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Table of Contents    
Year : 2013  |  Volume : 61  |  Issue : 6  |  Page : 662-664

Acute thrombotic occlusion of internal carotid artery: A rare neurological presentation of acute promyelocytic leukaemia

1 Fortis Hospital, Noida, Uttar Pradesh, India
2 Towers Health Network, Aldan, Philadelphia 19018, USA

Date of Submission15-Sep-2013
Date of Decision16-Sep-2013
Date of Acceptance04-Feb-2013
Date of Web Publication20-Jan-2014

Correspondence Address:
Jyoti Bala Sharma
Fortis Hospital, Noida, Uttar Pradesh
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0028-3886.125281

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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 2023 Jun 6];61:662-4. Available from:


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). [1],[2],[3],[4],[5] 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 [6] acute myocardial infarction, [7] and splenic infarction, pulmonary emboli and deep vein thrombosis after the initiation of ATRA. [7],[8] Thrombotic stroke is one of the rare manifestation of APML, of the 127 patients with APML, only five had cerebral infarction. [9] 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.

 » References Top

1.Bennett JM, Catovsky D, Daniel MT, Flandrin G, Galton DA, Gralnick HR, et al. Proposals for the classification of the acute leukaemias. French-American-British (FAB) co-operative group. Br J Haematol 1976;33:451-8.  Back to cited text no. 1
2.Barbui T, Falanga A. Disseminated intravascular coagulation in acute leukemia. Semin Thromb Hemost 2001;27:593-604.  Back to cited text no. 2
3.Fenaux P, Chomienne C, Degos L. Acute promyelocytic leukemia: Biology and treatment. Semin Oncol 1997;24:92-102.  Back to cited text no. 3
4.Tallman MS, Kwaan HC. Reassessing the hemostatic disorder associated with acute promyelocytic leukemia. Blood 1992;79:543-53.  Back to cited text no. 4
5.Drapkin RL, Gee TS, Dowling MD, Arlin Z, McKenzie S, Kempin S, et al. Prophylactic heparin therapy in acute promyelocytic leukemia. Cancer 1978;41:2484-90.  Back to cited text no. 5
6.Bandyopadhyay S, Bandyopadhyay D. Acute Budd-Chiari syndrome as an initial presentation of acute promyelocytic leukemia. J Cancer Res Ther 2010;6:567-9.  Back to cited text no. 6
7.Lou Y, Mai W, Jin J. Simultaneous presentation of acute myocardial infarction and acute promyelocytic leukemia. Ann Hematol 2006;85:409-10.  Back to cited text no. 7
8.Goldschmidt N, Gural A, Ben Yehuda D. Extensive splenic infarction, deep vein thrombosis and pulmonary emboli complicating induction therapy with all-trans-retinoic acid (ATRA) for acute promyelocytic leukemia. Leuk Lymphoma 2003;44:1433-7.  Back to cited text no. 8
9.Chang H, Kuo MC, Shih LY, Wu JH, Lin TL, Dunn P, et al. Acute promyelocytic leukemia-associated thrombosis. Acta Haematol 2013;130:1-6.  Back to cited text no. 9


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