Hereditary spherocytosis

Hereditary spherocytosis is a congenital hemolytic anemia with a wide clinical spectrum (from symptom-free carriers to severe hemolysis) characterized by anemia, variable jaundice, splenomegaly and cholelithiasis.

Jaundice

Yellow pigmentation of the skin due to bilirubin, which in turn is the result of increased bilirubin concentration in the bloodstream.


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PMID (PMCID)
20618221
 MALE Child
Orthotopic heart transplantation in a child with hereditary spherocytosis.
Johnson CE, Schmitz ML, McKamie WA, Edens RE, Imamura M, Jaquiss RD.
Artif Organs. 2010;34(12):1154-6.
Hereditary spherocytosis (HS) is a genetic, frequently familial hemolytic blood disease that presents with varying degrees of hemolytic anemia, splenomegaly, and jaundice.The disease arises as a result of defects in any of a number of proteins responsible for maintaining the shape and flexibility of the red blood cell, resulting in an osmotically fragile and characteristically spherical red blood cell.
19740448
(2753340)
 MALE Infant
Complications of Evans' syndrome in an infant with hereditary spherocytosis: a case report.
Yoshida H, Ishida H, Yoshihara T, Oyamada T, Kuwana M, Imamura T, Morimoto A.
J Hematol Oncol. 2009;2:40.
Hereditary spherocytosis (HS) is a genetic disorder of the red blood cell membrane clinically characterized by anemia, jaundice and splenomegaly.
12818755
 MALE Infant, Newborn
[Recombinant Human Erythropoietin (r-HuEPO) therapy in a newborn with hereditary spherocytosis].
Schiff M, Hays S, Sann L, Putet G.
Arch Pediatr. 2003;10(4):333-6.
Hereditary spherocytosis was diagnosed in a newborn who had severe and early jaundice.
11233774
 MALE Middle Aged
Massive hemothorax due to intrathoracic extramedullary hematopoiesis in a patient with hereditary spherocytosis.
Xiros N, Economopoulos T, Papageorgiou E, Mantzios G, Raptis S.
Ann Hematol. 2001;80(1):38-40.
We report on a 64-year-old man with hereditary spherocytosis, who presented with anemia, jaundice, intrathoracic EMH, and massive hemothorax.
7450669
 FEMALE Adult
[A case of hereditary spherocytosis--role of complement system in hemolysis (author's transl)].
Oikawa I, Hiratsuka M, Kasai S, Sekiguchi S.
Hokkaido Igaku Zasshi. 1980;55(3):235-8.
A female patient, 27 years old, with the chief complaint of anemia and jaundice, was diagnosed as hereditary spherocytosis with a complication of cholelithiasis and was examined before and after the operation of cholecystectomy and splenectomy.
686044
 MALE
Extreme hyperbilirubinemia in a patient with hereditary spherocytosis, Gilbert's syndrome, and obstructive jaundice.
Katz ME, Weinstein IM.
Am J Med Sci. 1978;275(3):373-9.
Extreme hyperbilirubinemia in a patient with hereditary spherocytosis, Gilbert's syndrome, and obstructive jaundice.