Spinal cord compression by extramedullary hemopoietic tissue in sickle cell anemia Case report
ABDEL A. AMMOUMI, M.D., JOANNA H. SHER, M.D., AND DANIEL SCHMELKA, M.D. Departments of Neurology, Pathology, and Neurosurgery, State University of New York, Downstate Medical Center, Brooklyn, New York The authors report a patient with sickle cell anemia who suffered from paraplegia of 18 months duration due to spinal cord compression by a hemopoietic mass. Recovery following removal of the mass was complete. KEy WORDS 9 sickle cell anemia 9 paraplegia 9 spinal cord compression 9 extramedullary hemopoietic tissue
C a s e Report
History. The patient was known to have sickle cell anemia and had suffered episodes of abdominal and joint pain in the past. He stated that gradually, over a period of 2 months, he had developed weakness in both legs, more severe on the right. This was followed by numbness, beginning in the feet, and then spreading up to the waist. He also experienced spontaneous jerking movements in his legs from time to time, mostly in the right leg. Two months after the onset of weakness the patient was unable to walk. At the time of admission he had been confined to a wheel chair for 18 months. There was no weakness or sensory loss in the arms, and sphincter function was preserved.
This 28-year-old black man was hospitalized because of spastic paraplegia of 18 months duration.
Examination. Neurological examination showed comp!ete spastic paraplegia. There was decreased appreciation of touch, pin-
HE protean neurological manifestations of sickle cell anemia are generally caused by thrombosis with resulting infarction or hemorrhage. 1'4,5,7 Both the degeneration of ganglion cells as well as posterior and lateral column demyelination have been observed in the spinal cord in sickle cell disease. 3'6 The following case of paraplegia secondary to spinal cord compression by a mass of extramedullary hemopoietic tissue (blood cell production outside the bone marrow) illustrates yet another way in which neural tissue may be damaged in sickle cell anemia.
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A. A. Ammoumi, J. H. Sher and D. Schmelka diomegaly. The lungs were clear. The spinal fluid drawn during the myelography procedure was slightly xanthochromic with a protein level of 86 mg%, glucose 75 mg%, and no cells. The myelogram showed an extradural pressure defect from the level of T-3 to T-5. Operation. A laminectomy was performed, and a dark, red mass measuring 2.0 X 1.0 • 0.5 cm found beneath the lamina and compressing the dura was removed. Microscopically, the tissue was basically fat, containing many blood vessels, and densely infiltrated by blood-forming elements. These were mainly of the erythroid series. Many megakaryocytes were present, as well as some foci of white-cell-forming tissue. The overall microscopic picture resembled hypercellular bone marrow with extremely severe erythroid hyperplasia (Fig. 1). Intravascular sickling of red cells was noted incidentally. Postoperative Course. A few days after surgery, the patient started to move his toes, but the paraplegia and the same sensory loss were still present. Motor and sensory function improved very gradually. In 5 months, the F1G. 1. Microscopic appearance of the extra- patient was walking with the help of two dural mass, showing hemopoietic tissue and meg- canes, and 3 months later he was walking unakaryocytes. H & E, • 250. supported with a normal gait. prick, and vibration and position sense below the T-7 dermatome. Tendon reflexes were normal and symmetrical in the arms, but hyperactive in the legs, with bilateral sustained clonus. There was no plantar response. Mental status, cranial nerve function, and coordination of the arms were all within normal limits. The optic fundi were normal. Laboratory findings were as follows: hemoglobin, 8 gin%; hematocrit, 22.5 ram; white blood cell count 10,000; red cell count 2.11 million. A sickle cell preparation was positive. Serum uric acid was 11 mg%; total serum protein 8.8 gm%; total bilirubin 6.5 mg%; serum alkaline phosphatase 105 mU/ml. Serum electrolytes, bicarbonate, creatinine, urea nitrogen, and glucose were all within normal limits. Hemoglobin electrophoresis showed the presence of SS hemoglobin, with 2.66% fetal hemoglobin. X-ray films revealed a generalized demineralization of the thoracic and lumbar vertebrae, cholelithiasis, and mild car484
