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J R Soc Med. 2004 March; 97(3): 125–127.
PMCID: PMC1079323

Unusual presentations of acute lymphoid malignancy in children

Reuben Antony, MRCP, Derek Roebuck, FRCR MRCPCH,1 and Ian M Hann, FRCPCH FRCPath

Acute paraplegia in childhood requires immediate diagnostic evaluation and treatment. Lymphoblastic leukaemia/lymphoma is a cause easily missed if signs of bone marrow dysfunction are absent.

CASE HISTORIES

Case 1

A 4-year-old boy complained of knee pain and had increasing difficulty with weight-bearing for five weeks. A week before admission he stopped walking completely and the pain spread to involve most of his bones, especially his spine and ribs. He also described numbness over his thighs and strained to pass urine and stool. At this time an antero-posterior X-ray of his spine showed no abnormality. Intermittent spinal traction for five days was of no benefit, and on the day before admission an MRI of his spine was done. This showed a paraspinal mass from T2 to T8 passing through the intervertebral foramina into the spinal epidural space and compressing the cord (Figure 1). The radiological differential diagnosis was neuroblastoma or non-Hodgkin lymphoma. After starting dexamethasone for tumour-related cord compression he was transferred to our centre for further management. The relevant physical findings were hepatomegaly, decreased power and sensation in the lower limbs, and bilateral calf wasting more pronounced on the left. Though his full blood count was normal, leukaemic blast cells were seen on a peripheral blood film. The bone marrow contained 61% blast cells and immunophenotyping was positive for CD10, CD19 and CD79a. Acute lymphoblastic leukaemia was diagnosed and he was started on treatment according to the UK ALL 2003 protocol. Within a week his lower limb function was improving.

Figure 1
Sagittal T1 weighted MR image showing epidural mass in the thoracic spinal canal (between arrows)

Case 2

A girl of 4 had experienced fourteen days of progressive lower limb weakness following two episodes of upper respiratory tract infection. The initial clinical diagnosis was Guillain–Barré syndrome and she was treated with intravenous immunoglobulin. An electromyogram three days after admission was normal and there was no response to immunoglobulin. MRI of her brain and spine then showed a mass from T2 to T10 compressing the spinal cord (Figure 2) and she was started on dexamethasone. On transfer to our centre she had flaccid paralysis of both lower limbs and absent deep tendon reflexes, but bladder and bowel function and lower limb sensation were intact. The white cell count was not raised and no blasts were seen on a peripheral smear. The initial diagnosis was neuroblastoma and she was treated with carboplatin, vincristine and etoposide. On histological examination the cellular elements of the mass were strongly positive for CD79a, TDT and CD10 and the diagnosis was revised to pre B cell lymphoblastic lymphoma. Bone marrow was normal. She was started on treatment according to the MRC ALL 97 protocol and a repeat MRI 2 days later showed almost complete disappearance of the tumour mass (Figure 2b). After intensive physiotherapy for eight months she was walking with support.

Figure 2
Sagittal T2 weighted MR images in case 2. (a) Image at diagnosis shows a large epidural mass (between arrows) causing severe compression of the thoracic spinal cord. There are patchy signal changes in the vertebral bodies, presumably related to leukaemic ...

COMMENT

The common causes of acute lower limb paraplegia in childhood are spinal cord tumours, epidural abscess, haematoma and transverse myelitis. Guillain–Barré syndrome can present as an ascending lower limb paralysis. Back pain in children is unusual and should always be investigated to rule out serious underlying illness. In both the children we are reporting, neurological symptoms progressed considerably before appropriate treatment was started. While a neurological examination can help determine the level of involvement of the spinal cord, imaging with MRI is necessary both for diagnosis and for planning of further investigations and treatment. Leukaemic/lymphomatous masses spread via lymphatics and through intervertebral foramina and cause cord compression before bone destruction.1 So while plain X-rays are useful in detecting bony involvement in primary or metastatic solid tumours they can be falsely reassuring in lymphoid malignancies.2 In the absence of bone marrow or peripheral blood involvement a biopsy of the spinal mass may be necessary. Examination of cerebrospinal fluid may help with the diagnosis but a lumbar puncture may not be safe and a neurosurgical opinion should first be sought. Acute lymphoid malignancies are common in childhood. Patients with acute lymphoblastic leukaemia/lymphoma usually present with infections or signs of bone marrow dysfunction such as anaemia and bruising. An initial presentation with lower limb weakness is exceedingly rare. In one series of 1412 children with acute lymphocytic leukaemia only 5 (0.35%) presented with a spinal mass.2 Early chemotherapy and physical rehabilitation offer the best chance of cure and functional recovery.

Acknowledgments

We thank the Department of Medical Illustration, Great Ormond Street Hospital for Children, for their help.

References

1. Ch'ien LT, Kalwinsky DK, Peterson G, et al. Metastatic epidural tumors in children. Med Pediatr Oncol 1982;10: 455–62 [PubMed]
2. Pui C-H, Dahl GV, Hustu HO, Murphy SB. Epidural spinal cord compression as the initial finding in childhood acute leukemia and non Hodgkin lymphoma. J Pediatr 1985;106: 788–92 [PubMed]

Articles from Journal of the Royal Society of Medicine are provided here courtesy of Royal Society of Medicine Press