Chest
Volume 157, Issue 4, April 2020, Pages e107-e109
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Venoocclusive Disease With Both Hepatic and Pulmonary Involvement

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Pulmonary venoocclusive disease (PVOD) is a rare form of pulmonary vascular disease with pulmonary hypertension characterized by preferential involvement of the pulmonary venous system. Hepatic venoocclusive disease (HVOD), also known as sinusoidal obstruction syndrome, is a condition that occurs in 13% to 15% of patients after hematopoietic stem cell transplantation (HSCT). Although hepatic and pulmonary venoocclusive diseases may share some pathologic features as well as some etiologies such as HSCT, these two disorders have never been described together in a single adult patient. We report the case of a patient who received HSCT and developed HVOD and PVOD within 9 months. Despite their differences, PVOD and HVOD share common risk factors and associated conditions, suggesting that in the context of HSCT, the two diseases share common pathophysiological mechanisms. Optimal treatment for HSCT-related PVOD remains to be determined.

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Case Report

We report a case of venoocclusive disease with hepatic and pulmonary involvement in a woman who had received HSCT. The 54-year-old woman was admitted to the hematology department with acute myeloid leukemia. Seven months later, she received allo-HSCT (unrelated donor) after two successive conditioning regimens including gemtuzumab and a pretransplant conditioning regimen including cyclophosphamide, busulfan, and fludarabine. Six days posttransplantation, she reported abdominal pain and rapid

Discussion

Diagnosis of PVOD and HVOD is challenging. Allo-HSCT, an unrelated donor, and use of gemtuzumab, busulfan, and cyclophosphamide are known risk factors for both diseases.2,11 The patient met European Society for Blood and Marrow Transplantation criteria for HVOD,2 with symptoms appearing within 21 days post-HSCT and elevated bilirubin. Diagnosis of PVOD was supported by precapillary PH, discrepancy between hemodynamic parameters and dyspnea, suggestive CT scan abnormalities, severe hypoxemia,

Acknowledgments

Financial/nonfinancial disclosures: The authors have reported to CHEST the following: D.M. reports grants and personal fees from Actelion and Bayer; personal fees from GSK, MSD, and Pfizer; and nonfinancial support from Jazz Pharmaceuticals, outside the submitted work. None declared (N.M., M.-F.S. B.D., T.S.).

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Drs Degano and Soumagne contributed equally to this work.

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