Sickle Cell Trait Hematuria Research Articles

Hematuria in sickle cell trait: the importance of ruling out occult cancer

Dear Editor, Sickle cell trait is characterized by a heterozygous inheritance of the sickle hemoglobin gene. Unlike sickle cell disease, sickle cell trait is commonly viewed as a benign process in asymptomatic patients [1]. However, a growing number of case reports and observational studies reveal that complications do occur in these individuals [2]. Both microscopic and gross hematuria are the most common complication [3]. About 50% of these cases have been attributed to renal papillary necrosis [4]. Despite this association, the diagnostic workup for cases of hematuria should always include a urine analysis, urine culture, urine cytology, computed tomography (CT) with and without contrast, and cystoscopy with ureteroscopy if necessary. This report aims to highlight the importance of careful diagnostic workup in patients with sickle cell trait presenting with hematuria. A 49-year-old African–American male with sickle cell trait presented with a history of intermittent, sharp left flank pain, and gross hematuria throughout micturition. Aside from hematuria, all other studies including a noncontrast abdomen/pelvis CT scan were unremarkable. His condition was initially attributed to a kidney stone due to spontaneous resolution. However, these episodes recurred with passage of dark red tissue 3 weeks later. A repeat CT was ordered, this time with contrast, along with cystoscopy. Both studies were again negative despite persistence of his symptoms. Given his long history of sickle cell trait and a negative workup, his hematuria was attributed to papillary necrosis. However, his flank pain progressively worsened with continued passage of necrotic tissue, unrelieved by hydration and urine alkalization. He was referred to the hematology clinic where repeat studies were obtained given the severity and increased frequency of his symptoms. An abdomen/pelvis CT revealed a filling defect in the upper collecting system of the left kidney (Fig. 1), suspicious for malignancy, along with multiple subcentimeter periaortic lymph nodes. A retrograde pyelogram confirmed these findings. Ureteroscopy and biopsy demonstrated a high-grade, non-invasive papillary urothelial carcinoma involving the renal pelvis, extending into the left proximal ureter. A metastatic workup including cystoscopy was unremarkable aside from previously noted lymphadenopathy. Laparoscopic left nephroureterectomy was performed with no further evidence of cancer. Two months later, multiple bladder lesions, biopsy-proven as “drop metastases”, were found on cystoscopy and treated with fulguration. He was treated with intravesical mitomycin C and a 6-week course of Bacille Calmette-Guerin (BCG). Since that time, his adenopathy has been stable and he has had only one recurrence at the bladder dome that was treated with fulguration. Intravesical BCG will be continued for another 6 weeks with frequent monitoring for recurrence. B. S. Gill (*) School of Medicine, The George Washington University, 922 24th Street NW, Apt 421, Washington, DC 20037, USA e-mail: bsgill@gwmail.gwu.edu

Protracted, gross hematuria in sickle cell trait: response to multiple doses of 1-desamino-8-D-arginine vasopressin

Protracted, gross hematuria in sickle cell trait: response to multiple doses of 1-desamino-8-D-arginine vasopressin

The effect of bicarbonate and distilled water on sickle cell trait hematuria and in vitro studies on the interaction of osmolarity and pH on erythrocyte sickling in sickle cell trait

The effect of bicarbonate and distilled water on sickle cell trait hematuria and in vitro studies on the interaction of osmolarity and pH on erythrocyte sickling in sickle cell trait

The Effect of Bicarbonate and Distilled Water on Sickle Cell Trait Hematuria and in Vitro Studies on the Interaction of Osmolality and pH on Erythrocyte Sickling in Sickle Cell Trait

The effect of intravenously administered distilled water was examined alone and during alkalization in a patient with gross hematuria associated with the sickle cell trait. On each of 4 occasions hematuria ceased promptly after the infusion of distilled water. Bicarbonate therapy also consistently decreased hematuria.In vitro studies on erythrocytes from another patient with sickle ceil trait and hematuria demonstrated that slight increases in urinary pH similar to those that occur in the urine during alkalization can reverse or prevent erythrocyte sickling in the sickle cell trait.If patients with the sickle cell trait are hydrated adequately and have a good rate of urine flow distilled water can be given intravenously with virtually no danger of acute tubular necrosis secondary to erythrocyte hemolysis.

Medical management of refractory hematuria in sickle-cell trait

Hematuria secondary to sickle-cell trait has on occasion proved refractory to medical management. We have adopted a new six-drug regimen to deal with this difficult problem. Herein is reported our experience with this protocol in 2 patients. Our results have been excellent, with hematuria clearing within twenty-four hours. The drug regimen is used in an attempt to alter the conditions that are favorable for sickling in the renal medulla.

Oral urea treatment of hematuria in sickle-cell trait

Oral urea treatment of hematuria in sickle-cell trait

Hematuria in sickle cell trait: the effect of intravenous administration of distilled water, urinary alkalinization, and diuresis.

Hematuria in patients with sickle cell trait might be related to sickling in the medullary vessels of the kidney. In vitro, hypertonicity, acidity, and anoxia may cause sickling. These conditions may also exist in the renal medulla of man. In two patients with long-standing hematuria associated with sickle cell trait, urinary hemoglobin loss consistently diminished or transiently stopped following urinary alkalinization or certain measures which theoretically diminish medullary tonicity and increase medullary oxygen tension. Transient cessation of hematuria also occurred repeatedly after an infusion of distilled water. This phenomenon may be related to red cell expansion and dilution of S-hemoglobin and supports experimental evidence that a critical concentration of S-hemoglobin is a prerequisite for sickling.

Gross hematuria in sickle cell trait and sickle cell hemoglobin-C disease

Summary 1.The occurrence of gross hematuria in five cases of sickle cell trait and three cases of sickle cell hemoglobin-C disease is reported. The investigation of these patients included electrophoretic and fetal hemoglobin studies. 2.Some of the previous cases of sickle cell trait reported with symptoms of crisis and/or hematuria might well have been sickle cell hemoglobin-C disease. The literature is reviewed in this connection. 3.The finding of hematuria with sickle cell trait in a significant number of patients is believed to indicate a significant relationship. 4.The singular susceptibility of the kidney to stress associated with sickle cell trait is offered as a possible etiologic mechanism for the vascular defects causing hematuria.