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Sickle Cell Disease: Renal Colic and Microscopic Hematuria
Vol. 100, Nov. Printed in U.S.A..
THE JOURNAL OF UROLOGY
Copyright © 1968 by The Williams & Wilkins Co.
SICKLE CELL DISEASE: RENAL COLIC AND MICROSCOPIC HKVIATURIA JOHN F. REDMAN
AND
JACK E. MOBLEY
From the Department of Urology, University of Arkansas Medical Center, Little Rock, Arkansas
Gross hematuria and priapism are the most common urological manifestations of sickle cell trait or disease. Pain of a dull or colicky nature has been reported in association with gross hematuria and the passage of clots from the urinary tract. Recently 2 patients with sickle cell trait, renal colic, clots in the renal collecting system and microscopic hematuria were admitted to the University of Arkansas Medical Center. Since the occurrence of renal pain in the absence of gross hematuria has not been recorded before in patients with sickle cell abnormality, the clinical courses of these patients are presented. CASE REPORTS
Case 1. A 35-year-old Negress was admitted to the medical center; on September 3, 1967. The patient had had intermittent right flank pain of moderate severity for a week before admission. One day before admission the pain was very severe and radiated to the right lower quadrant of the abdomen. The patient stated that she had never had hematuria. On admission her temperature was 102F, pulse rate was 108 per minute and blood pressure was 130/100 mm. Hg. The patient was in obvious discomfort, but the only positive physical finding was right flank tenderness and a palpable, tender right kidney. Laboratory studies were negative except for a positive sickle cell preparation and urinalysis which revealed many red and 10 to 20 white blood cells per high power field. Urine culture was negative. Partial thromboplastin time was 37 .2 seconds, prothrombin time was 13.5 seconds and platelets were considered normal on stained smear of the blood. Examination of the urine for acid-fast bacilli was negative. Hemoglobin electrophoresis confirmed the presence of SA hemoglobin. An excretory urogram demonstrated a nephrogram only on the right side at 20 minutes following the injection of contrast medium. The left kidney appeared normal. A definite pyelogram was not seen on the right side until the 12-hour delayed film, which also demonstrated a pelvic filling defect on the right side. The following day the temperature remained elevated and the patient's condition appeared worse. Anti-
Accepted for publication December 1, 1967. 594
biotics were started and a No. 6 whistle tip ureteral catheter was inserted in a retrograde manner to the right renal pelvis. At the time of cystoscopy the bladder urine was grossly clear and no effiux could be seen from the right ureteral orifice. A drip of dark, thick blood was obtained from the ureteral catheter. vVithin 24 hours the patient was afebrile and remained so. The ureteral catheter was removed after 5 days. A followup excretory urogram made the day after removal of the ureteral catheter was normal. The patient has remained well. Case 2. A 16-year-old Negress was admitted to the medical center on September 7, 1967. The patient had right flank pain which had started several hours before admission and was moderately severe in intensity. The patient stated that she had experienced several episodes of similar pain in the past month. Nausea and vomiting had occurred during the present episode. There had been some urgency and frequency of urination, but the patient denied ever having had hematuria. Physical examination was entirely normal. The patient was afebrile. Urinalysis revealed cloudy yellow urine which contained many red and 16 to 20 white blood cells per high power field. A sickle cell preparation was positive. Hemoglobin electrophoresis demonstrated the presence of SA hemoglobin. A platelet count was 270,000 per cu. mm., the partial thromboplastin time was 44.7 seconds and the prothrombin time was 14.4 seconds. Examination of the urine for acid-fast bacilli was negative. An excretory urogram showed filling defects in the right renal pelvis. The patient was placed at bed rest and observed. Her symptoms subsided without specific therapy. At the end of 5 days of hospitalization, an excretory urogram demonstrated absence of the previously noted pelvic filling defects and the right kidney, pelvis and ureter appeared normal. The patient has had no further difficulty. DISCUSSION
