Epidural spinal cord compression as initial clinical presentation of an acute myeloid leukaemia: case report and literature review
© The Author(s) 2016
Received: 11 September 2015
Accepted: 20 November 2015
Published: 18 January 2016
Epidural localization of myeloid leukaemia is rarely reported. Spinal cord compression as an initial presentation of acute myeloid leukaemia is extremely rare. This is a report of a 17-year-old black boy who presented to emergency department with neurological symptoms of spinal cord compression. Imaging modalities showed multiple soft tissue masses in the epidural space. After surgical treatment, histopathological examination of the epidural mass showed myeloid leukaemia cells infiltration. Literature review on Medline and “scholar Google” database was done. The characteristics and management of extra-medullary leukaemia are discussed. Granulocytic sarcoma, myeloid sarcoma or chloroma with acute myeloid leukaemia should be considered as part of epidural spinal cord compression. Therefore surgery is indicated on an emergent basis.
KeywordsSpinal cord compression Initial epidural localization Acute myeloid leukaemia
Acute myeloid leukaemia corresponds to malignant monoclonal proliferation of medullary blastic myeloid cells with an interrupted differentiation . Clinically acute myeloid leukaemia will result in adenopathy and hepatosplenic syndrome associated with bone marrow failure syndrome. Extra nodal involvement is rare and mostly made of frequently observed forms of lymphoblastic leukemia in children. These attacks represent 2 to 8 % of acute myeloid leukaemia. Epidural localization is unusual and is a rare cause of spinal cord compression. Spinal cord compression as an initial presentation of acute myeloid leukaemia is extremely rare. In this study, we did not consider Spinal cord compression as the initial presentation of acute biphenotypic leukaemia . The authors reported a case of acute myeloid leukaemia revealed by thoracic spinal cord compression. Literature review follows the case report.
Literature review from 2005 to 2015. characterization of acute myeloid leukemia associated to spinal cord compression
Histology and immunohistochemistry
Delay and diagnosis treatement and outcome
Meltzer et al. 2005 
-Myeloblastic Acute myeloid leukemia
-decreased sensation to vibration and light touch over the left leg
-decreased sensation to pain and temperature in the right leg
-paravertebral lesions with spinal
-posterior epidural and prevertebral mass
WBC 12,800/mm3, HB 9.4 g/dl, platelet 74,800/mm3.
-lactate dehydrogenase was 570 IU/l,
-Laminectomie + Chemotherapy + spinal radiation
-Total Neurological improvement after 6 month
-well more than 18 months of therapy
Ghalaut et al. 2005. 
Granulocytic sarcoma due to acute myeloid leukemia
-numbness of bilateral legs-sensory loss up to mid thoracic region anemia
-Epidural mass extending from T4-T6 with marrow signal alteration involving T1 and T5 to T7
- surgical excision + chemotherapy
Amalraj et al. 2009 
Granulocytic sarcoma promyelocytic leukemia AML M3
-weakness of both lower limbs
-numbness of both lower limbs
-back pain for the last
hypointense in T1 hyperintense in T2 dorsally compressing the spinal cord
Hemoglobin 13 gm%, total count 8,400, polymorph 60 %, and lymph 40 %, with high erythrocyte.
-paraplegia improved with radiotherapy and chemotherapy
RBC mild hypochromic microcytic, WBC count normal predominantly blast cells with high nuclear cytoplasmic ratio, moderate cytoplasm, hyperchromatic nuclei with multiple nucleoli, platelet count decreased.
Olcay et al. 2009 
Cytogenetic evaluation could not be done.
acute myeloblastic leukemia
Lumbar (L3) -Conus medullaris
-left maxillary mass
-Conus medullaris syndrome
Cauda equine nerve roots were thickened and clumped in the left anterolateral side of the spinal canal.
-Chemotherapy + triple intratecal therapy
-Symptom partially resolved died because of pneumonia and disseminated intravascular coagulation
CD45:95.15 %, CD15: 35.56 %, CD33:77.57 %, CD117:79.3 %, CD34:43.38 %, CD13:29.32 %.
WBC : 7.4 × 109/mm3
Lactate dehydrogenase: 691 U/l
HLA-DR:86, 4 %, CDw65:23.9 %, CD19:49.54 % CD20:21.65 %, CD14:3.14 %.
