Neurol India Home 

Year : 2013  |  Volume : 61  |  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

Correspondence Address:
Jyoti Bala Sharma
Fortis Hospital, Noida, Uttar Pradesh

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;61:662-664

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 2022 Aug 11 ];61:662-664
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Full Text


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}{Figure 2}

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.


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