Clinical features of patients with hemolytic anemia due to red blood cells membrane defect

  • Pustika Amalia W
  • Djajadiman Gatot
  • Teny Tjitrasari
  • Iswari Setianingsih
  • Nanis Sacharis Marzuki
Keywords: Hemolytic anemia, red blood cells membrane defect, thalassemia, DNA analysis

Abstract

Background Hemolytic anemia may result from corpuscular or
extracorpuscular abnormalities. One of the types of corpuscular
abnormalities is membrane defect. The diagnosis is sometimes
difficult and it may need special hematologic investigations. There
are no data yet on the clinical features of red blood cell membrane
defect in Cipto Mangunkusumo Hospital.
Objective To evaluate the clinical features and laboratory find-
ings of patients with hemolytic anemia due to red blood cells mem-
brane defect in Cipto Mangunkusumo Hospital.
Methods This was a descriptive study on patients with red blood
cells membrane defect who came to the Thalassemia Center at
Cipto Mangunkusumo Hospital during 2002-2004.
Results In 2002-2004, there were 241 new cases of hemolytic
anemia consisted of 116 patients with beta-thalassemia, 109 with
HbE-beta thalassemia, 3 with alpha-thalassemia, and 13 with red
blood cells membrane defect. The red cells membrane defect pa-
tients consisted of 4 males and 9 females, ranging in age from 1
months to 14 years. All subjects came to the hospital due to pale-
ness as a chief compaint. Hepato-splenomegaly was found in 5 of
13 cases. Laboratory findings revealed hemoglobin level 6.4-13.1
g/dl (mean 9.4+2.1 g/dl), MCV 58.4-94.5 fl (mean 81.2+10.2 fl),
MCHC 31.7-35.8 g/dl (mean 33.9+1.1g/dl), RDW 15.8-28.4%
(mean 20.1+3.6%) and normal hemoglobin electrophoresis. Pe-
ripheral blood smear showed anisocytosis, poikilocytosis,
spherocytes, ovalocytes, stomatocytes, target cells, and fragmented
cells. The most common diagnosis in this group was Southeast
Asian Ovalocytosis (5/13).
Conclusions In facing hemolytic anemia with normal Hb electro-
phoresis or normal RBC enzyme level, the possibility of red cells
membrane defect should be taken into consideration as a cause
of this disorder. The clinical features and laboratory findings of red
blood cells membrane defect patients are highly variable. Occa-
sionally, hematologic investigations are necessary

Author Biographies

Pustika Amalia W
Department of Child Health, Medical School, University of
Indonesia, Cipto Mangunkusumo Hospital, Jakarta, Indonesia
Djajadiman Gatot
Department of Child Health, Medical School, University of
Indonesia, Cipto Mangunkusumo Hospital, Jakarta, Indonesia
Teny Tjitrasari
Department of Child Health, Medical School, University of
Indonesia, Cipto Mangunkusumo Hospital, Jakarta, Indonesia
Iswari Setianingsih
Eijkman Institute for Molecular Biology, Jakarta, Indonesia
Nanis Sacharis Marzuki
Eijkman Institute for Molecular Biology, Jakarta, Indonesia

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Published
2016-10-18
How to Cite
1.
W P, Gatot D, Tjitrasari T, Setianingsih I, Marzuki N. Clinical features of patients with hemolytic anemia due to red blood cells membrane defect. PI [Internet]. 18Oct.2016 [cited 28Mar.2024];46(1):41-. Available from: https://paediatricaindonesiana.org/index.php/paediatrica-indonesiana/article/view/853
Section
Articles
Received 2016-10-10
Accepted 2016-10-10
Published 2016-10-18