International Journal of Clinical Case Reports 2018, Vol.8, No.1, 1-4
2
rods. Few faggot cells (cells containing multiple auer rods) were also seen. These cells stained strongly positive
with myeloperoxidase and sudan black. Immunophenotypic analysis was performed for confirmation of diagnosis,
and it was suggestive of acute myeloid leukemia - FAB M3 type i.e. Acute promyelocytic leukemia with CD13,
CD33, CD117, MPO, CD45, CD79a positive and characteristically negative for immature cell markers CD34 and
HLA-DR. On Fluorescence in situ hybridization (FISH) 90% of interphase cells were positive for PML-RARa
fusion. The patient was started on ATRA 45 mg/m
2
along with daunorubicin 50 mg/m
2
for 4 days. After induction
chemotherapy, bone marrow was in remission so consolidation treatment was started. Arsenic trioxide 0.15
mg/kg/day 5 days in a week for 5 weeks for two cycles followed by ATRA 45 mg/m
2
daily for 7 days and
daunorubicin 50 mg/m
2
/day for 3 days given. The patient developed symptoms of headache and diplopia after 5
days of treatment with ATRA. On fundus examination there was bilateral papilloedema. Magnetic resonance
imaging (MRI) of brain revealed no significant abnormality. On performing lumbar puncture, cerebrospinal fluid
(CSF) an increased opening pressure of 300 mm of water was found without any cytological or biochemical
abnormality. CSF cytology was negative and biochemistry revealed CSF protein- 16 mg/dl and CSF glucose- 67
mg/gl. Patient was non-obese and there was no significant history of drug intake such as oral contraceptive pills,
minocycline, tamoxifen, lithium, nitrofurantoin or vitamin A. Further lab results revealed hemoglobin of 12.4 g/dl,
total leucocyte count – 6300/mm
3
, platelet count – 4,00,000/mm
3
; TSH- 1.31 mU/L, Na
+
- 137 and K
+
- 4.5 mmol/l,
creatinine- 0.62 mg/dl, Liver function tests including SGOT- 26 U/l, SGPT- 28 U/l, and S. Bilirubin- 0.6 mg/dl.
Cortisol levels at 8 am were 300 nmol/l, S. calcium- 10 mg/dl and Parathyroid hormone- 25 pg/ml. Therefore, a
probable diagnosis of ATRA induced PC was made, and ATRA was stopped. The patient was started on
acetazolamide 500 mg thrice daily and steroids, resulting in complete resolution of the symptoms within 5 days.
ATRA was reinstituted after 2 weeks at reduced dose of 25 mg/m
2
with prophylactic acetazolamide 500 mg/day.
Later ATRA was tolerated well by the patient and consolidation therapy was completed.
2 Discussion
Acute promyelocytic leukemia comprises 10% to 15% of acute myeloid leukemia. There is no definite age
predeliction but is usually seen in adults. Acute promyelocytic leukemia (APML) is a medical emergency with a
high rate of early mortality. Therefore, it is necessary to start treatment as soon as the diagnosis is suspected. For
patients with newly diagnosed APML induction therapy is recommended which incorporates all-trans retinoic acid
(ATRA) at doses of 45 mg/m
2
orally until remission is achieved (Castaigne et al., 1990; Tallman et al., 1997).
Though ATRA is considered to be a well- tolerated agent it has many minor side effects including nasal stuffiness,
dry red skin, chapped lips, transient elevations in serum aminotransferases and bilirubin, and hypertriglyceridemia.
These rarely require an alteration in treatment. Two serious complications which may require an alteration in
treatment due to ATRA are differentiation syndrome and Idiopathic intracranial hypertension.
Pseudotumor cerebri (PC) is also commonly called Idiopathic intracranial hypertension (IIH). The PC is a rare
disorder with an incidence of approximately 1 case/1 lac population per annum. It primarily affects obese females
of reproductive age group. This disorder is characterized by various signs and symptoms produced by raised
intracranial pressure i.e. headache, papilledema and vision loss with normal cerebrospinal fluid composition along
with no evident cause of intracranial hypertension on neuroimaging or other laboratory investigations. A long list
of medications have been reported to be associated with PC. The evidence linking growth hormone treatment,
retinoids, and tetracycline antibiotics is strongest; however, the mechanisms by which these might produce PC is
not known. Other than obesity, the association between other medical conditions such as Cushing’s disease,
hypoparathyroidism, hypothyroidism, chronic kidney disease, anemia and PC is not proven.
Diagnosis-modified Dandy criteria is used for diagnosis of PC; each of the following apply.
Symptoms and signs of increased intracranial pressure (i.e. headache, transient visual obscurations, pulse
synchronous tinnitus, papilledema, visual loss)
No other neurologic abnormalities or impaired level of consciousness
