Sickle Cell Anemia: Recurrent Splenic Pain Relieved by Splenectomy OSCAR F. BALLESTER, M.D.; and JAMES WARTH, M.D. Harper-Grace Hospital, Wayne State University; Detroit, Michigan
SPLENOMEGALY is an uncommon finding in adults with homozygous sickle cell disease. Henderson (1) reported palpable spleens in three adults in a series of 54 patients with sickle cell anemia. Watson, Lichtman, and Shapiro (2) found splenomegaly in 3 3 % of patients with sickle cell disease in the first decade of life and in 10% of those older. Most of these early studies did not conclusively rule out double heterozygous states (Hb SC, Hb S-thalassemia, and so forth), which have been reported to have a higher incidence of splenomegaly. In 1970, Serjeant (3) correlated the persistence of splenomegaly in adults with sickle cell disease with low irreversible sickle cell counts and higher Hb F levels. He found an incidence of splenomegaly of 9 % among patients with sickle cell disease in the West Indies older than age 25. They were noted to have a more benign course. None of his adult patients with sickle cell disease with splenomegaly admitted symptoms that could be attributed to episodes of splenic infarction (4). We report the case of a patient with homozygous sickle cell disease with splenomegaly and severe episodes of leftupper-quadrant pain, in whom splenectomy produced complete relief of this symptom. A 29-year-old black woman, known to have sickle cell anemia since age 3, was hospitalized at Harper-Grace Hospital in October 1977. One and a half years before admission, she noted the onset of recurrent attacks of left-upper-quadrant pain. Each attack was of sudden onset, sharp and excruciating in nature, and lasted from a few minutes to an hour. From June to October 1977, the episodes that had occurred twice a week increased to once or twice a day. They became more severe and necessitated repeated hospitalizations. Exchange transfusions were of no benefit. These attacks could not be correlated with decreases in the patient's hemoglobin level, leukocyte count, or platelet count. There were no associated sudden increases in liver or spleen size. She had had numerous admissions in the past for generalized pain crises as well as for urinary- and respiratorytract infections and asthma. On examination, her temperature was 37.7 °C. Her sclerae showed slight icterus. A grade II/VI systolic ejection murmur Brief Reports
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for splenectomy in sickle cell disease. In 1927, Hahn and Gillespie (8) reported a case of a 4-year-old child with probable sickle cell disease who underwent successful splenectomy for hypersplenism. In 1951, Shotton, Crockett, and Leavell (9) reported a similar case and reviewed the literature. They found 23 patients, the oldest 18 years of age. Amelioration of the hypersplenic state occurred in 10, generally those with larger spleens. Recently, Stevenson (10) added two more cases. Our patient with proven homozygous sickle cell disease had severe daily attacks of left-upper-quadrant pain, suggesting repeated episodes of splenic infarction. H e r spleen progressively increased in size and in the amount of calcification over 10.5 years, but no evidence of hypersplenism or sequestration could be documented at any time. Splenectomy provided complete relief of her leftupper-quadrant pain.
Figure 1. Photomicrograph section of spleen showing necrosis with foreign-body giant cells, calcium and hemosiderin deposits, hemorrhage, and fibrosis. (Hematoxylin and eosin; original magnification, X75.)
The authors thank Dr. Donald Rucknagel for doing the hemoglobin biosynthetic studies and acknowledge the surgical expertise of Dr. Raman Golwala. This study was supported by a Sickle Cell Center Grant (USPHS-HL-16008) to Wayne State University. Dr. Warth is the recipient of a grant from the Medical Staff Trust Fund of Harper Hospital.
