American Journal of Hematology 37:201-203 (1991)
Spinal Cord Compression Secondary to Extramedullary Hematopoiesis: A Noninvasive Management Based on MRI Hani Hassoun, Lilly Lawn-Tsao, Eugene R. Langevin, Jr., Ellen S. Lathi, and Jiri Palek Department of HematologyiOncology and Radiation Therapy, St. Elizabeth’s Hospital of Boston, Tufts University School of Medicine, Boston, Massachusetts
We report the first case of spinal cord compression secondary to extramedullary hematopoiesis in which the diagnosis was made by magnetic resonance imaging (MRI). Based on the great advantage of this imaging test that visualizes the entire spine, the noninvasive approach to such patients, using low-dose radiation therapy and guided by MRI is discussed. This management appears to be both efficacious and safe. Key words: spinal cord compression, extramedullary hematopoiesis, MRI, radiation therapy
INTRODUCTION Extramedullary hematopoiesis (EMH) is associated with many hematological conditions [ 11. A rare localization to the extradural space of the spinal canal leading to spinal cord compression (SCC) has been described [ 1101. We report here a case of spinal cord compression in a young Lebanese man with thalassemia intermedia. This is the first reported case in which the clinical diagnosis was supported by magnetic resonance imaging (MRI). Based on the great advantage of this test to visualize the entire spine, implications for the management of these patients with low-dose radiation therapy are discussed. CASE REPORT The patient is a 22-year-old Lebanese man who presented to us complaining of backache, unsteady gait, and leg weakness that had progressed over four months. He carried the diagnosis of thalassemia since age 3, when splenectomy was performed. He had received two blood transfusions. His physical examination showed a characteristic thalassemic facies, scleral icterus, hepatomegaly , and atrophic testicles. His neurologic examination revealed a spastic gait, a positive Romberg sign, increased muscle tone with hyperreflexia, and atrophy in the lower extremities. Bilateral sustained clonus and Babinski signs were noted. No sensory deficit was detected. The laboratory data were remarkable for an indirect bilirubin of 3.2, an LDH of 954, a hemoglobin A, of 3.8%, a HbF 0 1991 Wiley-Liss, Inc.
of 47%, and a ferritin level of 767. The hemoglobin was 7.2, the platelet count 900,000, and the white blood cell (WBC) count 105,000, attributable to abundant nucleated red blood cells (RBCs). An MRI of the spine (Fig. 1A) revealed multiple extradural masses extending from T3 through T10. The spinal cord appeared compressed at the level of T5-1’7. The appearance of the masses along with the known underlying hematological condition, suggested the diagnosis of EMH. Radiation therapy was initiated and he received 1350 cGy to the spine over 11 days. The radiation port was delineated based on the MRI findings, extending from T1 to L1, and measured 7 cm X 29 cm. His clinical condition improved dramatically during the treatment and over the next few weeks. An MRI 7 weeks postradiation (Fig. iB) revealed regression of the extramedullary hematopoietic tissue and relief of the spinal cord compression. Three months after radiation, he was asymptomatic, and his physical examination presented only hyperactive DTR. The complete blood count (CBC) was unchanged, and he was entered in a transfusion program aimed at keeping his hemoglobin above 10.
Received for publication November 15, 1990; accepted December 20, 1990. Address reprint requests to Dr. Hani Hassoun, Department of Hematology/Oncology, St. Elizabeth’s Hospital of Boston, 736 Cambridge Street, Boston, MA 02135.
202
Brief Report: Hassoun et al.
