COPYRIGHT © 2004 by the American College of Chest Physicians.
Critical Care Monday, October 25, 2004
4:15 PM - 5:45 PM
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Respiratory Failure in a Patient with Hodgkins Lymphoma Transforming into an Aggressive Syncytial Variant Infiltrating the Lung
Steven C. Jacoby, MD*,
Doreen J. Addrizzo-Harris, MD and
Herman Yee, PhD, MD
New York University Medical Center, New York, NY
INTRODUCTION: Pulmonary complications after hematopoietic stem cell transplant have a wide differential diagnosis. We report a patient with diffuse nodular disease three months after autologous bone marrow transplant.
CASE PRESENTATION: 27 year old man with Hodgkins lymphoma presented with acute febrile illness with dry cough. He originally presented 15 months prior to admission with fevers and cough, and was found to have mediastinal adenopathy involving the chest wall and several lung nodules; pathology revealed nodular sclerosing Hodgkins lymphoma. He underwent chemotherapy with doxorubicin, bleomycin, vinblastine, and dacarbazine but social issues limited him to 2 of 28 planned radiation doses. He relapsed and received ifosfamide, carboplatin, and etoposide, but recurred 3 months prior to admission. He underwent an autologous peripheral bone marrow transplant after high dose BCNU, etoposide, cytarabine, and melphalan with apparent clinical remission. One week before admission, he was well and playing basketball until the acute onset of fevers, chills, dry cough then progressive hypoxemia, despite antibacterials. Chest CT revealed innumerable coalescent nodules and mediastinal adenopathy involving the left chest wall with pleural effusion (figure 1
). He was started on treatment for fungal, bacterial, PCP and mycobacterial etiologies with adjuvant steroids. Pathologic specimens included transbronchial biopsy which was negative for infection but with lymphocyte aggregates of uncertain significance, and chest wall biopsy was consistent with recurrent Hodgkins lymphoma (figure 2 demonstrates Reed-Sternberg cell surrounded by characteristic mixed cellular infiltrate). After a month of mechanical ventilation with progressively higher levels of oxygen and lower compliances, he expired. At autopsy, the lung was found to be infiltrated with sheets of anaplastic cells consistent with a syncytial varient of Hodgkins lymphoma.
DISCUSSIONS: The infectious pulmonary complications of autologous bone marrow transplant are similar to, but less common than, those of allogeneic transplants because of persistent t-cell defects. We suspected that the randomly distributed diffuse nodular pulmonary infiltration in this patient could represent milliary tuberculosis or fungal disease. Although recurrent Hodgkins disease involving the lung is common, it rarely causes respiratory failure due to infiltration of the lung parenchyma, as can be seen in NHL. In Hodgkins lymphoma, autopsy series show equal numbers of patients who die from tumor progression as from infection, with significant numbers of patients with clinically missed infections (1). Involvement of the lung in Hodgkins lymphoma occurs with increasing frequency as the disease advances from initial diagnosis (20%) to autopsy (60%) (2). Pulmonary manifestations of Hodgkins disease during relapse of disease include nodules (sometimes cavitating), consolidation, often with mediastinal nodal and chest wall involvement (3). Increased aggressiveness of tumor is often observed after failed hematopoietic transplantation, perhaps because of decreased immune surveillance. Transformation of Hodgkins disease into an aggressive variant infiltrating the lung is rare. A Japanese (4) case series recently documented the progression of 6 patients with Hodgkins disease into anaplastic large cell lymphoma with an aggressive clinical course. One patient had lung involvement and 5 of 6 died within 13 months.
CONCLUSION: Progressive pulmonary infiltrates leading to acute respiratory failure in patients who are post bone marrow transplantation for Hodgkins lymphoma are most commonly related to infectious etiologies. We report a rare case of transformation into an aggressive syncytial varient leading to hypoxemic respiratory failure.
DISCLOSURE: S.C. Jacoby, None.
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- Mori N, et al. Hodgkins Disease with subsequent transformation to CD30 positive non-Hodgkins lymphoma in six patients. Cancer1999; 85
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Copyright © 2004 by the American College of Chest Physicians.