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Weekly Chest CasesArchive of Old Cases

Case No : 1143 Date 2019-09-28

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  • Courtesy of Jung Hee Hong, Hyungjin Kim, Soon Ho Yoon / Seoul National University Hospital
  • Age/Sex 56 / F
  • Chief Complaintfever, acute promyelocytic leukemia
  • Figure 1
  • Figure 2
  • Figure 3

initial chest PA

Diagnosis With Brief Discussion

Diagnosis
ATRA (All trans-retinoic acid) syndrome
Radiologic Findings
Figs 1-2. Initial chest PA showed clear lung fields. Two weeks after ATRA treatment, chest PA showed enlarged heart and pulmonary congestion. Patchy opacities and septal lines were noted in both lungs. Small amount of bilateral pleural effusion was also noted.

Fig 3. Lung window setting of the chest CT scan showed multifocal peribronchovascular consolidation and ground glass opacities in both lungs.
Brief Review

Since Frankel et al. first described all trans-retinoic acid (ATRA) syndrome, it has been regarded as a life-threatening complication following ATRA therapy. ATRA syndrome specifically occurs as a result of ATRA usage which can differentiate acute promyelocytic leukemia blasts (M3 in the French-American-British classification of acute myelocytic leukemia) into mature granulocytes.

ATRA syndrome is characterized by fever, respiratory distress, pleural and pericardial effusion, weight gain, and pulmonary infiltrates noted on chest radiography which are also generally associated with an increasing WBC.

The pathophysiology of ATRA syndrome is not completely understood, but it is associated with the increased secretion of various cytokines, particularly interleukin-2, in a manner resembling capillary leak syndrome. The proposed mechanisms may involve changes in the cytokine secretion and adhesive qualities of AML cells during ATRA-induced differentiation.

The major radiographic abnormalities of ATRA syndrome are similar to typical features of cardiogenic pulmonary edema: these include cardiomegaly, vascular pedicle widening, ground-glass opacity, peribronchial cuffing, septal lines and pleural effusion. Nodules and consolidation can also occur.

Due to the nonspecific nature of the aforementioned radiologic features, ATRA syndrome is usually impossible to differentiate from other diseases such as edema, pneumonia or leukemic infiltration without definite clinical history. However, in combination with relevant clinical history, early recognition and quick treatment of the ATRA syndrome can be achieved by withdrawing ATRA combination in conjunction with steroid use.
Please refer to
Case 316, Case 348,
KSTR Imaging Conference 2011 Summer  Case 5,
References
1. Jung JI, Choi JE, Hahn ST, Kim CC, Park SH. adiologic features of all-trans-retinoic acid syndrome. AJR 2002;178:475-480
2. Frankel SR, Eardley A, Lauwers G, Weiss M, Warrel RP. The "retinoic acid syndrome" in acute promyelocytic leukemia. Ann Intern Med 1992;117:292-296
3. Cardinale L, Asteggiano F, Moretti F, et al. Pathophysiology, clinical features and radiological findings of differentiation syndrome/all-trans-retinoic acid syndrome. World J Radiol. 2014;6:583-588.
Keywords
lung, acute myelocytic leukemia, all trans retinoic acid syndrome,

No. of Applicants : 66

▶ Correct Answer : 40/66,  60.6%
  • - Saitama-Sekishinkai Hosptal , Japan MIHOKO YAMAZAKI
  • - Kinki University Faculty of Medicine, , Japan MITSURU MATSUKI
  • - Showa University Fujigaoka Hospital , Japan KYOKO NAGAI
  • - Chonnam National University Hospital , Korea (South) ARA A CHO
  • - Mayo Clinic , United States AKITOSHI INOUE
  • - Chonbuk National University Medical School , Korea (South) HYEJIN YANG
  • - NIMS, HYDERABAD , India AMIT K DADHANIA
  • - , Korea (South) HYEYOUNG CHOI
  • - NIMS, HYDERABAD , India BHASKAR K
  • - Other , Korea (South) INSOO JANG
  • - Vita Hospital , Brazil DIOGO LAGO PINHEIRO
  • - Other , Korea (South) SEONGSU KANG
  • - DELTA CARE HOSPITAL, THANJAVUR, INDIA , India SIVARAJA SUBRAMANIAM
  • - Seoul National University Hospital , Korea (South) Ju Gang Nam
  • - Universidad de Chile , Chile GONZALO PEREIRA RETAMALES
  • - Chonbuk National University Hospital , Korea (South) JUNGHWAN KIM
  • - Fujieda Municipal General Hospital , Japan HAYATO NOZAWA
  • - The University of Tokyo Hospital , Japan TOSHIHIRO FURUTA
  • - Myongji Hospital , Korea (South) KI EON KWON
  • - Gifu University Hospital , Japan Yo Kaneko
  • - Seoul National University Hospital , Korea (South) JI HEE KANG
  • - The University of Tokyo Hospital , Japan RYO KUROKAWA
  • - Chonbuk National University Hospital , Korea (South) YOUNGKWANG LEE
  • - University of Tsukuba Hospital , Japan HIROAKI TAKAHASHI
  • - , Japan NAOMI YUASA
  • - Ajou University Hospital , Korea (South) HYERIN KIM
  • - Chonbuk National University Hospital , Korea (South) KUM JU CHAE
  • - Chonnam National University Hospital , Korea (South) PARK SEUNG GWI
  • - Asan Medical Center , Korea (South) JIHOON KIM
  • - Chonnam National University Hospital , Korea (South) BYEONGHAK SIM
  • - Toranomon Hospital , Japan KAORU SUMIDA
  • - Chonbuk National University Hospital , Korea (South) DONG HAN SHIN
  • - Ichinomiya Nishi Hospital , Japan Takao Kiguchi
  • - Private sector , Greece VASILIOS TZILAS
  • - , Japan YUMI MAEHARA
  • - Hamamatsu University Hospital , Japan YUKI HAYASHI
  • - Diagnose.me (BV) / Royal Perth Hospital , Australia YURANGA WEERAKKODY
  • - Chonnam National University Hwasun Hospital , Korea (South) WONGI JEONG
  • - Chungnam National University Hospital , Korea (South) JONG EUN LEE
  • - the first affiliatited hospital of nanjing medical univercity , China HAI XU
▶ Correct Answer as Differential Diagnosis : 5/66,  7.6%
  • - Chonbuk National University Hospital , Korea (South) SANGHEON KIM
  • - Diskapi Yildirim Beyazit Hospital, Ankara , Turkey MERIC TUZUN
  • - Chonbuk National University Hospital , Korea (South) MINJEE KIM
  • - National Center for Global Health and Medicine , Japan HIROSHI TAKUMIDA
  • - Chonbuk National University Hospital , Korea (South) YOUNG JU SONG
▶ Semi-Correct Answer : 5/66,  7.6%
  • - The University of Kyoto Hospital , Japan TOMOAKI OTANI
  • - Chonbuk National University Hospital , Korea (South) MINGI SHIN
  • - Other , Korea (South) JUWON KIM
  • - Korea University Anam Hospital , Korea (South) KYU-CHONG LEE
  • - Kizawa Memorial Hospital , Japan Shoji Okuda
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