International Journal of Clinical Case Reports 2018, Vol.8, No.1, 1-4
1
Research Report Open Access
All-trans Retinoic Acid-induced Pseudotumor Cerebri during Treatment of
Acute Promyelocytic Leukemia: A Case Report
Alok Ranjan
, Harsha P. Panchal, Asha S. Anand, Apurva A. Patel, Sonia P. Parikh, Sandip A. Shah
Department of Medical Oncology, Gujarat Cancer Research Institute, Civil Hospital Campus, Ahmedabad, 380016, India
Corresponding author email
International Journal of Clinical Case Reports 2018, Vol.8, No.1 doi
Received: 15 Jan., 2018
Accepted: 15 Feb., 2018
Published: 02 Mar., 2018
Copyright © 2018
Ranjan et al., This is an open access article published under the terms of the Creative Commons Attribution License, which permits
unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
Preferred citation for this article:
Ranjan A., Panchal H.P., Anand A.S., Patel A.A., Parikh S.P., and Shah S.A., 2018, All-trans retinoic acid-induced pseudotumor cerebri during treatment
of acute promyelocytic leukemia: a case report, International Journal of Clinical Case Reports, 8(1): 1-4 (doi
Abstract
All-trans retinoic acid (ATRA), is a first line treatment of acute promyelocytic leukemia (APML). It is a derivative of
vitamin A, and a relatively safe drug, but use of ATRA can lead to side effects including some life-threatening conditions such as
differentiation syndrome and pseudotumor cerebri (PC). PC is a rare disorder characterized by headache, papilledema, and/or vision
loss despite normal composition of cerebrospinal fluid and normal radiological imaging of brain. It may arise as a complication of
ATRA during treatment of APML. ATRA induced PC occurs predominantly in the pediatric age group. We report a rare case of
ATRA-induced PC in an adult, 25 year old female undergoing consolidation treatment for APML. On discontinuation of ATRA and
treatment with acetazolamide and corticosteroids symptoms improved within 5 days. ATRA was reinstituted within 2 weeks at
reduced dose with prophylactic acetazolamide. Later ATRA was tolerated well by the patient and consolidation therapy was
completed.
Keywords
All-trans retinoic acid; Pseudotumor cerebri; Acute promyelocytic leukemia
Background
Acute promyelocytic leukemia (APML) is a biologically and clinically distinct variant of AML. All-trans retinoic
acid (ATRA), is the first line treatment in APML where it induces the terminal differentiation of malignant
promyelocytes to mature functioning neutrophils. ATRA is generally combined with other agents as remissions
induced by ATRA therapy alone are short-lived. When combined with anthracycline, a complete remission (CR)
rate of >90% is obtained in the patients with APML (Fenaux et al., 1999; Lo-Coco et al., 2010). Drug toxicities
due to ATRA are generally minor. Nasal stuffiness, dry red skin, chapped lips, transient elevations in serum
aminotransferases and bilirubin, and hypertriglyceridemia can occur, but rarely require an alteration in treatment
(Holmes et al., 2012). However, there are two serious complications that can result from ATRA treatment of
APML: (a) differentiation syndrome and (b) Pseudotumor cerebri. In patients with headache, papilledema, and/or
vision loss, the diagnosis of PC should be suspected. PC is more common in pediatric age group and adolescents
treated with ATRA (Vanier et al., 2003; Naithani et al., 2009), it is rarer in the adults (Castaigne et al., 1990).
Herein, we describe a case of ATRA-induced PC in a 25 year old adult female while she was on consolidation
treatment for APML.
1 Case Report
A 25-year-old non-obese female patient presented to our hospital with complain of fever and gum hyperplasia
since 3 months. On examination, the patient had pallor and bleeding from the gums. Laboratory investigations
revealed hemoglobin of 8.5 g/dl, total leucocyte count – 36,000/mm
3
(moderate leucocytosis) and platelet count –
41,000/mm
3
(moderate thrombocytopenia) with differential count of 70% blast cells + promyelocytes, 3%
neutrophils, 27% lymphocytes. The coagulation profile showed normal PT and APTT of 12 sec and 30 seconds
respectively. The peripheral blood film showed normocytic normochromic anemia, with predominant blasts and
hypergranular promeylocytes. Bone marrow examination revealed 90% blasts + promyelocytes. These blasts were
medium to large in size with high nucleus to cytoplasmic ratio, round to indented or lobed nucleus obscured by
prominent azurophilic granules with moderate amount of cytoplasm. Many of the cells showed prominent Auer