Yin et al. 2010 
myeloid sarcoma with acute myeloid leukemia
-low back pain
-numbness of his legs
-hypoesthesia below T12 paraparesis
-Posterior epidural mass T12-L1
White blood cell 3,900/mm3
- hemoglobin :11.0 g/dL
-hematocrit of 38.2 %.
-Decompression with T12-L1 laminectomy and tumor resection.
CD68, CD45, CD43, CD117 and lysozyme but not of MPO, CD20
- anti-AML therapy, but died of sepsis
Bittencourt et al. 2011 
-Acute promyelocytic leukemia presenting as extradural mass
-PCR for the PML-RARα gene was positive
-Progressive fatigue, pain legs weakness Paraparesis
- Gingival hemorrhage, hepatomegaly
Posterolateral extradural mass from T6-T8 with medullar compression
delais4 months/chemotherapy (daunorubicin) + radiotherapy/Evolution: no neurologic improvement + died of sepsis
INR: 1.86 (N:0,8-1,2)
Uric acid and lactate dehydrogenase were both elevated
Kyaw et al. 2012 
-Myeloid sarcoma: acute promyelocytic leukemia
-CD33, CD117, CD64, CD34 and cytoplasmic MPO were presented Reverse transcriptase-polymerase chain reaction showed BCR1-type PML-RARα fusion copies
T2-T4 and T12 – L2
Progressive back pain and bilateral leg weakness: -paraparesis Loss of pain and sensory perception
Multiple hyperintense T12,L1,L2, L4 and L5 vertebral bodies and sacrum
-Chemotherapy: retinoic acid + ida rubicin
-Neurological improvement -Good remission
Platelet : 102.109/L-1
Intraspinal extradural masses L4 and L5 vertebral bodies and sacrum
Intraspinal extradural masses located fromT2 toT4 and T12 to L2.
Ben et al. 2013 
karyotype: 46, XY, t (8, 21) (q22; q22) / 46, XY
CD3, CD20 = -
-positive for myeloperoxidase
acute myeloblastic leukaemia (AML) of the French-American-British M2 subtype
T4/ T7, T1-T2
Progressive paraplegia. urinary retention
intermediate in T1, hyperintense in T2
hemoglobin 8 g/dL-1, white blood cell count 3100/mm3, platelet count 44000/mm3 and 3 % neutrophils, 44 % lymphocytes and 44 % .
laminectomy + tumour was totally removed
-rubidomycine (45 mg/m2 daily for 3 days) and cytosine arabinoside (200 mg/m2 continuous infusion for 7 days).
- complete remission
Krishnamurthy et al. 2014 
Granulocytic sarcoma associated with leukaemia,
- acute myeloblastic leukaemia (AML) of the French-American-British M2 subtype.
Low backaches follow proptosis, spinal cord causing significant compression of the spinal cord.
Multiple midline fusiform extra-dural masses which are iso- intense to cord on T1W images seen extending from C3- C7, D3-D5, D11-D12 & S1-S2 are seen posteriorly
Hb : 7.2 g/dl, 3 RBC 2.5 million/mm3,TC : 11200 cells/mm3, with a differential ount of N 41 %, L-28 %, E-01 %, M-30 %, 3ESR 120 mm/hr, Platelet count: 51,000/mm3, PT/INR –
- dimorphic anemia, thrombocytopenia and myeloblasts
-radiotherapy and chemotherapy
-Cytogenetic evaluation could not be done.
- type 2 leukemia
hypointense lesion on T1 weighted sequence isointense T2 weighted sequence enhanced on T1 with gadolinium. (epidural mass (hyperintense)
blood count revealed leucocytosis with a major white blood cell count 200,000 / mm3, with 77 % blasts, hemoglobin: 10.9 g dl; platelet count: 106 × 109 L-1
-Died one week after diagnosis
This case associated with the literature review has shown that granulocytic sarcoma with acute myeloid leukaemia should be considered as part of epidural spinal cord compression .it might be the initial sign of epidural spinal cord compression . Therefore surgery is indicated in emergency. Chemotherapy and radiotherapy are secondly introduced according to the diagnosis.
We thank Sir Djo BI doctor Alban Mbende Professor koffi Gustave in haematology department in yopougon and Dr Doukouré Brahim Histopatology department for their help.
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