Weekly Chest CasesArchive of Old Cases

Case No : 316 Date 2003-11-15

  • Courtesy of 승혁 / Seoul National University Hospital, Seoul, Korea
  • Age/Sex 35 / F
  • Chief ComplaintDyspnea was developed during chemotherapy (AML, M3)
  • Figure 1
  • Figure 2

Diagnosis With Brief Discussion

Diagnosis
ATRA (all-trans retinoic acid) syndrome
Radiologic Findings
Figs 1. Chest CT shows bilateral pleural effusion.
Fig 2. High-resolution CT scan shows interlobular septal thickening and small nodular infiltrations in both lungs.
Brief Review
All-trans retinoic acid (ATRA) is an effective agent for the treatment of acute promyelocytic leukemia, that is, M3 type of acute myelogenous leukemia. This agent can differentiate the acute promyelocytic leukemic cells and reduce the risk of disseminated intravascular coagulation with few cytotoxic effects, resulting in improvement of the outcome of acute promyelocytic leukemia. One complication of this agent is to cause ATRA syndrome characterized by dyspnea, fever, weight gain, and pleural and pericardial effusions (1.2). Some patients with ATRA syndrome have to receive mechanical ventilation because of severe hypoxemia (1,2). However, a prompt recovery from ATRA syndrome can be achieved by treatment with prednisolone (1-3).
The ATRA syndrome occurs in up to 25% of the patients treated with ATRA, and no predictive factors of this syndrome could be found in the previous report. Post-mortem reports of the cases dying from this syndrome have shown the infiltration of maturing myeloid cells and edema in the lung (1). The maturing myeloid cells are considered to release various types of cytokines, which may explain several symptoms of ATRA syndrome such as fever, weight gain, and heart failure. These symptoms can be promptly improved by the steroid treatment which may suppress the effects of cytokines (4,5)
References
1. Frankel SR, Eardley A, Lauwers G et al. The "retinoic acid syndrome" in acute promyelocytic leukemia. Ann Intern Med 1992;117:292-296
2. De Botton S, Dombret H, Sanz M et al. Incidence, clinical features, and outcome of all trans-retinoic acid syndrome in 413 cases of newly diagnosed acute promyelocytic leukemia. Blood 1998;92:2712-2718
3. Davis BA, Cervi P, Amin Z et al. Retinoic acid syndrome: pulmonary computed tomography (CT) findings. Leuke Lymphoma 1996;23:113-117
4. Amano Y, Tajika K, Mizuki T, Amano M, Dan K, Kumazaki T. All-trans retinoic acid syndrome: chest CT assessment. Eur Radiol 2001;11:1516-1517
5. ATRA (all-trans-retinoic acid) Syndrome in Acute Promyelocytic Leukemia: Clinical and Radiologic Findings. Kim KH, Goo JM, Im JG, Chung MJ, Do KH, Seo JB, Park S. Journal of the Korean Radiological Society 2001;44: 339-343
Keywords
Lung, Iatrogenic lung disease, Drug complication,

No. of Applicants : 21

▶ Correct Answer : 7/21,  33.3%
  • - Annecy Hospital, France Gilles Genin Dumont
  • - Geneva Hospital, Switzerland Philippe
  • - Korea University Anam Hospital, Korea Bo Kyung Je
  • - Kyunghee University Hospital, Korea Kyung Ran Ko
  • - Mallinckrodt Institute of Radiology, USA Jin Mo Goo
  • - National Cancer Center , Korea Tae Jung Kim
  • - Shin Kong Wu Ho-Su Memorial Hsopital, Taipei, Taiwan Chen-Chun Lin
▶ Semi-Correct Answer : 7/21,  33.3%
  • - CHU Nancy-Brabois, France Denis Regent
  • - Ev. Krkhs. Hubertus, Berlin, Germany Michael Weber
  • - Ewha Women's University Hospital, Korea Sung Shine Shim
  • - Kangbuk Samsung Hospital, Korea Semin Chong
  • - Ohio State University Med Cntr, Ohio, USA Sumit Seth
  • - Ondokuz Mayis University, Samsun, Turkey Cetin Celenk
  • - Tokyo Kouseinenkin Hospital , Japan Noriatsu Ichiba
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