment with defibrotide at the time of diagnosis, but 14%, 7%, and 6% of the providers waited for reversal of portal venous flow, renal dysfunction, or pulmonary dysfunction, respectively, to develop before initiating therapy. Only 50% of the providers restricted fluids to 75% of the daily maintenance, whereas 21% did not restrict fluids at all. Albumin with diuretics was used by 95% of respondents. Platelets counts were maintained at 20,000 to 50,000/mm(3) and 10,000 to 20,000/mm(3) by 64% and 20% of the respondents, respectively. Paracentesis was generally initiated in the setting of oliguria or hypoxia, and nearly 50% of the providers used continuous drainage to gravity, whereas the remainder used an intermittent drainage approach. Nearly 73% of the transplant providers used VOD prophylaxis, whereas the remainder did not use any medications for VOD prophylaxis. There was also considerable variation in the management strategies among the transplant and critical care providers. We conclude that there is considerable self-reported variability in the diagnosis and management of VOD in children. The practice variations reported in this study should encourage the development of standard practice guidelines, which will be helpful in improving the outcome of this potentially fatal complication.

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“…With increased applications of HCT for different disease indications, evaluation and treatment of HCT‐related complications have become increasingly important to mitigate early transplant‐related mortality and improve outcomes . SOS can be a devastating complication after HCT . Several studies have demonstrated that up to 50% of patients with SOS develop renal or pulmonary dysfunction .…”

Section: Discussionmentioning

confidence: 99%

“…In this regard, it is critical to avoid exacerbating AKI with aggressive diuresis or rapid removal of ascetic fluid. HCT recipients often require intravenous parenteral nutrition, frequent administration of blood products, and multiple nephrotoxic medications, all of which may worsen FO, further contributing to AKI . Therefore, management of AKI and FO in HCT patients is particularly challenging; and when FO >20% or is refractory to conventional pharmaceutical diuresis, CRRT may need to be considered.…”

Section: Discussionmentioning

confidence: 99%

The role of continuous renal replacement therapy in the management of acute kidney injury associated with sinusoidal obstruction syndrome following hematopoietic cell transplantation

Raina

Abusin

Vijayaraghavan

et al. 2018

Pediatric Transplantation

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Maintaining fluid balance, pre- and post-MA-HCT is essential and usually requires frequent administration of diuretics. Hepatic sinusoidal obstructive syndrome is potentially life-threatening, especially when associated with AKI and MOF. This study describes six patients who developed AKI-associated SOS and diuretic-resistant FO who subsequently underwent CRRT using standardized management guidelines for fluid balance post-HCT. Retrospective chart review was done for HCT patients between September 2011 and October 2013 at a tertiary care children's hospital. Thirty-four patients underwent MA-HCT in the study period. Six patients had SOS complicated by diuretic-resistant FO and underwent CRRT. Defibrotide was used in three patients. Median time on CRRT was 10.5 days. Sixty-six percent (N = 4 of 6) of patients had full resolution of SOS symptoms with a mortality rate of 34% (N = 2 of 6). Among patients who had full recovery of SOS symptoms, one patient developed AKI, end-stage renal diseases and underwent kidney transplantation 34-months post-HCT. Thus, of six included patients, two died and one developed ESRD with only 50% (N = 3 of 6) good outcome. Use of a standardized, evidence-based fluid balance protocol and early initiation of CRRT for HCT-related AKI/SOS was associated with good outcomes.

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Anicteric veno-occlusive disease after hematopoietic stem cell transplantation in children

Cited by 26 publications

References 12 publications

Diagnosis and Treatment of VOD/SOS After Allogeneic Hematopoietic Stem Cell Transplantation

Diagnosis and Treatment of VOD/SOS After Allogeneic Hematopoietic Stem Cell Transplantation

High Variability in the Reported Management of Hepatic Veno-Occlusive Disease in Children after Hematopoietic Stem Cell Transplantation

The role of continuous renal replacement therapy in the management of acute kidney injury associated with sinusoidal obstruction syndrome following hematopoietic cell transplantation

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