Gross hematuria as a manifestation of sickle cell trait, or disease, has been reported frequently smce the original report of Abel and Brown in
595
SICKLE CELL DISEASE
1948.1-3 Pain, described as a dull ache in the flank, cramplike abdominal pain and renal colic have been associated with gross hematuria, but there are no reports of the occurrence of pain in the absence of gross hematuria. 4- 9 Goodwin reported on a patient with severe renal colic and excretory urographic evidence of renal clot. At cystoscopy the bladder was filled with clots and dark urine. It is not clear whether the patient had gross hematuria.10 The presence of flank pain, pelvic filling defects on excretory urography and grossly clear urine was misleading in the clinical evaluation of 1 Abel, M. S. and Brown, C.R.: Sickle cell disease with severe hematuria simulating renal neoplasm. J.A.M.A., 136: 624-625, 1948. 2 Chapman, A. Z., Reeder, P. S., Freedman, I. A. and Baker, L. A.:. Gross hematuria in sickle cell trait and sickle cell hemoglobin C disease. Amer. J. Med., 19: 773-782, 1955. • Harrison, F. G. and Harrison, F. G., Jr.: Hematuria with sickle cell disease. J. Urol.,
68: 943-949, 1952. 4 Alleyne, G. A. 0. and Went, L. N.: Sickling and haematuria. A preliminary report. W. Indian Med. J., 8: 171-178, 1959. 5 Arduino, L. J.: Urologic complications of sickle cell disease. Amer. Surg., 20: 1213-1218, 1954. 6 Crone, R. I., Jefferson, S. C., Pileggi, V. J. and Lowry, E. C.: Gross hematuria in sickle cell trait. Arch. Int. Med., 100: 597-603, 1957. 7 Lund, H. G., Cordonnier, J. J. and Forbes, K. A.: Gross hematuria in sickle cell disease. J. Urol., 71: 151-158, 1954. 8 Rudner, H. G., Jr. and Rudner, H. G.: Abdominal manifestations of sickle cell anemia. Amer. J. Gastroenterol., 26: 11-21, 1956. 9 Sharpe, A. R., Jr., Fox, P. G., Jr. and Dodson, A. I., Sr.: Unilateral renal hematuria associated with sickle cell C disease and sickle cell trait: study of five patients and review of literature. J. Urol., 81: 780, 1959. 10 Goodwin, W. E., Alston, E. F. and Semans, J. H.: Hematuria and sickle cell disease: unexplained, gross unilateral, renal hematuria in Negroes, coincident with blood sickling trait. J. Urol., 63: 79-96, 1950.
the patients herein presented. Pelvic calculi were seriously considered as the possible cause of the filling defects, particularly in the older patient. The disappearance of the defects and the return of kidneys and collecting systems to normal established the fact that significant bleeding with clot formation can occur without gross hematuria in the patient with sickle cell abnormality. The diagnosis of sickle cell trait, or disease, is established by sickle cell preparation and hemoglobin electrophoresis. Occasional falsely negative sickle cell preparations occur; consequently, it is advisable to repeat this examination several times if one strongly suspects this hemoglobin abnormality. Conservative therapy is the management of choice since the bleeding in most of these patients subsides spontaneously. Blood transfusion is rarely necessary, Neither renal pelvic irrigation with hemostatic agents nor systemic therapy has proved of benefit in controlling bleeding. As in the case of any renal pelvic clot, a period of waiting will allow the clot to resolve. N ephrectomy is not recommended because there is a strong likelihood of subsequent bleeding from the opposite kidney.11· i2 SUMMARY
The case histories of 2 patients with sickle cell trait, renal colic, renal pelvic clots and grossly clear urine are presented. The diagnosis of sickle cell abnormality should be considered in any patient of Negro ancestry who presents with pain which may be of renal origin. Conservative management of such patients is recommended. 11 Allen, T. D.: Sickle cell disease and hematuria: a report of 29 cases. J. Urol., 91: 177, 1964. 12 Lucas, W. M. and Bullock, W. H.: Hematuria in sickle cell disease. J. Urol., 83: 733-741, 1960.
