PRIAPISM ASSOCIATED WITH ASPLENIC STATE ANTHONY ATALA, M.D. MOHAMMAD AMIN, M.D. JAMES I. HARTY, M.D. YONG K. LIU, M.D. MARIE M. KEELING, M.D.
From the Departments of Surgery (Division of Urology), Medicine, and Pathology, University of Louisville School of Medicine, Louisville, Kentucky ABSTRACT-Priapism may be primary (idiopathic) OT secondary to sickle cell anemia, trauma, leukemia, drugs, venous thromboembolic diseases, and other less common disorders. This study concerns 21 patients with priapism treated during a period of ten years. Nine patients (43 %) had sickle cell anemia. Of the 12 individuals (57%) classified as idiopathic, 3 (25%) had previously undetgone surgical splenectomy for benign conditions. Considering the propensity for this unusual condition to develop in patients with hemoglobinopathy-induced hyposplenism, the possibility of a relationship between the asplenic state and priapism is considered.
Priapism is a prolonged painful erection of the corpora cavernosa unaccompanied by sexual desire. It has been classified into 2 main groups, primary (idiopathic) and secondary, which are attributed to more than 38 different causes.’ The more common conditions associated with secondary priapism include sickle cell anemia, trauma, leukemia, cancerous invasion of the penis, drugs, alcohol ingestion, various thromboembolic diseases, and intravenous fat for parenteral nutrition. Splenectomy has not been previously identified as a predisposing factor, except in a single case report of a patient with thalassemia intermedia.2 However, we have encountered 3 patients, 25 percent of our patients with idiopathic priapism, who had a splenectomy. This observation stimulated us to conduct a retrospective review of our experience. Material and Methods In the past ten years, 21 patients had 40 episodes of priapism, requiring admission to University of Louisville hospitals. The hospital records were reviewed to determine the etiology, evidence of asplenic state, and common hema-
UROLOGY
/ OCTOBER
1992
I
VOLUME
40, NUMBER 4
tologic factors which may have contributed to priapism. Sixty-eight percent of the patients were black and 32 percent were white. Nine patients (43%) had sickle cell anemia. Twelve patients (57 % ) did not have any of the known associated conditions with priapism and were classified in the idiopathic group. Three of 12 patients (25%) in the idiopathic group had previously undergone splenectomy. Case abstracts of these 3 patients follow. Case Abstracts Case 1 A thirty-one-year-old white man presented with a forty-eight-hour history of priapism. He had a splenectomy due to an injury from a motor vehicle accident two years prior to this admission. There were no associated genitourinary injuries. He denied any drug ingestion or family history of blood disease. Hematologic evaluation showed that his hemoglobin (Hgb) was 14.7 g/l00 mL, hematocrit (HCT) 46%) platelets 412,OOO/cu mm, red blood cells (RBC) 4.73 millionlcu mm, mean
371
corpuscular volume (MCV) 96 cu pm, mean corpuscular hemoglobin (MCH) 32.3 pg, mean corpuscular hemoglobin concentration (MCHC) 34 % , white blood cell count (WBC) 19,8OO/cu mm, with 1% polymorphonucleocytes, 75% segmented neutrophils, 15% lym7 % mononucleocytes, 1% phocytes, eosinophils, and 1% basophils. The peripheral blood smear showed normochromic and normocytic RBC without polychromasia. Many RBC had 1 to 2 small inclusion bodies, there was an increase in target cells, and platelets appeared adequate in number and had normal morphology. No immature leukocytes were seen. The hematologic changes were consistent with a post-splenectomy state. The coagulation studies did not show any abnormality. Cavernous-glandular and cavernous-spongiosum shunts produced temporary relief, and a second cavernous-glandular shunt was needed before detumescence was achieved. Case 2 After priapism of thirty-six hours’ duration, a twenty-nine-year-old white man was admitted to the hospital. Past history was significant for a gunshot injury to the abdomen, requiring a splenectomy five years previously. There were no associated genitourinary injuries. The hematologic evaluation showed a Hgb of 15.4 g/100 mL, Hct 45%) platelets 465,OOO/cu