Discussion Spinal cord involvement in sickle cell anemia is very uncommon. The present case differs from those previously described in that the spinal cord damage was associated with compression by an extramedullary hemopoietic mass, rather than with any intrinsic vascular lesion. Extramedullary hemopoiesis is known to occur mostly as a compensatory mechanism in patients with various hematological disorders in which there is severe anemia. Seven cases of hemopoiesis in the extradural space, with spinal cord compression, were reviewed by Heffner and Koehl? Four of those cases had thalassemia, and three were cases of myelosclerosis. Our patient's complete recovery from chronic paraplegia during the year following surgery is most unusual, considering the long duration of his disability prior to treatment. Occasionally remarkable recovery from paraplegia in cases in which treatment was delayed for 2 to 18 months has been reported,
J. Neurosurg. / Volume 43 / October, 1975
Hemopoietic tissue cord compression in sickle cell anemia but it is r a r e ) The prognosis for recovery after delayed decompression is apparently better in those cases in which the neurological deficit developed slowly over a relatively long period of time. References 1. Baird RL, Weiss DL, Fergusson AD, et al: Studies in sickle cell anemia, 21. Clinicopathological aspects of neurological manifestations. Pediatrics 34:92-100, 1964 2. Bucy PC, Ladpli R: Recoverable paraplegia. JAMA 185:685-691, 1963 3. D'Eramo N, Levi M: Neurological Symptoms in Blood Diseases. Baltimore, University Park Press, 1972, p 19
J. Neurosurg. / Volume 43 / October, 1975
4. Greer N, Schotland D: Abnormal hemoglobin as a cause of neurologic disease. Neurology (Minneap) 12:114-123, 1962 5. Heffner RI Jr, Koehl RH: Hemopoiesis in the spinal epidural space, d Neurosurg 32:485-490, 1970 6. Song J: Pathology of Sickle Cell Disease. Springfield, Ill, Charles C Thomas, 1971, pp 316, 323 7. Wolman L, Hardy AG: Spinal cord infarction associated with the sickle cell trait. Paraplegia 7:282-291, 1970
Address reprint requests to: Abdel A. Ammoumi, M.D., Bird S. Coler Memorial Hospital, New York, New York 10017.
485
ABDEL A. AMMOUMI, M.D., JOANNA H. SHER, M.D., AND DANIEL SCHMELKA, M.D. Departments of Neurology, Pathology, and Neurosurgery, State University of New York, Downstate Medical Center, Brooklyn, New York The authors report a patient with sickle cell anemia who suffered from paraplegia of 18 months duration due to spinal cord compression by a hemopoietic mass. Recovery following removal of the mass was complete. KEy WORDS 9 sickle cell anemia 9 paraplegia 9 spinal cord compression 9 extramedullary hemopoietic tissue
C a s e Report
History. The patient was known to have sickle cell anemia and had suffered episodes of abdominal and joint pain in the past. He stated that gradually, over a period of 2 months, he had developed weakness in both legs, more severe on the right. This was followed by numbness, beginning in the feet, and then spreading up to the waist. He also experienced spontaneous jerking movements in his legs from time to time, mostly in the right leg. Two months after the onset of weakness the patient was unable to walk. At the time of admission he had been confined to a wheel chair for 18 months. There was no weakness or sensory loss in the arms, and sphincter function was preserved.
This 28-year-old black man was hospitalized because of spastic paraplegia of 18 months duration.
Examination. Neurological examination showed comp!ete spastic paraplegia. There was decreased appreciation of touch, pin-
HE protean neurological manifestations of sickle cell anemia are generally caused by thrombosis with resulting infarction or hemorrhage. 1'4,5,7 Both the degeneration of ganglion cells as well as posterior and lateral column demyelination have been observed in the spinal cord in sickle cell disease. 3'6 The following case of paraplegia secondary to spinal cord compression by a mass of extramedullary hemopoietic tissue (blood cell production outside the bone marrow) illustrates yet another way in which neural tissue may be damaged in sickle cell anemia.