Fig. 1. A: MRI of the spine showing multiple nodular soft tissue masses in the retrothecal area extending from T3 to T10 (small arrows) with compression of the cord at the level of T5, 6, and 7 (large arrow). B: MRI of the spine after radiotherapy showing marked regression of the soft tissue masses and relief of the spinal cord compression.
often impossible [3,5,6]. However, some issues remain unsettled: The dose of radiation has ranged between EMH is a well-described complication of many hema- 900 cGy and 3,500 cGy with different fraction sizes tological conditions, including thalassemia [ 11, other hemo- [%lo]. The extent of the radiation field has been at best globinopathies [2,3], hemolytic anemias [4], myeloscle- delineated by indirect evidence provided by the myelorosis [ 5 ] , and other conditions [ 1,6,7]. To this date, about gram. Finally, the possible effects of radiation induced 30 cases of SCC caused by EMH have been published. In bone marrow suppression has not been well documented. In the case presented here, a noninvasive approach the earlier reports, because of the acute nature of SCC, the typical approach consisted of laminectomy following centered on MRI was taken. Based on the patient’s the myelogram. This intervention provided a diagnostic history as well as the appearance of hematopoietic tissue, and therapeutic advantage. Because of the well-known which characteristically extended through the entire thoradiosensitivity of hematopoietic tissue, radiation therapy racic spine, the diagnosis of SCC attributable to EMH was rapidly introduced as an adjunct to surgery. This was made. The patient was treated with low-dose radiaapproach has been effective in most reported cases [2-71. tion therapy ( I ,350 cGY). The radiation ports were More recently, this management has been challenged delineated by the extent of spinal cord at risk as precisely in a few reports [8-101 by a less invasive approach that visualized by MRI. His neurologic improvement was consists of establishing a clinical diagnosis based on evident by the objective regression of the spine lesions by history, computed tomography (CT) scan, and myelo- MRI. The CBC has not been affected by the therapy. gram, and administering local radiation therapy witbout This case report supports the noninvasive approach to surgery. The advantage of this approach is to avoid a SCC due to EMH using low-dose radiation therapy and major surgical intervention that presents a high risk of guided by MRI. This test was valuable to make the bleeding and where complete resection of the mass is diagnosis, to tailor accurately the radiation therapy, and DISCUSSION
Brief Report: Spinal Cord Compression Based on MRI
to confirm the successful response to treatment. As MRI is becoming available for diagnostic imaging, we believe this approach to be both efficacious and safe. ACKNOWLEDGMENTS
We are indebted to Ms. Gabriella Maitino for her help in typing this manuscript and to Ms. Joan Joos for preparing the photographic illustrations. REFERENCES 1. Papavasiliou C, Gouliamos A, Andreou J: The marrow heterotopia in
thalassemia. Eur J Radial 6:92-96, 1986. 2. Lewkow L, Shah I: Sickle cell anemia and epidural extramedullary hematopoiesis. Am J Med 76:748-751, 1984.
203
3. Chin D, Tse TM, Wong WS, Todd D, Yu CP, Mann KS: Paraparesis with hemoglobin E-P thalassemia. Aust NZ J Med 15:263-264, 1985. 4. Ross P, Logan W: Roentgen findings in extramedullary hematopoiesis. AJR 106:604-613, 1969. 5 . Oustwani MB, Kurtides ES, Christ M, Ciric I: Spinal cord compression with paraplegia in myelofibrosis. Arch Neural 37:389-390, 1980. 6. Heffez D, Sawaya R, Udvarhelyi G, Mann R: Spinal epidural extramedullary hematopoiesis with cord compression in a patient with refractory sideroblastic anemia. J Neurosurg 57:399406, 1982. 7. Rice GPA, Assis LJP, Ban RM, Ebers GC: Extramedullary hematopoiesis and spinal cord compression complicating polycythemia rubra vera. Ann Neural 7231-84, 1980. 8. Issaragrisil S, Piankijagum A, Wasi P: Spinal cord compression in thalassemia. Arch Intern Med 141:1033-1036, 1981. 9. Papavasiliou C, Sandilos P: Effect of radiotherapy on symptoms due to heterotopic marrow in P thalassemia. Lancet 1:14, 1987. 10. Jackson DV, Randall ME, Richards F 11: Spinal cord compression due to extramedullary hematopoiesis in thalassemia: Long term follow-up after radiotherapy. Surg Neural 29:389-392, 1988.