mm, RBC 4.62 millionlcu mm, MCV 96.4 cu pm, MCH 33.3 pg, MCHC 35%) WBC 27,800/ cu mm, with 1% Pmn, 80% segmented neutrophils, 8 % lymphocytes, 7 % mononucleocytes, 2% eosinophils, and 2% basophils. The peripheral blood smear showed normochromic and normocytic red blood cells with slight polychromasia and many Howell-Jolly bodies. The morphologic changes in the blood smear were consistent with a post-splenectomy state. Sickle cell screen was negative, and results of coagulation studies were within normal limits. There was no family history of blood disease. This patient required corporeal irrigation and cavernous-glandular shunt on three separate occasions within the next seventy-two hours before full detumescence could be achieved. Case 3 A thirty-seven-year-old white man with a twenty-four-hour history of painful persistent erection on admission denied recent sexual stimulation or alcohol ingestion. The patient’s
372
only medications were aluminum hydroxide tablets and a daily nutritional supplement. He had been on hemodialysis for six years because of renal failure due to polycystic kidney disease. This episode of priapism occurred five days after his last weekly scheduled hemodialysis. He had had a splenectomy ten years previously, following an automobile accident with no associated genitourinary injuries. Hematologic evaluation showed a Hgb of 13.5 g/100 mL, Hct 40%) platelets 278,OOO/cu mm, RBC 4.55 millionlcu mm, MCV 86.7 cu pm, MCH 29.8 pg, MCHC 34 % , WBC 9,100/ cu mm, with 1% Pmn, 63% segmented neutrophils, 22 % lymphocytes, 8 % mononucleocytes, 4 % eosinophils, and 2% basophils. The peripheral blood smear showed normochromic and normocytic RBC with mild polychromasia. There were occasional spherocytes and schistocytes. Howell-Jolly bodies were seen. Platelets appeared adequate in number, and no immature leukocytes or rouleaux formation were seen. The changes in the blood smear were consistent with the expected morphology after splenectomy. The sickle cell screen was negative. No coagulation abnormalities were found. There was no family history of blood disease. The patient was treated with sedation, hydration, and analgesics for sixteen hours without any relief. A cavernous-glandular shunt was performed with immediate detumescence, but the priapism recurred forty-eight hours later. Subsequent irrigation of the corporeal bodies with normal saline was carried out with good results. Comment Large series of patients with priapism are not available; possibly it is underreported. Although many conditions have been implicated in secondary priapism, the idiopathic group remains significant. The pathophysiology of priapism is not fully understood, but it is thought that vascular, hematologic, and neurogenie factors are involved singularly or in different combinations. Sickle cell disease has been implicated in up to 88 percent of patients.3 The sludging of red blood cells in the corporeal spaces is presumed to be the main cause of priapism in this condition. The sludging of white blood cells in leukemia4 and platelets in thrombocytosis5 also have been reported as the cause of priapism. Hypercoagulability, including that incurred during anticoagulation, and
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/
OCTOBER
1992
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VOLUME
40, NUMBER 4
heparin itself also have been cited as possible secondary to etiologies. 6-8 Hypercoagulability heparin is perhaps the major contributing factor toward priapism during hemodialysis.* Peritoneal dialysis, which does not involve heparin, is not associated with priapism.7 One of our splenectomy patients had renal failure requiring hemodialysis. However, his episode of priapism occurred more than five days after, not during, hemodialysis. The asplenic state produces significant hematologic disturbance, but has not been widely reported to cause priapism. Welford, Spies, and Green5 reported a case in 1981 of priapism in a patient with primary thrombocythemia, who had had a splenectomy for idiopathic thrombocytopenic purpura twenty-three years previously. However, the association of splenectomy and priapism was not commented on in that report. Jackson, Franklin, and