J Korean Med Sci.  2007 Dec;22(6):1090-1093. 10.3346/jkms.2007.22.6.1090.

Chronic Idiopathic Myelofibrosis Presenting as Cauda Equina Compression due to Extramedullary Hematopoiesis: A Case Report

• Goh DH
• Lee SH
• Cho DC
• Park SH
• Hwang JH
• Sung JK

• KMID: 1785777
• DOI: http://doi.org/10.3346/jkms.2007.22.6.1090

Abstract

Extramedullary hematopoiesis (EMH) is occasionally reported in idiopathic myelofibrosis and is generally found in the liver, spleen, and lymph nodes several years after diagnosis. Myelofibrosis presenting as spinal cord compression, resulting from EMH tissue is very rare. A 39-yr-old man presented with back pain, subjective weakness and numbness in both legs. Sagittal magnetic resonance imaging showed multiple anterior epidural mass extending from L4 to S1 with compression of cauda equina and nerve root. The patient underwent gross total removal of the mass via L4, 5, and S1 laminectomy. Histological analysis showed islands of myelopoietic cells surrounded by fatty tissue, consistent with EMH, and bone marrow biopsy performed after surgery revealed hypercellular marrow and megakaryocytic hyperplasia and focal fibrosis. The final diagnosis was chronic idiopathic myelofibrosis leading to EMH in the lumbar spinal canal. Since there were no abnormal hematological findings except mild myelofibrosis, additional treatment such as radiothepary was not administered postoperatively for fear of radiotoxicity. On 6 month follow- up examination, the patient remained clinically stable without recurrence. This is the first case of chronic idiopathic myelofibrosis due to EMH tissue in the lumbar spinal canal in Korea.

Keyword

Hematopoiesis, Extramedullary; Myelofibrosis; Spinal Canal

MeSH Terms

Adult
*Cauda Equina
Chronic Disease
*Hematopoiesis, Extramedullary
Humans
Male
Primary Myelofibrosis/*complications
Spinal Cord Compression/*etiology

Figure

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  Fig. 1 Epidural extramedullary hematopoiesis in idiopathic myelofibrosis. Sagittal T2 (A), T1-weighted (B), and enhanced magnetic resonance (MR) images (C) of the lumbar spine revealing a posterior L4-S1 epidural mass causing spinal cord compression, as a homogenous isointense signal on T1-weighted and slightly hyperintense signal on T2-weighted images compared with adjacent red bone marrow, which were well enhanced with contrast (Gadolinium). Axial T2- weighted images (D) reveal an extradural mass extending into the neural foramen bilaterally on the L5 vertebral level.

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  Fig. 2 Intraoperative photograph showing the discrete, flesh-colored, elastic, and extradural mass (arrowheads) between lumbar nerve roots (arrows).

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  Fig. 3 Microscopic photograph of the extradural mass revealing cellular tissue of hematopoietic origin with small megakaryocytes (H&E, ×400).

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