Spinal Cord Compression Secondary to Extramedullary Hematopoiesis: A Noninvasive Management Based on MRI Hani Hassoun, Lilly Lawn-Tsao, Eugene R. Langevin, Jr., Ellen S. Lathi, and Jiri Palek Department of HematologyiOncology and Radiation Therapy, St. Elizabeth’s Hospital of Boston, Tufts University School of Medicine, Boston, Massachusetts
We report the first case of spinal cord compression secondary to extramedullary hematopoiesis in which the diagnosis was made by magnetic resonance imaging (MRI). Based on the great advantage of this imaging test that visualizes the entire spine, the noninvasive approach to such patients, using low-dose radiation therapy and guided by MRI is discussed. This management appears to be both efficacious and safe. Key words: spinal cord compression, extramedullary hematopoiesis, MRI, radiation therapy
INTRODUCTION Extramedullary hematopoiesis (EMH) is associated with many hematological conditions [ 11. A rare localization to the extradural space of the spinal canal leading to spinal cord compression (SCC) has been described [ 1101. We report here a case of spinal cord compression in a young Lebanese man with thalassemia intermedia. This is the first reported case in which the clinical diagnosis was supported by magnetic resonance imaging (MRI). Based on the great advantage of this test to visualize the entire spine, implications for the management of these patients with low-dose radiation therapy are discussed. CASE REPORT The patient is a 22-year-old Lebanese man who presented to us complaining of backache, unsteady gait, and leg weakness that had progressed over four months. He carried the diagnosis of thalassemia since age 3, when splenectomy was performed. He had received two blood transfusions. His physical examination showed a characteristic thalassemic facies, scleral icterus, hepatomegaly , and atrophic testicles. His neurologic examination revealed a spastic gait, a positive Romberg sign, increased muscle tone with hyperreflexia, and atrophy in the lower extremities. Bilateral sustained clonus and Babinski signs were noted. No sensory deficit was detected. The laboratory data were remarkable for an indirect bilirubin of 3.2, an LDH of 954, a hemoglobin A, of 3.8%, a HbF 0 1991 Wiley-Liss, Inc.
of 47%, and a ferritin level of 767. The hemoglobin was 7.2, the platelet count 900,000, and the white blood cell (WBC) count 105,000, attributable to abundant nucleated red blood cells (RBCs). An MRI of the spine (Fig. 1A) revealed multiple extradural masses extending from T3 through T10. The spinal cord appeared compressed at the level of T5-1’7. The appearance of the masses along with the known underlying hematological condition, suggested the diagnosis of EMH. Radiation therapy was initiated and he received 1350 cGy to the spine over 11 days. The radiation port was delineated based on the MRI findings, extending from T1 to L1, and measured 7 cm X 29 cm. His clinical condition improved dramatically during the treatment and over the next few weeks. An MRI 7 weeks postradiation (Fig. iB) revealed regression of the extramedullary hematopoietic tissue and relief of the spinal cord compression. Three months after radiation, he was asymptomatic, and his physical examination presented only hyperactive DTR. The complete blood count (CBC) was unchanged, and he was entered in a transfusion program aimed at keeping his hemoglobin above 10.
Received for publication November 15, 1990; accepted December 20, 1990. Address reprint requests to Dr. Hani Hassoun, Department of Hematology/Oncology, St. Elizabeth’s Hospital of Boston, 736 Cambridge Street, Boston, MA 02135.
202
Brief Report: Hassoun et al.