Hughes2 reported a case in 1986 of recurrent priapism following splenectomy for thalassemia intermedia. They implied that the thrombocytosis and elevated nucleated red blood cell count that followed splenectomy was a cause of priapism. Reduction of these counts with hydroxyurea treatment prevented further episodes of priapism. Rao and PateP also described a case in 1986 of thalassemia intermedia where priapism developed four weeks following splenectomy. Our series includes 3 patients who had splenectomy following abdominal trauma and in whom priapism subsequently developed. This accounted for 25 percent of patients considered idiopathic. While the exact mechanism of priapism after splenectomy is not fully understood, the spleen in humans has a dual function of filtration and immunologic destruction an,d processing. It removes unwanted blood cells, particles, and bacteria from the blood.1° Continually, its regulatory role is elucidated and defined by what happens in the asplenic individual. In this respect, its importance in preventing fulminant, often fatal, septicemia with encapsulated organisms like pneumococcus or meningococcus is unchallenged. Other harmful consequences of the loss of splenic function, either through surgery or disease, may not have been given sufficient attention.” Indeed, a long-term survey of 740 patients with splenectomy showed not only the expected excess mortality from infection but also significant ischemic heart disease possibly from hypercoagulability associated with asple-
UROLOGY
/
OCTOBER
1992
/ VOLUME 40, NUMBER 4
nia.12 The hematologic changes observed after splenectomy include leukocytosis, thrombocytosis, fragmented red blood cells, nucleated red blood cells, and those containing Howell-Jolly bodies. Any of these can cause sludging of blood in the corporeal bodies producing priapism. It is important to note that a majority of patients with sickle cell anemia have functional asplenia . l3 The spleen of sickle cell patients, even when enlarged, is inactive as a biologic filter, as demonstrated by radioisotope scanning. The hematologic picture also resembles an asplenic state with classic indicators, such as Howell-Jolly bodies, commonplace. Any intraerythrocytic inclusions such as these might decrease the red blood cell’s ability to change in shape which is so critical in certain areas of the circulation. This is a contributing factor to the slowdown of blood flow. The subsequent hemodynamic change could provoke priapism. The hematologic similarities between patients with sickle cell disease and those who have had a splenectomy are multiple. The occurrence of priapism in both these groups could relate to the asplenic state. Such patients should be considered at a higher risk of this disorder developing. Division of Urology University of Louisville Louisville, Kentucky 40292 (DR. AMIN) References 1. Pohl J, Pott B, and Kleinhaus G: Priapism: a three-phase concept of management according to aetiology and prognosis, Br J Urol 58: 113 (1986). 2. Jackson N, Franklin IM, and Hughes MA: Recurrent priapism following splenectomy for thalassaemia intermedia, Br J Surg 73: 678 (1986). 3. Hasen HB, and Raines SL: Priapism associated with sickle cell disease, J Urol 88: 71 (1962). 4. Steinhardt GF, and Steinhardt E: Priapism in children with leukemia, Urology 18: 604 (1981). 5. Welford C, Spies SM, and Green D: Priapism in primary thrombocythemia, Arch Intern Med 141: 807 (1981). 6. Port FK, et al: Priapism during regular haemodialysis, Lancet 2: 1287 (1974). 7. Singhal PC, Lynn RI, and Scharschmidt LA: Priapism and dialysis, Am J Nephro16: 358 (1986). 8. Dahlke MB, et al: Priapism during filtration leukapheresis, Transfusion 19: 482 (1979). 9. Rao KRP, and Pate1 AR: Priapism and thalassaemia intermedia, Br J Surg 73: 1048 (1986). 10. Rosx WF: The spleen as a filter, N Engl J Med 317: 704 (1987). 11. Nomikos IN: Is sepsis the only possible harmful consequence of splenectomy?, N Engl J Med 311: 198 (1984). 12. Robinette CD: Splenectomy and subsequent mortality in veterans of the 1939-45 War, Lancet 2: 127 (1977). 13. Pearson HA, Spencer RP, and Cornelius EA: Functional asplenia in sickle-cell anemia, N Engl J Med 281: 923 (1969).
