Early Ultrasonographic Diagnosis and Clinical Follow-up of Hepatic Veno-Occlusive Disease after Allogeneic Bone Marrow Transplantation in a Patient with Acute Lymphoblastic Leukemia

Matsumoto, Yosuke; Horiike, Shigeo; Sato, Junichi; Fujimoto, Yoshiko; Taniguchi, Kyoko; Shimizu, Daisuke; Shimura, Kazuho; Uchiyama, Hitoji; Kuroda, Junya; Nomura, Kenichi; Shimazaki, Chihiro

doi:10.2169/internalmedicine.48.1404

Cited by 16 publications

(16 citation statements)

References 17 publications

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“…Matsumoto et al [8] described the resolution of VOD in 1 case where reversal of portal flow confirmed by ultrasound preceded the development of hyperbilirubinemia. Our observations suggest that clinically significant VOD without hyperbilirubinemia may be more common than previously suspected.…”

Section: Discussionmentioning

confidence: 99%

Veno-Occlusive Disease of the Liver in the Absence of Elevation in Bilirubin in Pediatric Patients after Hematopoietic Stem Cell Transplantation

Myers

Dandoy

El-Bietar

et al. 2015

Biology of Blood and Marrow Transplantation

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Veno-occlusive disease (VOD) of the liver is a well-described and significant complication of hematopoietic stem cell transplantation (HSCT), with limited successful therapeutic options in severe cases. Prompt diagnosis and initiation of treatment is crucial to restrict the extent of disease. However, a subset of patients may not meet all current diagnostic criteria at presentation, and waiting for these to be met may delay therapy. We retrospectively reviewed 794 HSCT patients treated at our institution between 2003 and 2013, identifying 17 (2.1%) who developed VOD. Of these, 5 (29%) were noted to have an absence of elevated bilirubin at the time of VOD diagnosis and reversal of portal venous flow on ultrasound. Median total and conjugated bilirubin at VOD diagnosis were 1.0 and 0.2 mg/dL, respectively. All 5 patients were subsequently diagnosed with multiorgan failure associated with VOD, including 1 with encephalopathy. Four were treated with intravenous high-dose methylprednisolone (500 mg/m2 per dose every 12 hours for 6 doses). One patient received defibrotide therapy in addition to steroids and another supportive care alone. VOD resolved in 4 of 5 patients, with median time to resolution of VOD, defined as recovery of all organ function and normalization of bilirubin and portal venous flow, of 8 days. Two patients died later from progressive primary disease and chronic graft-versus-host disease, respectively. We conclude that a high index of suspicion for VOD should be maintained in patients despite lack of bilirubin elevation in the presence of other diagnostic criteria such as hepatomegaly, abdominal pain, ascites, or weight gain. Early ultrasound evaluation in these patients may lead to more timely diagnosis and therapeutic interventions.

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Section: Discussionmentioning

confidence: 99%

Veno-Occlusive Disease of the Liver in the Absence of Elevation in Bilirubin in Pediatric Patients after Hematopoietic Stem Cell Transplantation

Myers

Dandoy

El-Bietar

et al. 2015

Biology of Blood and Marrow Transplantation

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“…In the case of allogeneic HCT recipients, differential diagnosis of SOS/VOD and other causes of liver dysfunction, particularly GVHD, may be difficult because such entities may be associated with similar biochemical abnormalities (Matsumoto et al 2009). The disease resembles BuddÀChiari syndrome (BCS) clinically; however, hepatic venous outflow obstruction in SOS/ VOD is due to intrahepatic microscopic endothelial damage leading to occlusion of hepatic sinusoids and terminal hepatic venules, rather than large hepatic veins and inferior vena cava obliteration (McDonald et al 1984;Shulman et al 1987).…”

Section: Differential Diagnosismentioning

confidence: 99%

“…Ultrasound revealed improvements in reversed portal venous flow before the peak in serum bilirubin level and coagulopathy. This analysis was useful for both early diagnosis and clinical follow-up of SOS/VOD (Matsumoto et al 2009). Ultrasound is often abnormal in patients undergoing HCT in the absence of SOS/VOD.…”