was heard at the apex. A tender spleen was palpated 6 cm below the left costal margin in the anterior axillary line. There was no hepatomegaly. Other findings were normal. Initial laboratory results were hematocrit, 29.6%; hemoglobin, 9.8 g/dl; reticulocyte count, 19.8%; leukocyte count, 13 800 mm3 (59% neutrophils, 10% bands, 2 1 % lymphocytes, 5% monocytes, 4% eosinophils, and 1% basophils). The serum amylase level was 58 IU. Arterial Po2 was normal. Urine analysis showed a specific gravity of 1.006 but was otherwise unremarkable. Results of chest roentgenogram and ECG were within normal limits. A plain film of the abdomen showed an enlarged spleen with multiple punctate calcifications. On review of previous roentgenograms, a gradual increase in size and calcification of the spleen could be documented over a 10.5-year period. Results of intravenous pyelogram, renal flow studies, renal scan, and renograms were normal. Splenic scans showed markedly decreased to absent uptake. Hemoglobin electrophoresis showed type SS with A2 of 3.1% and Hb F of 10.0%. Hemoglobin biosynthetic studies showed that the alpha/beta chain ratio was 0.98, which is normal. Before transfusion, the mean corpuscular volume was 92 and direct Coombs' test results were negative. After an exchange transfusion brought her Hb S level to 23%, she underwent splenectomy and incidental cholecystectomy. The spleen weighed 311 g and measured 9 by 15 cm. Hemorrhagic areas were evident on cutting the spleen. The capsule was moderately thickened. Areas of necrosis, fibrosis, hemosiderin, and calcium deposition occurred diffusely throughout. The white pulp was depleted and compressed. Foreign-body giant cells were noted in areas of tissue necrosis (Figure 1). There was no evidence of storage disease, granulomata, tumor, or abscess formation. The splenic imprint was normal. The gallbladder displayed chronic cholecystitis and contained one large gallstone. The postoperative period was unremarkable, and she was discharged on the 17th hospital day. As of the time of this report, more than 6 months after surgery, she has had no further episodes of left-upper-quadrant or left-flank pain.
REFERENCES
1. HENDERSON AB: Sickle cell anemia. Am J Med 9:757-765, 1950 2. W A T S O N RJ, L I C H T M A N HC, SHAPIRO H D : Splenomegaly in sickle cell
anemia. Am J Med 20:196-206, 1956 3. SERJEANT GR: Irreversibly sickled cells and splenomegaly in sickle cell anemia. Brit J Haematol 19:635-641, 1970 4. SERJEANT GR, R I C H A R D S R, BARBOR P R H , M I L N E R PF: Relatively
benign sickle cell anemia in 60 patients over age 30 in the West Indies. Brit Med J 3:86-91, 1968 5. O ' B R I E N RT, PEARSON HA, G O D L E Y JA, SPENCER RP: Splenic infarct
in sickle (cell) trait. N Engl J Med 287:720-721, 1972 6. K I N G DT, LINDSTROM RR, S T A T E D, H I R O S E FM, S C H W A R T Z A:
Unusual case of acute abdomen. JAMA 238:2173-2174, 1977 7. SEELER RA, SHWIAKI MZ: Acute splenic sequestration crisis (ASSC) in young children with sickle cell anemia. Clin Ped 11:701-704, 1972 8. H A H N EV, GILLESPIE EB: Sickle cell anemia: report of a case greatly improved by splenectomy. Arch Intern Med 39:233, 1927 9. SHOTTON D, C R O C K E T T CL, L E A V E L L BS: Splenectomy in sickle cell
anemia: report of a case and review of the literature. Blood 6:365-371, 1951 10. STEVENSON AR: Splenectomy in sickle cell anemia. Arch Intern Med 125:883-884, 1970 © 1 9 7 9 American College of Physicians
Renal Excretion of "mTc-Diphosphonate in Osteomalacia J. D. MACFARLANE, B.M.; M. R. A. KHAIRI, M.D.; M. RICCIARDONE, B.S.; H. N. WELLMAN, M.D.; and C. C. JOHNSTON, Jr., M.D. Indiana University School of Medicine; Indianapolis, Indiana I N T E R E S T I N BONE SCANNING in metabolic bone disease
has led to the identification of pseudofractures in osteomalacia (1), the assessment of the severity of renal osteodystrophy (2), and the suggestion that calculation of bone to soft-tissue ratios may help distinguish patients with bone disease from normal control persons (3). These techniques are relatively crude and do not necessarily indicate the real skeletal accretion or whole body retention of the radionuclide. During a study of sequential bone to soft-tissue ratios in patients with various bone diseases, we estimated the renal excretion of 99m Tc-ethane-1 -hydroxy- 1, 1-diphosphonate ( 9 9 m Tc-EHDP). We report here our findings from a study of the renal excretion of 99m Tc-