373
From the Departments of Surgery (Division of Urology), Medicine, and Pathology, University of Louisville School of Medicine, Louisville, Kentucky ABSTRACT-Priapism may be primary (idiopathic) OT secondary to sickle cell anemia, trauma, leukemia, drugs, venous thromboembolic diseases, and other less common disorders. This study concerns 21 patients with priapism treated during a period of ten years. Nine patients (43 %) had sickle cell anemia. Of the 12 individuals (57%) classified as idiopathic, 3 (25%) had previously undetgone surgical splenectomy for benign conditions. Considering the propensity for this unusual condition to develop in patients with hemoglobinopathy-induced hyposplenism, the possibility of a relationship between the asplenic state and priapism is considered.
Priapism is a prolonged painful erection of the corpora cavernosa unaccompanied by sexual desire. It has been classified into 2 main groups, primary (idiopathic) and secondary, which are attributed to more than 38 different causes.’ The more common conditions associated with secondary priapism include sickle cell anemia, trauma, leukemia, cancerous invasion of the penis, drugs, alcohol ingestion, various thromboembolic diseases, and intravenous fat for parenteral nutrition. Splenectomy has not been previously identified as a predisposing factor, except in a single case report of a patient with thalassemia intermedia.2 However, we have encountered 3 patients, 25 percent of our patients with idiopathic priapism, who had a splenectomy. This observation stimulated us to conduct a retrospective review of our experience. Material and Methods In the past ten years, 21 patients had 40 episodes of priapism, requiring admission to University of Louisville hospitals. The hospital records were reviewed to determine the etiology, evidence of asplenic state, and common hema-
UROLOGY
/ OCTOBER
1992
I
VOLUME
40, NUMBER 4
tologic factors which may have contributed to priapism. Sixty-eight percent of the patients were black and 32 percent were white. Nine patients (43%) had sickle cell anemia. Twelve patients (57 % ) did not have any of the known associated conditions with priapism and were classified in the idiopathic group. Three of 12 patients (25%) in the idiopathic group had previously undergone splenectomy. Case abstracts of these 3 patients follow. Case Abstracts Case 1 A thirty-one-year-old white man presented with a forty-eight-hour history of priapism. He had a splenectomy due to an injury from a motor vehicle accident two years prior to this admission. There were no associated genitourinary injuries. He denied any drug ingestion or family history of blood disease. Hematologic evaluation showed that his hemoglobin (Hgb) was 14.7 g/l00 mL, hematocrit (HCT) 46%) platelets 412,OOO/cu mm, red blood cells (RBC) 4.73 millionlcu mm, mean
371
corpuscular volume (MCV) 96 cu pm, mean corpuscular hemoglobin (MCH) 32.3 pg, mean corpuscular hemoglobin concentration (MCHC) 34 % , white blood cell count (WBC) 19,8OO/cu mm, with 1% polymorphonucleocytes, 75% segmented neutrophils, 15% lym7 % mononucleocytes, 1% phocytes, eosinophils, and 1% basophils. The peripheral blood smear showed normochromic and normocytic RBC without polychromasia. Many RBC had 1 to 2 small inclusion bodies, there was an increase in target cells, and platelets appeared adequate in number and had normal morphology. No immature leukocytes were seen. The hematologic changes were consistent with a post-splenectomy state. The coagulation studies did not show any abnormality. Cavernous-glandular and cavernous-spongiosum shunts produced temporary relief, and a second cavernous-glandular shunt was needed before detumescence was achieved. Case 2 After priapism of thirty-six hours’ duration, a twenty-nine-year-old white man was admitted to the hospital. Past history was significant for a gunshot injury to the abdomen, requiring a splenectomy five years previously. There were no associated genitourinary injuries. The hematologic evaluation showed a Hgb of 15.4 g/100 mL, Hct 45%) platelets 465,OOO/cu mm, RBC 4.62 millionlcu mm, MCV 96.4 cu pm, MCH 33.3 pg, MCHC 