THE JOURNAL OF UROLOGY
Copyright © 1968 by The Williams & Wilkins Co.
SICKLE CELL DISEASE: RENAL COLIC AND MICROSCOPIC HKVIATURIA JOHN F. REDMAN
AND
JACK E. MOBLEY
From the Department of Urology, University of Arkansas Medical Center, Little Rock, Arkansas
Gross hematuria and priapism are the most common urological manifestations of sickle cell trait or disease. Pain of a dull or colicky nature has been reported in association with gross hematuria and the passage of clots from the urinary tract. Recently 2 patients with sickle cell trait, renal colic, clots in the renal collecting system and microscopic hematuria were admitted to the University of Arkansas Medical Center. Since the occurrence of renal pain in the absence of gross hematuria has not been recorded before in patients with sickle cell abnormality, the clinical courses of these patients are presented. CASE REPORTS
Case 1. A 35-year-old Negress was admitted to the medical center; on September 3, 1967. The patient had had intermittent right flank pain of moderate severity for a week before admission. One day before admission the pain was very severe and radiated to the right lower quadrant of the abdomen. The patient stated that she had never had hematuria. On admission her temperature was 102F, pulse rate was 108 per minute and blood pressure was 130/100 mm. Hg. The patient was in obvious discomfort, but the only positive physical finding was right flank tenderness and a palpable, tender right kidney. Laboratory studies were negative except for a positive sickle cell preparation and urinalysis which revealed many red and 10 to 20 white blood cells per high power field. Urine culture was negative. Partial thromboplastin time was 37 .2 seconds, prothrombin time was 13.5 seconds and platelets were considered normal on stained smear of the blood. Examination of the urine for acid-fast bacilli was negative. Hemoglobin electrophoresis confirmed the presence of SA hemoglobin. An excretory urogram demonstrated a nephrogram only on the right side at 20 minutes following the injection of contrast medium. The left kidney appeared normal. A definite pyelogram was not seen on the right side until the 12-hour delayed film, which also demonstrated a pelvic filling defect on the right side. The following day the temperature remained elevated and the patient's condition appeared worse. Anti-
Accepted for publication December 1, 1967. 594
biotics were started and a No. 6 whistle tip ureteral catheter was inserted in a retrograde manner to the right renal pelvis. At the time of cystoscopy the bladder urine was grossly clear and no effiux could be seen from the right ureteral orifice. A drip of dark, thick blood was obtained from the ureteral catheter. vVithin 24 hours the patient was afebrile and remained so. The ureteral catheter was removed after 5 days. A followup excretory urogram made the day after removal of the ureteral catheter was normal. The patient has remained well. Case 2. A 16-year-old Negress was admitted to the medical center on September 7, 1967. The patient had right flank pain which had started several hours before admission and was moderately severe in intensity. The patient stated that she had experienced several episodes of similar pain in the past month. Nausea and vomiting had occurred during the present episode. There had been some urgency and frequency of urination, but the patient denied ever having had hematuria. Physical examination was entirely normal. The patient was afebrile. Urinalysis revealed cloudy yellow urine which contained many red and 16 to 20 white blood cells per high power field. A sickle cell preparation was positive. Hemoglobin electrophoresis demonstrated the presence of SA hemoglobin. A platelet count was 270,000 per cu. mm., the partial thromboplastin time was 44.7 seconds and the prothrombin time was 14.4 seconds. Examination of the urine for acid-fast bacilli was negative. An excretory urogram showed filling defects in the right renal pelvis. The patient was placed at bed rest and observed. Her symptoms subsided without specific therapy. At the end of 5 days of hospitalization, an excretory urogram demonstrated absence of the previously noted pelvic filling defects and the right kidney, pelvis and ureter appeared normal. The patient has had no further difficulty. DISCUSSION