Splenectomy in sickle cell states has been done for more than 50 years. In sickle cell trait, splenic infarctions under a variety of hypoxic and nonhypoxic conditions have been reported. Most of these episodes have been reported to resolve spontaneously (5), but on rare occasions splenectomy was necessary (6). In children with sickle cell disease splenectomy has been recommended after the first or second episode of splenic sequestration crisis because of the high incidence of recurrence (7). Hypersplenism has been a relatively common reason 3 5 0
March 1979 • Annals of Internal Medicine • Volume 90 • Number
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SPLENOMEGALY is an uncommon finding in adults with homozygous sickle cell disease. Henderson (1) reported palpable spleens in three adults in a series of 54 patients with sickle cell anemia. Watson, Lichtman, and Shapiro (2) found splenomegaly in 3 3 % of patients with sickle cell disease in the first decade of life and in 10% of those older. Most of these early studies did not conclusively rule out double heterozygous states (Hb SC, Hb S-thalassemia, and so forth), which have been reported to have a higher incidence of splenomegaly. In 1970, Serjeant (3) correlated the persistence of splenomegaly in adults with sickle cell disease with low irreversible sickle cell counts and higher Hb F levels. He found an incidence of splenomegaly of 9 % among patients with sickle cell disease in the West Indies older than age 25. They were noted to have a more benign course. None of his adult patients with sickle cell disease with splenomegaly admitted symptoms that could be attributed to episodes of splenic infarction (4). We report the case of a patient with homozygous sickle cell disease with splenomegaly and severe episodes of leftupper-quadrant pain, in whom splenectomy produced complete relief of this symptom. A 29-year-old black woman, known to have sickle cell anemia since age 3, was hospitalized at Harper-Grace Hospital in October 1977. One and a half years before admission, she noted the onset of recurrent attacks of left-upper-quadrant pain. Each attack was of sudden onset, sharp and excruciating in nature, and lasted from a few minutes to an hour. From June to October 1977, the episodes that had occurred twice a week increased to once or twice a day. They became more severe and necessitated repeated hospitalizations. Exchange transfusions were of no benefit. These attacks could not be correlated with decreases in the patient's hemoglobin level, leukocyte count, or platelet count. There were no associated sudden increases in liver or spleen size. She had had numerous admissions in the past for generalized pain crises as well as for urinary- and respiratorytract infections and asthma. On examination, her temperature was 37.7 °C. Her sclerae showed slight icterus. A grade II/VI systolic ejection murmur Brief Reports
Downloaded from https://annals.org by Tulane University user on 01/18/2019
349
for splenectomy in sickle cell disease. In 1927, Hahn and Gillespie (8) reported a case of a 4-year-old child with probable sickle cell disease who underwent successful splenectomy for hypersplenism. In 1951, Shotton, Crockett, and Leavell (9) reported a similar case and reviewed the literature. They found 23 patients, the oldest 18 years of age. Amelioration of the hypersplenic state occurred in 10, generally those with larger spleens. Recently, Stevenson (10) added two more cases. Our patient with proven homozygous sickle cell disease had severe daily attacks of left-upper-quadrant pain, suggesting repeated episodes of splenic infarction. H e r spleen progressively increased in size and in the amount of calcification over 10.5 years, but no evidence of hypersplenism or sequestration could be documented at any time. Splenectomy provided complete relief of her leftupper-quadrant pain.
Figure 1. Photomicrograph section of spleen showing necrosis with foreign-body giant cells, calcium and hemosiderin deposits, hemorrhage, and fibrosis. (Hematoxylin and eosin; original magnification, X75.)
The authors thank Dr. Donald Rucknagel for doing the hemoglobin biosynthetic studies and acknowledge the surgical expertise of Dr. Raman Golwala. This study was supported by a Sickle Cell Center Grant (USPHS-HL-16008) to Wayne State University. Dr. Warth is the recipient of a grant from the Medical Staff Trust Fund of Harper Hospital.