35%) WBC 27,800/ cu mm, with 1% Pmn, 80% segmented neutrophils, 8 % lymphocytes, 7 % mononucleocytes, 2% eosinophils, and 2% basophils. The peripheral blood smear showed normochromic and normocytic red blood cells with slight polychromasia and many Howell-Jolly bodies. The morphologic changes in the blood smear were consistent with a post-splenectomy state. Sickle cell screen was negative, and results of coagulation studies were within normal limits. There was no family history of blood disease. This patient required corporeal irrigation and cavernous-glandular shunt on three separate occasions within the next seventy-two hours before full detumescence could be achieved. Case 3 A thirty-seven-year-old white man with a twenty-four-hour history of painful persistent erection on admission denied recent sexual stimulation or alcohol ingestion. The patient’s
372
only medications were aluminum hydroxide tablets and a daily nutritional supplement. He had been on hemodialysis for six years because of renal failure due to polycystic kidney disease. This episode of priapism occurred five days after his last weekly scheduled hemodialysis. He had had a splenectomy ten years previously, following an automobile accident with no associated genitourinary injuries. Hematologic evaluation showed a Hgb of 13.5 g/100 mL, Hct 40%) platelets 278,OOO/cu mm, RBC 4.55 millionlcu mm, MCV 86.7 cu pm, MCH 29.8 pg, MCHC 34 % , WBC 9,100/ cu mm, with 1% Pmn, 63% segmented neutrophils, 22 % lymphocytes, 8 % mononucleocytes, 4 % eosinophils, and 2% basophils. The peripheral blood smear showed normochromic and normocytic RBC with mild polychromasia. There were occasional spherocytes and schistocytes. Howell-Jolly bodies were seen. Platelets appeared adequate in number, and no immature leukocytes or rouleaux formation were seen. The changes in the blood smear were consistent with the expected morphology after splenectomy. The sickle cell screen was negative. No coagulation abnormalities were found. There was no family history of blood disease. The patient was treated with sedation, hydration, and analgesics for sixteen hours without any relief. A cavernous-glandular shunt was performed with immediate detumescence, but the priapism recurred forty-eight hours later. Subsequent irrigation of the corporeal bodies with normal saline was carried out with good results. Comment Large series of patients with priapism are not available; possibly it is underreported. Although many conditions have been implicated in secondary priapism, the idiopathic group remains significant. The pathophysiology of priapism is not fully understood, but it is thought that vascular, hematologic, and neurogenie factors are involved singularly or in different combinations. Sickle cell disease has been implicated in up to 88 percent of patients.3 The sludging of red blood cells in the corporeal spaces is presumed to be the main cause of priapism in this condition. The sludging of white blood cells in leukemia4 and platelets in thrombocytosis5 also have been reported as the cause of priapism. Hypercoagulability, including that incurred during anticoagulation, and
UROLOGY
/
OCTOBER
1992
I
VOLUME
40, NUMBER 4
heparin itself also have been cited as possible secondary to etiologies. 6-8 Hypercoagulability heparin is perhaps the major contributing factor toward priapism during hemodialysis.* Peritoneal dialysis, which does not involve heparin, is not associated with priapism.7 One of our splenectomy patients had renal failure requiring hemodialysis. However, his episode of priapism occurred more than five days after, not during, hemodialysis. The asplenic state produces significant hematologic disturbance, but has not been widely reported to cause priapism. Welford, Spies, and Green5 reported a case in 1981 of priapism in a patient with primary thrombocythemia, who had had a splenectomy for idiopathic thrombocytopenic purpura twenty-three years previously. However, the association of splenectomy and priapism was not commented on in that report. Jackson, Franklin, and Hughes2 reported a case in 1986 of recurrent priapism following splenectomy for