Hemolysis-associated hemoglobin cast nephropathy results from a range of clinicopathologic disorders

Abstract

Intravascular hemolysis is relatively rare but can lead to acute kidney injury (AKI), from increased destruction of erythrocytes and release of free hemoglobin. Since hemolysis and hemoglobinuria are known causes of acute kidney injury we sought to define clinicopathologic findings and outcomes of patients with hemolysisassociated hemoglobin cast nephropathy through a retrospective analysis of 27 cases. The mean patient age was 47 years (range 19-79) and the female-to-male ratio was 1.3:1. All patients presented with AKI with a mean serum creatinine of 8.0 (range 2.9-17.0) mg/dL. Etiologies included autoimmune hemolytic anemia (30%), medication (26%), paroxysmal nocturnal hemoglobinuria (7%), procedural/mechanical causes (7%), transfusion of incompatible blood (4%), toxin ingestion (4%), disseminated intravascular coagulation (4%), and hemoglobinopathy (4%). All biopsies showed acute tubular injury and pigmented, proteinaceous casts characterized by positive hemoglobin immunohistochemistry. After a mean follow-up of nine months (range 0.5-26), the mean serum creatinine was 1.3 (range 0.6-3.3) mg/dL, with 78% of patients returning to normal kidney function. Thus, based on our clinicopathologic case series, hemolysisassociated hemoglobin cast nephropathy is an important entity for clinicians and pathologists to recognize as treatment hinges upon elimination of the pathogenic driver of intravascular hemolysis.

Circulating FH Protects Kidneys From Tubular Injury During Systemic Hemolysis

“…Hemolysis leads to pronounced tubular injury (2,(32)(33)(34)(35). Complement AP activation is an important pathogenic mechanism in the development of AKI, especially in the tubulointerstitium, where it has been shown to drive severe injury (19,20,36).…”

Hemoglobin Cast Nephropathy

“…Hemolysis results in hemoglobinuria, which can lead to acute and chronic kidney injury. 2,4,5 Hemoglobin released into the circulation binds to haptoglobin, which is not filtered by the glomerulus owing to its large size, and is degraded in the reticuloendothelial system. During overwhelming hemolysis, haptoglobin molecules are saturated and free hemoglobin levels rise in the blood.…”

“…Few studies have demonstrated immunohistochemically proven hemoglobin casts on kidney biopsy associated with intravascular hemolysis. 2,3 We present 2 distinct cases of hemolysis-associated hemoglobin cast nephropathy related to the use of rifampin and autoimmune hemolytic anemia.…”

Gross Hematuria in Patients with Wasp Sting: A Retrospective Study

“…So that patients who develop gross hematuria are likely those who are most severely affected by the venom. While rhabdomyolysis and intravascular hemolysis can cause AKI, the renal injury can be exacerbated in the states of shock [35,[37][38][39]. However it is rather di cult to explain why patients in the gross hematuria group had developed less shock than in non-gross hematuria group.…”