Fig. 1. A: MRI of the spine showing multiple nodular soft tissue masses in the retrothecal area extending from T3 to T10 (small arrows) with compression of the cord at the level of T5, 6, and 7 (large arrow). B: MRI of the spine after radiotherapy showing marked regression of the soft tissue masses and relief of the spinal cord compression.
often impossible [3,5,6]. However, some issues remain unsettled: The dose of radiation has ranged between EMH is a well-described complication of many hema- 900 cGy and 3,500 cGy with different fraction sizes tological conditions, including thalassemia [ 11, other hemo- [%lo]. The extent of the radiation field has been at best globinopathies [2,3], hemolytic anemias [4], myeloscle- delineated by indirect evidence provided by the myelorosis [ 5 ] , and other conditions [ 1,6,7]. To this date, about gram. Finally, the possible effects of radiation induced 30 cases of SCC caused by EMH have been published. In bone marrow suppression has not been well documented. In the case presented here, a noninvasive approach the earlier reports, because of the acute nature of SCC, the typical approach consisted of laminectomy following centered on MRI was taken. Based on the patient’s the myelogram. This intervention provided a diagnostic history as well as the appearance of hematopoietic tissue, and therapeutic advantage. Because of the well-known which characteristically extended through the entire thoradiosensitivity of hematopoietic tissue, radiation therapy racic spine, the diagnosis of SCC attributable to EMH was rapidly introduced as an adjunct to surgery. This was made. The patient was treated with low-dose radiaapproach has been effective in most reported cases [2-71. tion therapy ( I ,350 cGY). The radiation ports were More recently, this management has been challenged delineated by the extent of spinal cord at risk as precisely in a few reports [8-101 by a less invasive approach that visualized by MRI. His neurologic improvement was consists of establishing a clinical diagnosis based on evident by the objective regression of the spine lesions by history, computed tomography (CT) scan, and myelo- MRI. The CBC has not been affected by the therapy. gram, and administering local radiation therapy witbout This case report supports the noninvasive approach to surgery. The advantage of this approach is to avoid a SCC due to EMH using low-dose radiation therapy and major surgical intervention that presents a high risk of guided by MRI. This test was valuable to make the bleeding and where complete resection of the mass is diagnosis, to tailor accurately the radiation therapy, and DISCUSSION
Brief Report: Spinal Cord Compression Based on MRI
to confirm the successful response to treatment. As MRI is becoming available for diagnostic imaging, we believe this approach to be both efficacious and safe. ACKNOWLEDGMENTS
We are indebted to Ms. Gabriella Maitino for her help in typing this manuscript and to Ms. Joan Joos for preparing the photographic illustrations. REFERENCES 1. Papavasiliou C, Gouliamos A, Andreou J: The marrow heterotopia in
thalassemia. Eur J Radial 6:92-96, 1986. 2. Lewkow L, Shah I: Sickle cell anemia and epidural extramedullary hematopoiesis. Am J Med 76:748-751, 1984.
203
3. Chin D, Tse TM, Wong WS, Todd D, Yu CP, Mann KS: Paraparesis with hemoglobin E-P thalassemia. Aust NZ J Med 15:263-264, 1985. 4. Ross P, Logan W: Roentgen findings in extramedullary hematopoiesis. AJR 106:604-613, 1969. 5 . Oustwani MB, Kurtides ES, Christ M, Ciric I: Spinal cord compression with paraplegia in myelofibrosis. Arch Neural 37:389-390, 1980. 6. Heffez D, Sawaya R, Udvarhelyi G, Mann R: Spinal epidural extramedullary hematopoiesis with cord compression in a patient with refractory sideroblastic anemia. J Neurosurg 57:399406, 1982. 7. Rice GPA, Assis LJP, Ban RM, Ebers GC: Extramedullary hematopoiesis and spinal cord compression complicating polycythemia rubra vera. Ann Neural 7231-84, 1980. 8. Issaragrisil S, Piankijagum A, Wasi P: Spinal cord compression in thalassemia. Arch Intern Med 141:1033-1036, 1981. 9. Papavasiliou C, Sandilos P: Effect of radiotherapy on symptoms due to heterotopic marrow in P thalassemia. Lancet 1:14, 1987. 10. Jackson DV, Randall ME, Richards F 11: Spinal cord compression due to extramedullary hematopoiesis in thalassemia: Long term follow-up after radiotherapy. Surg Neural 29:389-392, 1988.