Gross hematuria as a manifestation of sickle cell trait, or disease, has been reported frequently smce the original report of Abel and Brown in
595
SICKLE CELL DISEASE
1948.1-3 Pain, described as a dull ache in the flank, cramplike abdominal pain and renal colic have been associated with gross hematuria, but there are no reports of the occurrence of pain in the absence of gross hematuria. 4- 9 Goodwin reported on a patient with severe renal colic and excretory urographic evidence of renal clot. At cystoscopy the bladder was filled with clots and dark urine. It is not clear whether the patient had gross hematuria.10 The presence of flank pain, pelvic filling defects on excretory urography and grossly clear urine was misleading in the clinical evaluation of 1 Abel, M. S. and Brown, C.R.: Sickle cell disease with severe hematuria simulating renal neoplasm. J.A.M.A., 136: 624-625, 1948. 2 Chapman, A. Z., Reeder, P. S., Freedman, I. A. and Baker, L. A.:. Gross hematuria in sickle cell trait and sickle cell hemoglobin C disease. Amer. J. Med., 19: 773-782, 1955. • Harrison, F. G. and Harrison, F. G., Jr.: Hematuria with sickle cell disease. J. Urol.,
68: 943-949, 1952. 4 Alleyne, G. A. 0. and Went, L. N.: Sickling and haematuria. A preliminary report. W. Indian Med. J., 8: 171-178, 1959. 5 Arduino, L. J.: Urologic complications of sickle cell disease. Amer. Surg., 20: 1213-1218, 1954. 6 Crone, R. I., Jefferson, S. C., Pileggi, V. J. and Lowry, E. C.: Gross hematuria in sickle cell trait. Arch. Int. Med., 100: 597-603, 1957. 7 Lund, H. G., Cordonnier, J. J. and Forbes, K. A.: Gross hematuria in sickle cell disease. J. Urol., 71: 151-158, 1954. 8 Rudner, H. G., Jr. and Rudner, H. G.: Abdominal manifestations of sickle cell anemia. Amer. J. Gastroenterol., 26: 11-21, 1956. 9 Sharpe, A. R., Jr., Fox, P. G., Jr. and Dodson, A. I., Sr.: Unilateral renal hematuria associated with sickle cell C disease and sickle cell trait: study of five patients and review of literature. J. Urol., 81: 780, 1959. 10 Goodwin, W. E., Alston, E. F. and Semans, J. H.: Hematuria and sickle cell disease: unexplained, gross unilateral, renal hematuria in Negroes, coincident with blood sickling trait. J. Urol., 63: 79-96, 1950.
the patients herein presented. Pelvic calculi were seriously considered as the possible cause of the filling defects, particularly in the older patient. The disappearance of the defects and the return of kidneys and collecting systems to normal established the fact that significant bleeding with clot formation can occur without gross hematuria in the patient with sickle cell abnormality. The diagnosis of sickle cell trait, or disease, is established by sickle cell preparation and hemoglobin electrophoresis. Occasional falsely negative sickle cell preparations occur; consequently, it is advisable to repeat this examination several times if one strongly suspects this hemoglobin abnormality. Conservative therapy is the management of choice since the bleeding in most of these patients subsides spontaneously. Blood transfusion is rarely necessary, Neither renal pelvic irrigation with hemostatic agents nor systemic therapy has proved of benefit in controlling bleeding. As in the case of any renal pelvic clot, a period of waiting will allow the clot to resolve. N ephrectomy is not recommended because there is a strong likelihood of subsequent bleeding from the opposite kidney.11· i2 SUMMARY
The case histories of 2 patients with sickle cell trait, renal colic, renal pelvic clots and grossly clear urine are presented. The diagnosis of sickle cell abnormality should be considered in any patient of Negro ancestry who presents with pain which may be of renal origin. Conservative management of such patients is recommended. 11 Allen, T. D.: Sickle cell disease and hematuria: a report of 29 cases. J. Urol., 91: 177, 1964. 12 Lucas, W. M. and Bullock, W. H.: Hematuria in sickle cell disease. J. Urol., 83: 733-741, 1960.