thalassemia intermedia. They implied that the thrombocytosis and elevated nucleated red blood cell count that followed splenectomy was a cause of priapism. Reduction of these counts with hydroxyurea treatment prevented further episodes of priapism. Rao and PateP also described a case in 1986 of thalassemia intermedia where priapism developed four weeks following splenectomy. Our series includes 3 patients who had splenectomy following abdominal trauma and in whom priapism subsequently developed. This accounted for 25 percent of patients considered idiopathic. While the exact mechanism of priapism after splenectomy is not fully understood, the spleen in humans has a dual function of filtration and immunologic destruction an,d processing. It removes unwanted blood cells, particles, and bacteria from the blood.1° Continually, its regulatory role is elucidated and defined by what happens in the asplenic individual. In this respect, its importance in preventing fulminant, often fatal, septicemia with encapsulated organisms like pneumococcus or meningococcus is unchallenged. Other harmful consequences of the loss of splenic function, either through surgery or disease, may not have been given sufficient attention.” Indeed, a long-term survey of 740 patients with splenectomy showed not only the expected excess mortality from infection but also significant ischemic heart disease possibly from hypercoagulability associated with asple-
UROLOGY
/
OCTOBER
1992
/ VOLUME 40, NUMBER 4
nia.12 The hematologic changes observed after splenectomy include leukocytosis, thrombocytosis, fragmented red blood cells, nucleated red blood cells, and those containing Howell-Jolly bodies. Any of these can cause sludging of blood in the corporeal bodies producing priapism. It is important to note that a majority of patients with sickle cell anemia have functional asplenia . l3 The spleen of sickle cell patients, even when enlarged, is inactive as a biologic filter, as demonstrated by radioisotope scanning. The hematologic picture also resembles an asplenic state with classic indicators, such as Howell-Jolly bodies, commonplace. Any intraerythrocytic inclusions such as these might decrease the red blood cell’s ability to change in shape which is so critical in certain areas of the circulation. This is a contributing factor to the slowdown of blood flow. The subsequent hemodynamic change could provoke priapism. The hematologic similarities between patients with sickle cell disease and those who have had a splenectomy are multiple. The occurrence of priapism in both these groups could relate to the asplenic state. Such patients should be considered at a higher risk of this disorder developing. Division of Urology University of Louisville Louisville, Kentucky 40292 (DR. AMIN) References 1. Pohl J, Pott B, and Kleinhaus G: Priapism: a three-phase concept of management according to aetiology and prognosis, Br J Urol 58: 113 (1986). 2. Jackson N, Franklin IM, and Hughes MA: Recurrent priapism following splenectomy for thalassaemia intermedia, Br J Surg 73: 678 (1986). 3. Hasen HB, and Raines SL: Priapism associated with sickle cell disease, J Urol 88: 71 (1962). 4. Steinhardt GF, and Steinhardt E: Priapism in children with leukemia, Urology 18: 604 (1981). 5. Welford C, Spies SM, and Green D: Priapism in primary thrombocythemia, Arch Intern Med 141: 807 (1981). 6. Port FK, et al: Priapism during regular haemodialysis, Lancet 2: 1287 (1974). 7. Singhal PC, Lynn RI, and Scharschmidt LA: Priapism and dialysis, Am J Nephro16: 358 (1986). 8. Dahlke MB, et al: Priapism during filtration leukapheresis, Transfusion 19: 482 (1979). 9. Rao KRP, and Pate1 AR: Priapism and thalassaemia intermedia, Br J Surg 73: 1048 (1986). 10. Rosx WF: The spleen as a filter, N Engl J Med 317: 704 (1987). 11. Nomikos IN: Is sepsis the only possible harmful consequence of splenectomy?, N Engl J Med 311: 198 (1984). 12. Robinette CD: Splenectomy and subsequent mortality in veterans of the 1939-45 War, Lancet 2: 127 (1977). 13. Pearson HA, Spencer RP, and Cornelius EA: Functional asplenia in sickle-cell anemia, N Engl J Med 281: 923 (1969).
373
