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|LETTER TO EDITOR
|Year : 2013 | Volume
| Issue : 6 | Page : 662-664
Acute thrombotic occlusion of internal carotid artery: A rare neurological presentation of acute promyelocytic leukaemia
Jyoti Bala Sharma1, Sushil Kumar Gupta2
1 Fortis Hospital, Noida, Uttar Pradesh, India
2 Towers Health Network, Aldan, Philadelphia 19018, USA
|Date of Submission||15-Sep-2013|
|Date of Decision||16-Sep-2013|
|Date of Acceptance||04-Feb-2013|
|Date of Web Publication||20-Jan-2014|
Jyoti Bala Sharma
Fortis Hospital, Noida, Uttar Pradesh
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Sharma JB, Gupta SK. Acute thrombotic occlusion of internal carotid artery: A rare neurological presentation of acute promyelocytic leukaemia. Neurol India 2013;61:662-4
|How to cite this URL:|
Sharma JB, Gupta SK. Acute thrombotic occlusion of internal carotid artery: A rare neurological presentation of acute promyelocytic leukaemia. Neurol India [serial online] 2013 [cited 2019 Dec 8];61:662-4. Available from: http://www.neurologyindia.com/text.asp?2013/61/6/662/125281
Acute promyelocytic leukemia (APML), classified as acute myeloid leukaemia-M3 (AML-M3) type by French-American-British group, is characterized by clone proliferation and accumulation of promyelocytes with characteristic morphology, fibrinolysis, proteolysis and disseminated intravascular coagulation (DIC). ,,,, All trans retinoic acid (ATRA) treatment has improved outcomes; yet hemorrhagic complications remain the most frequent cause of mortality. However, APML-associated thrombosis is relatively under appreciated.
A 35-year-old woman presented with 2 days history of fluctuating left side weakness, progressed to complete paralysis on the day of presentation. On examination, patient was conscious, oriented, afebrile and hemodynamically stable. Neurological examination revealed upper motor neuron facial palsy and left hemiplegia (power 0/5 in upper limb and 2/5 in lower limb).
Laboratory investigation revealed Hb 5.6 g/dL, white blood cells 3.0 thousand/μL, platelet 94 thousand/μL. Coagulation profile and blood chemistry were normal. Echocardiography revealed normal ejection fraction, no vegetation, clot or evidence of valvular disease. Magnetic resonance imaging brain revealed right front-parietal acute infarct [Figure 1]a and b. Magnetic resonance-angiography neck vessels revealed a long segment narrowing of right internal carotid arteries [Figure c and d]. Peripheral smear examination showed pancytopenia with blast cells. Bone marrow aspiration and biopsy confirmed the diagnosis of AML-M3 [Figure 2]. Immuno-phenotyping test were also positive for CD13, CD33 and CD117 and negative for CD15, CD7 and CD34. Reverse transcriptase polymerase chain reaction showed positivity of breakpoint cluster region 1 isoform of promyelocytic leukemia retinoic acid receptor alpha fusion transcript in bone marrow cells and hence ATRA was initiated at a dose of 45 mg/m 2 to which patient responded well. The patient is in remission with residual paralysis in left hand with modified Rankin scale score of 3 and Barthel index of 40.
|Figure 1: (a) Magnetic resonance image brain fluid-attenuated inversion recovery sequence showing right front parietal infarct. (b) Magnetic resonance image diffusion - weighted imaging sequence showing acute infarct in the right front parietal region. (c) Magnetic resonance angiography showing obliteration of the right internal carotid artery (ICA) at the origin. (d) Magnetic resonance angiography showing non visualization of right ICA in circle of Willis|
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|Figure 2: Photomicrograph of bone marrow aspirate smear (Giemsa stain magnification × 40) showing promyelocytes and blast cells with bundles of Auer rods More Details and atypical mitotic figure|
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Association of APML with thrombosis in various organs has been mentioned in literature and these include Budd Chairi syndrome  acute myocardial infarction,  and splenic infarction, pulmonary emboli and deep vein thrombosis after the initiation of ATRA. , Thrombotic stroke is one of the rare manifestation of APML, of the 127 patients with APML, only five had cerebral infarction.  Chemotherapy in APML causes the release of large amounts of procoagulants, thus predisposing to DIC. The advent of ATRA has improved outcome as it acts by the maturation of blast cells in to polymorphonuclear leukocytes and not by killing cells hence reducing the incidence of DIC.
The unusual features in this patient are thrombotic stroke as the presenting feature in APML and thrombosis event occurring with pancytopenia, which is commonly associated with hemorrhagic events. We have not instituted fibrinolytic therapy along with ATRA in this patient. The most gratifying aspect in this patient is good neurological outcome.
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[Figure 1], [Figure 2]