was heard at the apex. A tender spleen was palpated 6 cm below the left costal margin in the anterior axillary line. There was no hepatomegaly. Other findings were normal. Initial laboratory results were hematocrit, 29.6%; hemoglobin, 9.8 g/dl; reticulocyte count, 19.8%; leukocyte count, 13 800 mm3 (59% neutrophils, 10% bands, 2 1 % lymphocytes, 5% monocytes, 4% eosinophils, and 1% basophils). The serum amylase level was 58 IU. Arterial Po2 was normal. Urine analysis showed a specific gravity of 1.006 but was otherwise unremarkable. Results of chest roentgenogram and ECG were within normal limits. A plain film of the abdomen showed an enlarged spleen with multiple punctate calcifications. On review of previous roentgenograms, a gradual increase in size and calcification of the spleen could be documented over a 10.5-year period. Results of intravenous pyelogram, renal flow studies, renal scan, and renograms were normal. Splenic scans showed markedly decreased to absent uptake. Hemoglobin electrophoresis showed type SS with A2 of 3.1% and Hb F of 10.0%. Hemoglobin biosynthetic studies showed that the alpha/beta chain ratio was 0.98, which is normal. Before transfusion, the mean corpuscular volume was 92 and direct Coombs' test results were negative. After an exchange transfusion brought her Hb S level to 23%, she underwent splenectomy and incidental cholecystectomy. The spleen weighed 311 g and measured 9 by 15 cm. Hemorrhagic areas were evident on cutting the spleen. The capsule was moderately thickened. Areas of necrosis, fibrosis, hemosiderin, and calcium deposition occurred diffusely throughout. The white pulp was depleted and compressed. Foreign-body giant cells were noted in areas of tissue necrosis (Figure 1). There was no evidence of storage disease, granulomata, tumor, or abscess formation. The splenic imprint was normal. The gallbladder displayed chronic cholecystitis and contained one large gallstone. The postoperative period was unremarkable, and she was discharged on the 17th hospital day. As of the time of this report, more than 6 months after surgery, she has had no further episodes of left-upper-quadrant or left-flank pain.
REFERENCES
1. HENDERSON AB: Sickle cell anemia. Am J Med 9:757-765, 1950 2. W A T S O N RJ, L I C H T M A N HC, SHAPIRO H D : Splenomegaly in sickle cell
anemia. Am J Med 20:196-206, 1956 3. SERJEANT GR: Irreversibly sickled cells and splenomegaly in sickle cell anemia. Brit J Haematol 19:635-641, 1970 4. SERJEANT GR, R I C H A R D S R, BARBOR P R H , M I L N E R PF: Relatively
benign sickle cell anemia in 60 patients over age 30 in the West Indies. Brit Med J 3:86-91, 1968 5. O ' B R I E N RT, PEARSON HA, G O D L E Y JA, SPENCER RP: Splenic infarct
in sickle (cell) trait. N Engl J Med 287:720-721, 1972 6. K I N G DT, LINDSTROM RR, S T A T E D, H I R O S E FM, S C H W A R T Z A:
Unusual case of acute abdomen. JAMA 238:2173-2174, 1977 7. SEELER RA, SHWIAKI MZ: Acute splenic sequestration crisis (ASSC) in young children with sickle cell anemia. Clin Ped 11:701-704, 1972 8. H A H N EV, GILLESPIE EB: Sickle cell anemia: report of a case greatly improved by splenectomy. Arch Intern Med 39:233, 1927 9. SHOTTON D, C R O C K E T T CL, L E A V E L L BS: Splenectomy in sickle cell
anemia: report of a case and review of the literature. Blood 6:365-371, 1951 10. STEVENSON AR: Splenectomy in sickle cell anemia. Arch Intern Med 125:883-884, 1970 © 1 9 7 9 American College of Physicians
Renal Excretion of "mTc-Diphosphonate in Osteomalacia J. D. MACFARLANE, B.M.; M. R. A. KHAIRI, M.D.; M. RICCIARDONE, B.S.; H. N. WELLMAN, M.D.; and C. C. JOHNSTON, Jr., M.D. Indiana University School of Medicine; Indianapolis, Indiana I N T E R E S T I N BONE SCANNING in metabolic bone disease
has led to the identification of pseudofractures in osteomalacia (1), the assessment of the severity of renal osteodystrophy (2), and the suggestion that calculation of bone to soft-tissue ratios may help distinguish patients with bone disease from normal control persons (3). These techniques are relatively crude and do not necessarily indicate the real skeletal accretion or whole body retention of the radionuclide. During a study of sequential bone to soft-tissue ratios in patients with various bone diseases, we estimated the renal excretion of 99m Tc-ethane-1 -hydroxy- 1, 1-diphosphonate ( 9 9 m Tc-EHDP). We report here our findings from a study of the renal excretion of 99m Tc-
Splenectomy in sickle cell states has been done for more than 50 years. In sickle cell trait, splenic infarctions under a variety of hypoxic and nonhypoxic conditions have been reported. Most of these episodes have been reported to resolve spontaneously (5), but on rare occasions splenectomy was necessary (6). In children with sickle cell disease splenectomy has been recommended after the first or second episode of splenic sequestration crisis because of the high incidence of recurrence (7). Hypersplenism has been a relatively common reason 3 5 0
March 1979 • Annals of Internal Medicine • Volume 90 • Number
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