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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.

Hemolytic anemia

A type of anemia caused by premature destruction of red blood cells (hemolysis).

Total: 15

(per page)

PMID (PMCID)
27108201	MIXED_SAMPLE	Adult
	Disease-modifying influences of coexistent G6PD-deficiency, Gilbert syndrome and deletional alpha thalassemia in hereditary spherocytosis: A report of three cases.
	Jamwal M, Aggarwal A, Kumar V, Sharma P, Sachdeva MU, Bansal D, Malhotra P, Das R. Clin Chim Acta. 2016;458:51-4.
	Hereditary spherocytosis (HS) is a common inherited hemolytic anemia characterized by heterogeneous clinical presentations with variable degrees of anemia, jaundice, splenomegaly and gallstones.
27566068 (5002123)	MALE	Middle Aged
	Open-heart surgery using a centrifugal pump: a case of hereditary spherocytosis.
	Matsuzaki Y, Tomioka H, Saso M, Azuma T, Saito S, Aomi S, Yamazaki K. J Cardiothorac Surg. 2016;11(1):138.
	Hereditary spherocytosis is a genetic, frequently familial hemolytic blood disease characterized by varying degrees of hemolytic anemia, splenomegaly, and jaundice.
26711368	MALE
	Pyoderma Gangrenosum in a Patient with Hereditary Spherocytosis.
	Kwon HI, Paek JO, Kim JE, Ro YS, Ko JY. Int J Low Extrem Wounds. 2016;15(1):92-5.
	However, there have been no previous reports of PG in a patient with hereditary spherocytosis, a common inherited hemolytic anemia.
26379354 (4567607)	OTHER
	Hereditary Spherocytosis Unmasked by Human Parvovirus B19 Induced Aplastic Crisis in a Family.
	Alavi S Md, Arabi N Md, Yazdi MK Md, Arzanian MT Md, Zohrehbandian F MSc. Iran J Med Sci. 2015;40(5):461-4.
	The clinical importance of this report is that in the case of an abrupt onset of unexplained severe anemia and jaundice, one should consider underlying hemolytic anemias mostly hereditary spherocytosis complicated by HPV B19 aplastic crisis.
25587528 (4291453)	OTHER
	Coexistence of gilbert syndrome and hereditary spherocytosis in a child presenting with extreme jaundice.
	Lee JH, Moon KR. Pediatr Gastroenterol Hepatol Nutr. 2014;17(4):266-9.
	Hereditary spherocytosis is the most common inherited hemolytic anemia and is characterized by spherical, osmotically fragile erythrocytes that are selectively trapped by the spleen.
23724634	MALE
	A case of hereditary spherocytosis misdiagnosed as pyruvate kinase deficient hemolytic anemia.
	Vercellati C, Marcello AP, Fermo E, Barcellini W, Zanella A, Bianchi P. Clin Lab. 2013;59(3-4):421-4.
	A case of hereditary spherocytosis misdiagnosed as pyruvate kinase deficient hemolytic anemia.
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.
19373154	FEMALE	Young Adult
	Dural venous thrombosis following splenectomy in a patient with hereditary spherocytosis.
	Perkins LA, Jones SF, Bhargava RS. South Med J. 2009;102(5):542-5.
	Hereditary spherocytosis is a common form of hemolytic anemia sometimes requiring splenectomy in recalcitrant cases.
18520574	FEMALE	Middle Aged
	Pulmonary extramedullary hematopoiesis.
	Bowling MR, Cauthen CG, Perry CD, Patel NP, Bergman S, Link KM, Sane AC, Conforti JF. J Thorac Imaging. 2008;23(2):138-41.
	There have been several reports of patients presenting with pulmonary EMH published in the literature and the majority are due to a secondary process, such as myeloproliferative disorders, hemolytic anemias, hereditary spherocytosis, and Gaucher disease.
15919335	OTHER	Infant
	Beating heart off-pump myocardial revascularization in an infant.
	Walther T, Dahnert I, Kiefer H, Falk V, Kostelka M, Mohr FW. Ann Thorac Surg. 2005;79(6):2151-3.
	Reimplantation of the coronary artery with cardiopulmonary bypass was impossible because the patient also had hereditary spherocytosis, which put him at risk for hemolytic anemia.
8471774	FEMALE	Adult
	Human erythrocyte protein 4.2 deficiency associated with hemolytic anemia and a homozygous 40glutamic acid-->lysine substitution in the cytoplasmic domain of band 3 (band 3Montefiore).
	Rybicki AC, Qiu JJ, Musto S, Rosen NL, Nagel RL, Schwartz RS. Blood. 1993;81(8):2155-65.
	Red blood cell (RBC) protein 4.2 deficiency is often associated with a moderate nonimmune hemolytic anemia, splenomegaly, and osmotically fragile RBCs resembling, but not identical to, hereditary spherocytosis (HS).
8512401	MIXED_SAMPLE	Middle Aged
	Open heart operation in patients suffering from hereditary spherocytosis.
	Gayyed NL, Bouboulis N, Holden MP. Ann Thorac Surg. 1993;55(6):1497-500.
	Hereditary spherocytosis is a clinically heterogeneous, genetically determined red blood cell membrane disorder resulting in hemolytic anemia.
1948000	FEMALE	Adult
	Coexistence of hereditary spherocytosis and beta-thalassemia: case report of severe hemolytic anemia in an American black.
	White BP, Farver M. S D J Med. 1991;44(9):257-61.
	Coexistence of hereditary spherocytosis and beta-thalassemia: case report of severe hemolytic anemia in an American black.
2445253	MIXED_SAMPLE	Child
	The affinity glycated hemoglobin in a family with hereditary spherocytosis and in other non-hemoglobinopathic hemolytic anemias.
	Krauss JS, Hahn DA, Harper D, Shell S, Baisden CR. Ann Clin Lab Sci. 1987;17(5):331-8.
	Therefore, the affinity GHb and HbF was determined in four members of an HS family and in nine other cases of non-hemoglobinopathic hemolytic anemia, including three autoimmune hemolytic anemias, four red cell fragmentation syndromes (two "Waring blender" syndromes, one thrombotic thrombocytopenic purpura in association with tumor, and one case of disseminated intravascular coagulation), and two red cell membrane defects: paroxysmal nocturnal hemoglobinuria and another case of hereditary spherocytosis.
7350706	MIXED_SAMPLE	Child
	Circulatory control of splenic hyperfunction in children with peripheral blood dyscrasia.
	Witte CL, Corrigan JJ Jr, Witte MH, Van Wyck DB, O'Mara RE, Woolfenden JM. Surg Gynecol Obstet. 1980;150(1):75-80.
	While this procedure initially improved the peripheral blood values in five patients--two patients with hereditary spherocytosis, one patient with idiopathic thrombocytopenic purpura, one patient with pyruvate-kinase hemolytic anemia and one patient with posthepatitic cirrhosis and splenomegaly--the hematologic derangement gradually recurred in four, necessitating eventual splenectomy in two.