T
J. Neurosurg. / Volume 43 / October, 1975
483
A. A. Ammoumi, J. H. Sher and D. Schmelka diomegaly. The lungs were clear. The spinal fluid drawn during the myelography procedure was slightly xanthochromic with a protein level of 86 mg%, glucose 75 mg%, and no cells. The myelogram showed an extradural pressure defect from the level of T-3 to T-5. Operation. A laminectomy was performed, and a dark, red mass measuring 2.0 X 1.0 • 0.5 cm found beneath the lamina and compressing the dura was removed. Microscopically, the tissue was basically fat, containing many blood vessels, and densely infiltrated by blood-forming elements. These were mainly of the erythroid series. Many megakaryocytes were present, as well as some foci of white-cell-forming tissue. The overall microscopic picture resembled hypercellular bone marrow with extremely severe erythroid hyperplasia (Fig. 1). Intravascular sickling of red cells was noted incidentally. Postoperative Course. A few days after surgery, the patient started to move his toes, but the paraplegia and the same sensory loss were still present. Motor and sensory function improved very gradually. In 5 months, the F1G. 1. Microscopic appearance of the extra- patient was walking with the help of two dural mass, showing hemopoietic tissue and meg- canes, and 3 months later he was walking unakaryocytes. H & E, • 250. supported with a normal gait. prick, and vibration and position sense below the T-7 dermatome. Tendon reflexes were normal and symmetrical in the arms, but hyperactive in the legs, with bilateral sustained clonus. There was no plantar response. Mental status, cranial nerve function, and coordination of the arms were all within normal limits. The optic fundi were normal. Laboratory findings were as follows: hemoglobin, 8 gin%; hematocrit, 22.5 ram; white blood cell count 10,000; red cell count 2.11 million. A sickle cell preparation was positive. Serum uric acid was 11 mg%; total serum protein 8.8 gm%; total bilirubin 6.5 mg%; serum alkaline phosphatase 105 mU/ml. Serum electrolytes, bicarbonate, creatinine, urea nitrogen, and glucose were all within normal limits. Hemoglobin electrophoresis showed the presence of SS hemoglobin, with 2.66% fetal hemoglobin. X-ray films revealed a generalized demineralization of the thoracic and lumbar vertebrae, cholelithiasis, and mild car484
Discussion Spinal cord involvement in sickle cell anemia is very uncommon. The present case differs from those previously described in that the spinal cord damage was associated with compression by an extramedullary hemopoietic mass, rather than with any intrinsic vascular lesion. Extramedullary hemopoiesis is known to occur mostly as a compensatory mechanism in patients with various hematological disorders in which there is severe anemia. Seven cases of hemopoiesis in the extradural space, with spinal cord compression, were reviewed by Heffner and Koehl? Four of those cases had thalassemia, and three were cases of myelosclerosis. Our patient's complete recovery from chronic paraplegia during the year following surgery is most unusual, considering the long duration of his disability prior to treatment. Occasionally remarkable recovery from paraplegia in cases in which treatment was delayed for 2 to 18 months has been reported,
J. Neurosurg. / Volume 43 / October, 1975
Hemopoietic tissue cord compression in sickle cell anemia but it is r a r e ) The prognosis for recovery after delayed decompression is apparently better in those cases in which the neurological deficit developed slowly over a relatively long period of time. References 1. Baird RL, Weiss DL, Fergusson AD, et al: Studies in sickle cell anemia, 21. Clinicopathological aspects of neurological manifestations. Pediatrics 34:92-100, 1964 2. Bucy PC, Ladpli R: Recoverable paraplegia. JAMA 185:685-691, 1963 3. D'Eramo N, Levi M: Neurological Symptoms in Blood Diseases. Baltimore, University Park Press, 1972, p 19
J. Neurosurg. / Volume 43 / October, 1975
4. Greer N, Schotland D: Abnormal hemoglobin as a cause of neurologic disease. Neurology (Minneap) 12:114-123, 1962 5. Heffner RI Jr, Koehl RH: Hemopoiesis in the spinal epidural space, d Neurosurg 32:485-490, 1970 6. Song J: Pathology of Sickle Cell Disease. Springfield, Ill, Charles C Thomas, 1971, pp 316, 323 7. Wolman L, Hardy AG: Spinal cord infarction associated with the sickle cell trait. Paraplegia 7:282-291, 1970
Address reprint requests to: Abdel A. Ammoumi, M.D., Bird S. Coler Memorial Hospital, New York, New York 10017.
485