Section: Follow-upmentioning

confidence: 99%

New Ultrasound Techniques Challenge the Diagnosis of Sinusoidal Obstruction Syndrome

Dietrich

Trenker

Fontanilla

et al. 2018

Ultrasound in Medicine & Biology

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Sinusoidal obstruction syndrome, also known as veno-occlusive disease (SOS/VOD), is a potentially life-threatening complication that can develop after hematopoietic cell transplantation. Clinically, SOS/VOD is characterized by hepatomegaly, right upper quadrant pain, jaundice and ascites, most often occurring within the first 3 wk after hematopoietic cell transplantation. Early therapeutic intervention is pivotal for survival in SOS/VOD. Thus, a rapid and reliable diagnosis has to be made. Diagnosis of SOS/VOD is based on clinical criteria, such as the Seattle, Baltimore or recently issued European Society for Blood and Marrow Transplantation criteria, to which hemodynamic and/or ultrasound evidence of SOS were added for the first time. However, to rule out major differential diagnoses and to verify the diagnosis, a reliable imaging method is needed. Ultrasound techniques have been proposed in SOS/VOD. Nevertheless, the sensitivity and specificity of transabdominal ultrasound and Doppler techniques need to be improved. Innovative ultrasound methods such as a combination of Doppler ultrasound with shear wave elastography and contrast-enhanced ultrasound techniques should be evaluated for diagnosis and follow-up of SOS/VOD. The goals of this review are to discuss currently available ultrasound techniques and to identify areas for future studies in SOS/VOD.

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“…In particular, an AST level of >750 UI L is a significant marker for the diagnosis of VOD and poor prognosis. [67] Furthermore, the modulation of drug doses in children developing VOD has been described in the literature. Hasegawa et al [66] showed that low serum albumin levels (£3.7 g dL) before the start of pretransplant conditioning were most strongly associated with the development of VOD, suggesting that hepatic function reserve is important in the development and onset time of VOD.…”

Section: Preventionmentioning

confidence: 99%

Hepatic Veno-Occlusive Disease

et al. 2010

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Hepatic veno-occlusive disease (VOD) is a major manifestation of liver toxicity associated with conventional and high-dose chemotherapy in children affected by hematologic malignancies and certain solid tumors. Clinically, patients present with jaundice, painful hepatomegaly, and fluid retention, which may evolve into multi-organ failure, a hallmark of severe disease. The pathogenesis is complex and not completely understood, but the damage to sinusoidal endothelium, typically caused by toxic metabolites released from antineoplastic drugs, is thought to play a crucial role, together with cytokine activation, immune deregulation, and coagulopathy. Diagnosis is based on clinical criteria supported by characteristic ultrasound findings, with the gold standard investigation being hepatic-venous pressure gradient measurement and biopsy. Several treatment options have been tested; the most convincing approach to date is the use of defibrotide, a novel oligonucleotide with antithrombotic and antiplatelet aggregating properties, as well as endothelial-stabilizing effects. This agent, together with other specific forms of supportive care, has shown efficacy in the treatment of established VOD and promising results in the prevention of VOD in pediatric patients receiving chemotherapy.

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Early Ultrasonographic Diagnosis and Clinical Follow-up of Hepatic Veno-Occlusive Disease after Allogeneic Bone Marrow Transplantation in a Patient with Acute Lymphoblastic Leukemia

Abstract: Hepatic veno-occlusive disease (VOD) is a typical complication

Cited by 16 publications

References 17 publications

Veno-Occlusive Disease of the Liver in the Absence of Elevation in Bilirubin in Pediatric Patients after Hematopoietic Stem Cell Transplantation

Veno-Occlusive Disease of the Liver in the Absence of Elevation in Bilirubin in Pediatric Patients after Hematopoietic Stem Cell Transplantation

New Ultrasound Techniques Challenge the Diagnosis of Sinusoidal Obstruction Syndrome

Hepatic Veno-Occlusive Disease

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