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| CASE REPORT |
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| Year : 2018 | Volume
: 2
| Issue : 1 | Page : 16-18 |
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Status epilepticus as initial presentation of acute promyelocytic leukemia
Sunil Kumar Garg, Pragya Garg
Department of Critical Care, NMC Hospital, Dubai, UAE
| Date of Web Publication | 23-Mar-2018 |
Correspondence Address:
Dr. Sunil Kumar Garg
NMC Hospital, Dubai
UAE
 Source of Support: None, Conflict of Interest: None
DOI: 10.4103/oji.oji_10_17
Coagulopathy is a complex finding in acute promyelocytic leukemia (APL), and it may occur due to interplay of the various factors. Neurological manifestations are rare as the initial presentation in APL. This case illustrates status epilepticus as the initial presentation in this disease. Atypical cells on peripheral smear led to the diagnosis of leukemia. This report demonstrates that the patient of APL can present as status epilepticus.
Keywords: Acute promyelocytic leukemia, acute stroke, atypical cells, status epilepticus
How to cite this article:
Garg SK, Garg P. Status epilepticus as initial presentation of acute promyelocytic leukemia. Oncol J India 2018;2:16-8 |
| Introduction | |  |
Acute promyelocytic leukemia (APL) is a subtype of acute myelogenous leukemia (AML) and is characterized by the presence of abnormal hypergranular promyelocytes in peripheral blood and bone marrow.[1] Most of the signs and symptoms of APL are seen in AML, and it includes fatigue, weakness, and dyspnea due to low hemoglobin, easy bruising or bleeding due to thrombocytopenia, and fever or infection related to leukopenia. Most of the patients with APL present with pancytopenia. APL differs from AML in that most of the patients present with coagulopathy, and it has been described as disseminated intravascular coagulation with associated hyperfibrinolysis. In 40% of the untreated patients, pulmonary and cerebral hemorrhage can occur.[2] However, a few cases present with thromboses.
| Case Report | | |
A 26-year-old male presented to the emergency department with a history of recurrent seizure for the last 1 hour without regaining consciousness. He was intubated, ventilated, and shifted to the Intensive Care Unit on midazolam infusion after loading with levetiracetam. His heart rate was 107/min regular, blood pressure 188/116 mmHg, respiratory rate 22/min, and temperature 36°C in the emergency department. He was unconscious and pupils were semidilated and fixed. His serum electrolytes, renal and liver function values were within the normal range. Complete blood counts revealed hemoglobin 16.2 g/dl, total white blood cell count 10,600/cumm, neutrophils 28%, lymphocytes 41%, monocytes 03%, eosinophils 05%, atypical cells 23%, and platelet count 158,000/cumm. Electrocardiography (ECG) showed the sinus tachycardia, and echocardiography was normal. Electroencephalography (EEG) done in Intensive Care Unit showed fast beta activity without any seizure discharges. His initial lactate values were elevated. Cerebrospinal fluid (CSF) examination done on the 2nd day was normal, but serum values of calcium, magnesium, and uric acid were elevated. His sedation was stopped after 24 h, but no improvement in the sensorium was noticed thereafter. EEG was repeated and it revealed the generalized slowing. Magnetic resonance imaging (MRI) of the brain showed multiple acute to subacute infarcts in bilateral thalami, midbrain involving both cerebral peduncles, pons, right corona radiata, right posteromedial temporal, right occipital cortex, and right parahippocampal gyrus [Figure 1] and [Figure 2]. In view of the anticipation of prolonged ventilation, tracheostomy was done and he was weaned off from ventilator. Atypical cells in peripheral smear continued to increase steadily thereafter and reached more than 90% on the 7th day of admission. He developed further worsening of neurological response on the 6th day and became completely unresponsive with the development of apnea, so he was again connected to mechanical ventilator. At this stage, plain computed tomography (CT) scan of the brain revealed large hypodensity involving left temporo-parieto-occipital lobe exerting mass effect [Figure 3]. CT scan of the neck angiography showed significant reduced caliber of the left internal carotid artery, and the left anterior and middle cerebral arteries were not contrast opacified [Figure 4]. There was also evidence of eccentric filling defect in the left vertebral artery, and distal basilar artery as well as its branches was not contrast opacified. The patient received supportive management including antibiotics and antifungals because of later development of candidemia. Morphologically, the flow cytometry revealed approximately 75% blast cells and cytochemical myeloperoxidase positivity. Finding of immunophenotyping includes blast cells (moderate side scatter/dim CD 45) express CD 33 (heterogeneous), and CD 117 (partial/dim). All other markers including human leukocyte antigen – antigen D related were negative. Confirmation of the APL was done by cytogenetic analysis and demonstration of promyelocytic leukemia-retinoic acid receptor alpha rearrangement by reverse transcriptase-polymerase chain reaction in the peripheral blood cells. The patient succumbed to his illness on the 10th day of admission. | Figure 1: Axial diffusion-weighted image at the level of thalamus showing diffusion restriction in bilateral thalamus and right occipital cortex
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 | Figure 2: Axial flair image at the level of midbrain showing hyperintensity in midbrain, right parahippocampal gyrus, and right occipital cortex
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 | Figure 3: Computed tomography scan showing large infarct with mass effects
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 | Figure 4: Coronal maximum intensity projection angiogram image showing significantly reduced caliber of the left internal carotid artery
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| Discussion | | |
Central nervous system involvement is a rare complication of APL and is encountered more frequently at the relapse stage than at presentation, and only few cases have been reported in medical literature.[3],[4],[5],[6],[7] The present case is an APL initially presenting with status epilepticus.
APL is a unique disease entity associated with the distinctive morphology and chromosomal abnormality, and it is often accompanied by severe coagulopathy.[8] In an observational cohort study of 379 patients with acute leukemia, the overall incidence of thrombosis was 6.3% and it was the presenting manifestation in 3.4% of all patients, with a higher rate among patients with APL (9.6%).[9] Data regarding the risk factors for thrombosis emerged in a larger PETHEMA study. The overall incidence of thrombosis was 5.1% (39/759), with 6 out of the 26 patients who died before initiation of chemotherapy presenting with thrombotic events (three cerebral strokes, two pulmonary emboli, and one acute myocardial infarction).[10]
The patient with APL can present with ischemic stroke, and acute stroke can be associated with status epilepticus. Initial MRI of the brain done 3 days later revealed multiple acute to subacute infarct bilaterally. We initially attributed MRI changes to status epilepticus and atypical cells in the peripheral smear as reactive. We believe that the initial MRI findings were not due to status epilepticus but was the cause of status epilepticus as we could not find other reasons of status epilepticus as cerebrospinal fluid was also normal. Only when the patient stopped seizuring, atypical cells continued to rise and the patient started to have fall in hemoglobin and platelets we thought of leukemia as the underlying problem.
Blood dyscrasias should not be overlooked in patients with the acute onset of neurological symptoms.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4]
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