Literature DB >> 22993492

Hereditary spherocytosis in a malay patient with chronic haemolysis.

Muhammad Kamil Sheikh1, Narazah Mohd Yusoff, Gurjeet Kaur, Farhat Aziz Khan.   

Abstract

This case report describes a 35-year-old lady who presented with generalized weakness and lethargy of two weeks duration and jaundice of more than 20 years duration. Her initial workup was suggestive of haemolysis and blood film showed a leucoerythoblastic picture with moderate microspherocytes. She was finally diagnosed as a case of hereditary spherocytosis after ruling out other possible causes of chronic haemolysis and supported by an abnormal osmotic fragility test, although family members refused for screening. Hereditory spherocytosis is uncommon in Malay population and presentation with jaundice of 20 years duration with leucoerythroblastic picture on blood film were interesting features in this case. Patient is being followed closely for need of splenectomy in near future as per severity of haemolysis and currently being managed with folic acid supplement.

Entities:  

Keywords:  Chronic haemolysis; Hereditary spherocytosis; Jaundice; Leucoerythroblastic picture; Microspherocytes

Year:  2007        PMID: 22993492      PMCID: PMC3442627     

Source DB:  PubMed          Journal:  Malays J Med Sci        ISSN: 1394-195X


  7 in total

1.  Genetics of spherocytosis.

Authors:  N E MORTON; A A MACKINNEY; N KOSOWER; R F SCHILLING; M P GRAY
Journal:  Am J Hum Genet       Date:  1962-06       Impact factor: 11.025

2.  Natural history of hereditary spherocytosis during the first year of life.

Authors:  F Delhommeau; T Cynober; P O Schischmanoff; P Rohrlich; J Delaunay; N Mohandas; G Tchernia
Journal:  Blood       Date:  2000-01-15       Impact factor: 22.113

3.  Hereditary spherocytosis in the elderly.

Authors:  E W Friedman; J C Williams; L Van Hook
Journal:  Am J Med       Date:  1988-03       Impact factor: 4.965

4.  Diagnosis of hereditary spherocytosis in newborn infants.

Authors:  W Schröter; E Kahsnitz
Journal:  J Pediatr       Date:  1983-09       Impact factor: 4.406

5.  High prevalence of increased osmotic fragility of red blood cells among Norwegian blood donors.

Authors:  H C Godal; H Heistø
Journal:  Scand J Haematol       Date:  1981-07

6.  Frequency of very late fatal sepsis after splenectomy for hereditary spherocytosis: impact of insufficient antibody response to pneumococcal infection.

Authors:  S W Eber; C M Langendörfer; M Ditzig; D Reinhardt; G Stöhr; W Soldan; W Schröter; G Tchernia
Journal:  Ann Hematol       Date:  1999-11       Impact factor: 3.673

7.  Inheritance pattern and clinical response to splenectomy as a reflection of erythrocyte spectrin deficiency in hereditary spherocytosis.

Authors:  P Agre; A Asimos; J F Casella; C McMillan
Journal:  N Engl J Med       Date:  1986-12-18       Impact factor: 91.245

  7 in total

北京卡尤迪生物科技股份有限公司 © 2022-2023.