Unusual presentation of acute myeloid leukemiaPD Anjaria, UG Gadgil, SM Gaikwad
Department of Medicine, K.E.M. Hospital and Seth G.S. Medical College, Parel, Bombay-400012, India
A case of acute myeloid leukemia with unusual- presentation of compression paraplegia of acute onset, with extensive epidural plaque is reported. Mechanisms of neurologic manifestations, especially involvement of the spinal cord, of leukemia, are discussed.
"Neurological complications, apart from those due to cerebral haemorrhage, are sufficiently rare in acute leukemia for them to be reported as curiosities. An awareness of them is important, though they are not likely to be met with frequently." (Gunz and Baikie, 1974).
Leukemia may present with meningitis, encephalitis, hemiplegia, paraplegia, convulsions or involvement of either cranial or peripheral nerves, motor or sensory. A case of acute myeloid leukemia presenting as compression paraplegia of acute onset is presented here.
A 16 years old boy was transferred from another hospital with history of weakness of both lower limbs and inability to walk for 10 days. He had backache for 3 days prior to weakness. There was no history of trauma to back. He did not have root pains. There was no bladder and bowel involvement.
He was an averagely built and nourished person with mild pallor of nails and conjunctiva. Lymphadenopathy, oedema of legs and bony tenderness were absent.
Central nervous system examination revealed, marked hypotonia with complete loss of movements of both lower limbs. All sensations were markedly impaired below T 8 level. Deep reflexes in upper limbs were normally elicitable. Knee jerks were barely elicitable, while ankle and planter reflexes were absent. Abdominal and cremasteric reflexes were also absent.
Examination of abdomen revealed a hard lump, about 5 c ms. x 5 cms. in
Per rectal examination showed hard nodular masses in anterior rectal wall.
Haemoglobin and white cell counts done at the other hospital were within normal limits; sedimentation rate was 70 mm. at the end of one hour. CSF examination showed proteins to be 900 mg%, with no other abnormal findings. Myelogram showed a block at T 12 , level.
Cisternal myelogram was done and showed upper level of block at T 4 level indicating the extent of the block from T 4 -T 12 level. CSF obtained from this site was within normal limits. Haemogram on admission, showed Haemoglobin of 4.6 gm%, packed cell volume 21%, sedimentation rate 140 mm. at the end of 1 hour, WBC count 27000/cmm. with plenty of myeloblasts in peripheral smear. Bone marrow smear was full of blast cells.
With these findings a diagnosis of acute myeloid leukemia was established. Patient expired within 3 weeks of diagnosis.
Involvement of the nervous system by leukemia may take many forms. The first known account was by Allan Burns, and was published in 1811. His case 16 usually regarded as one of Chloroma, arising in the bones of skull (Gunz and Baikie, 1974).
Careful examination of anatomical, pathological and clinical aspects of nevous system involvement in leukemia has led to the recognition of several clinical pictures, known collectively as neuroleukemic affections (d'Ermo and Levi, 1972). Neurological involvement is more common in acute than in chronic leukemia and commoner in acute lymphatic than in acute myeloid type. Infants are more frequently afflicted with neurological complications than children or adults and males are more commonly affected than females.
Neuropathology of leukemia discloses chiefly:
(A) Impaction of blood vessels with leukemic cells.
(B) Infiltration of blood vessel walls with leukemic cells. The blood vessels may be in meninges, brain parenchyma or peripheral nerves (Firkin and Moore, 1960; Moore et al, 1960; Wells and Silver, 1957).
Thus, vascular stasis, ischaemia, occlusion, haemorrhage and edema occur in the affected tissues.
Leukemia shows clinical neurologic involvement as intracranial haemorrhage, non-haemorrhagic cerebral involvement, meningeal leukemia or infection Fritz et al, 1959; Gilbert and Rice, 1957; Hunt et al, 1959 and Sullivan, 1957). Rarely, spinal root and cord syndromes, cranial nerve palsies and peripheral nerve lesions may manifest.
Leukemias give rise to spinal cord involvement due to vertebral destruction, epidural leukemic masses, haemorrhage, myelomalacia or demyelinization. In a series reported by Wilhyde et al, spinal cord involvement was the presenting manifestation of 'leukemia in 28 out of 42 cases; progressive paraplegia was the commonest feature (Wilhyde et al, 1963).
Occasionally, chloroma of vertebra is the causative factor giving rise to compression of spinal cord. Chloroma is rare feature of acute or subacute myeloid leukemia, seen in children or young adults, affecting males 2:1. It presents a--single or multiple green-hued tumour masses, consisting of immature leukemic cells and pigments, growing in subperiosteum or marrow. Such masses commonly appear in orbit or any where on skull, including air sinuses and mastoid. Spine, sacrum and ribs are not unusual sites. X-Ray discloses local erosion and cortical thinning. These masses never invade dura but may compress at extradural or peripheral nerve sites (Aita, 1964; Rao et al, 1962).
Thanks are due to the Dean, K. E. M.Hospital, Bombay-12 for permission to report this case.