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.

Cholelithiasis

Hard, pebble-like deposits that form within the gallbladder.


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PMID (PMCID)
27423290
 MALE Child
Hereditary Spherocytosis with Splenomegaly and Cholelithiasis in a Young Male of Western Region of Nepal - A Case Report.
Ghimire P, Gurung NV, Shrestha S, Poudel SR, Chapagain A.
Kathmandu Univ Med J (KUMJ). 2015;13(52):366-8.
Hereditary spherocytosis is an autosomal dominant congenital hemolytic anemia due to defect in RBC membrane protein that commonly presents with intermittent jaundice, anemia, abdominal pain, splenomegaly and sometimes cholelithiasis.
27423290
 MALE Child
Hereditary Spherocytosis with Splenomegaly and Cholelithiasis in a Young Male of Western Region of Nepal - A Case Report.
Ghimire P, Gurung NV, Shrestha S, Poudel SR, Chapagain A.
Kathmandu Univ Med J (KUMJ). 2015;13(52):366-8.
This is a report of an 11 years old male diagnosed case of hereditary spherocytosis who presented with jaundice, splenomegaly and cholelithiasis.
17917996
 MIXED_SAMPLE Adult
Laparoscopic splenectomy plus cholecystectomy for treating hereditary spherocytosis combined with cholelithiasis in siblings.
Choi YS, Han HS, Yoon YS, Jang JY, Kim SW, Park YH.
Minim Invasive Ther Allied Technol. 2007;16(5):317-8.
In this paper we describe how we perform laparoscopic splenectomy plus cholecystectomy at the same time to treat hereditary spherocytosis combined with cholelithiasis in two siblings.
17917996
 MIXED_SAMPLE Adult
Laparoscopic splenectomy plus cholecystectomy for treating hereditary spherocytosis combined with cholelithiasis in siblings.
Choi YS, Han HS, Yoon YS, Jang JY, Kim SW, Park YH.
Minim Invasive Ther Allied Technol. 2007;16(5):317-8.
Laparoscopic splenectomy plus cholecystectomy for treating hereditary spherocytosis combined with cholelithiasis in siblings.
9168768
 FEMALE
Concomitant laparoscopic cholecystectomy and splenectomy for surgical management of hereditary spherocytosis.
Patton ML, Moss BE, Haith LR Jr, Shotwell BA, Milliner DH, Simeone MR, Kraut JD, Patton JN.
Am Surg. 1997;63(6):536-9.
In conclusion, we present a combined laparoscopic cholecystectomy and splenectomy for hereditary spherocytosis associated with splenomegaly, cholelithiasis, and cholecystitis.
9168768
 FEMALE
Concomitant laparoscopic cholecystectomy and splenectomy for surgical management of hereditary spherocytosis.
Patton ML, Moss BE, Haith LR Jr, Shotwell BA, Milliner DH, Simeone MR, Kraut JD, Patton JN.
Am Surg. 1997;63(6):536-9.
Hereditary spherocytosis is the most common red blood cell membrane disorder, and its diagnosis is often associated with hemolytic crisis and premature cholelithiasis.
2667500
 MALE Adult
Ectopic or heterotopic liver (choristoma) associated with the gallbladder.
Tejada E, Danielson C.
Arch Pathol Lab Med. 1989;113(8):950-2.
A 43-year-old man with a history and symptomatology of hereditary spherocytosis underwent splenectomy and cholecystectomy for treatment of his underlying disease and of cholelithiasis.
6086995
 MALE
An autopsy case of hemochromatosis and hepatoma combined with hereditary spherocytosis.
Takegoshi T, Nishino T, Tanino M, Nonokura A, Ohta G.
Jpn J Med. 1984;23(1):48-52.
When the patient, a 56 years old man, was admitted for receiving cholecystectomy for cholelithiasis, a diagnosis of hemochromatosis secondary to hereditary spherocytosis was made by liver biopsy and hematological examination data.
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.
413463
 MIXED_SAMPLE Middle Aged
Monoclonal gammopathy in hereditary spherocytosis: a possible pathogenetic relation.
Schafer AI, Miller JB, Lester EP, Bowers TK, Jacob HS.
Ann Intern Med. 1978;88(1):45-6.
We propose that the chronic reticuloendothelial stimulation due to extravascular hemolysis, possibly potentiated by the inflammation associated with cholelithiasis and cholecystitis, may foster neoplastic transformation of immunocytes in patients with hereditary spherocytosis, ultimately leading to the development of monoclonal gammopathy.