title: Previously undiagnosed hereditary spherocytosis in a patient with jaundice and pyelonephritis: a case report.
abstract: BACKGROUND
  Hereditary spherocytosis is autosomal dominant inherited extravascular hemolytic disorder and is the commonest cause of inherited hemolysis in northern Europe and the United States. The classical clinical features of hereditary spherocytosis are anemia, jaundice, and splenomegaly. However, all of these classical features are not always revealed in the case of mild hemolysis or when hemolysis is well compensated. Patients with hereditary spherocytosis may remain undiagnosed for years if their hemolysis is mild.
  
  
  METHODS
  A 42-year-old Asian woman presented to our clinic with a sudden onset of high fever with shaking chills and jaundice, suggesting septicemia; however, following detailed investigation, the patient was diagnosed with pyelonephritis and accelerated hemolysis of hereditary spherocytosis due to infection.
  
  
  CONCLUSIONS
  It is important to note that transient anemia or jaundice can sometimes be the only initial presenting symptoms in cases of undiagnosed latent hereditary spherocytosis. This case also highlights the fact that physicians should consider concomitant hemolytic disease in patients in whom jaundice and infections that rarely cause jaundice coexist.
authors.affiliation: Miyake Central Clinic, 1859-16, Kamitsuki, Miyake, Tokyo, Japan. ytateno13044@gmail.com.
authors.forename: Yuki
authors.initials: Y
authors.lastname: Tateno
authors.affiliation: Miyake Central Clinic, 1859-16, Kamitsuki, Miyake, Tokyo, Japan.
authors.forename: Ryoji
authors.initials: R
authors.lastname: Suzuki
authors.affiliation: Miyake Central Clinic, 1859-16, Kamitsuki, Miyake, Tokyo, Japan.
authors.forename: Yukihiro
authors.initials: Y
authors.lastname: Kitamura
mesh_terms: D034841:Abdominal Cavity / Q000000981:diagnostic imaging*; D000328:Adult; D000900:Anti-Bacterial Agents / Q000008:administration & dosage; D002506:Cephalexin / Q000008:administration & dosage; D005260:Female; D006801:Humans; D007565:Jaundice / Q000150:complications*; D000077731:Meropenem; D009996:Osmotic Fragility / Q000235:genetics; D011704:Pyelonephritis / Q000175:diagnosis* / Q000188:drug therapy; D011856:Radiographic Image Enhancement; D013103:Spherocytosis, Hereditary / Q000175:diagnosis* / Q000188:drug therapy / Q000473:pathology; D013163:Splenomegaly / Q000000981:diagnostic imaging* / Q000473:pathology; D013845:Thienamycins / Q000008:administration & dosage; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome
chemical_list: D000900:Anti-Bacterial Agents; D013845:Thienamycins; D000077731:Meropenem; D002506:Cephalexin
keywords: Anemia; Hemolytic disease; Hereditary spherocytosis; Jaundice