Serum Immunoglobulin Levels in Nigerian Patients with Sickle Cell Anaemia in Bone Pain Crisis and Steady State

Special Article - Anaemia

J Blood Disord. 2018; 5(1): 1049.

Serum Immunoglobulin Levels in Nigerian Patients with Sickle Cell Anaemia in Bone Pain Crisis and Steady State

Ino-Ekanem MB1, Ekwere TA1* and Kotila TR2

1Department of Haematology, University of Uyo/ University of Uyo Teaching Hospital, Uyo, Akwa-Ibom State, Nigeria

2Department of Haematology, University College Hospital/College of Medicine, University of Ibadan, Nigeria

*Corresponding author: Ekwere TA, Department of Haematology, University of Uyo/University of Uyo Teaching Hospital, Uyo, Akwa-Ibom State, Nigeria

Received: February 12, 2018; Accepted: April 13, 2018; Published: April 20, 2018


Background: Sickle cell patients are prone to recurrent episodes of vaso-occlusive crisis. Bone pain crises, a form of vaso-occlusive crisis are frequently precipitated by infection. Immunoglobulin is effectors of humoral immunity produced by plasma cells and help fight infection.

We determine serum immunoglobulin levels (IgG, IgA & IgM) in sickle cell patients during bone pain crisis and steady as well as correlate the immunoglobulin levels with total leucocyte count, a known prognostic factor for disease severity.

Methods: 100 participants including 50 sickle cell patients in bone pain crisis, 35 of whom were seen again 4 weeks post crisis (steady state) and 50 healthy Hb AA individuals as controls were recruited. Socio-demographic information of participants was documented. Blood samples was taken for haematological and serum immunoglobulin determination.

Results: The mean level of serum IgG, and IgA during bone pain crisis was significantly higher that of the HbAA controls (p= 0.001 respectively). Serum IgG and IgA during bone pain crisis was also significantly higher than during steady state (p=0.01 & 0.034 respectively). Furthermore, the mean leucocyte count though significantly higher during bone pain crisis than during steady state (p=0.001), however it did not correlate with the serum immunoglobulin levels (p =0.395, 0.810, 0.746 for IgG, IgA and IgM respectively).

Conclusion: Serum immunoglobulin especially IgG and IgA was significantly higher during bone pain compared with Hb AA controls as well as during steady state. However, the immunoglobulin levels did not correlate with total leucocyte count, a known prognostic factor for disease severity.

Keywords: Sickle cell anaemia; Vaso-occlusive crisis; Bone pain crisis; Steady state; Immunoglobulin.




Sickle Cell Disease (SCD) is a group of haemoglobin disorder resulting from the inheritance of the sickle β-globin gene. The homozygous state (Hb SS or sickle cell anaemia) is the most common form of the disease, however the interaction of the abnormal haemoglobin S (HbS) with thalassemia and other haemoglobin variants also result in sickling [1,2].

SCD is a major public health problem and the most common genetic disease disorder that affect mankind [3]. The disease has a global distribution, but certain regions of the world have high prevalence for the sickle cell gene including Equatorial Africa, Sicily in Southern Italy, Middle East and Central India [3,4].

In Nigeria, the magnitude of the problem is much more pronounced given that 25% of the population carry the sickle cell gene [5]. The World Health Organization (WHO) estimates the prevalence of SCA to be 20 per 1000 live birth annually which translate to about 150,000 children born annually with SCA in Nigeria, thus making Nigeria the country with the highest disease burden globally [5,6].

The common and most distressing clinical manifestation of SCA is the episodic and unpredictable sickle cell vaso-occlusive crisis (VOC) [7]. The crisis occurs unpredictably over a life-time and account for most common cause of emergency room visits and hospitalization. The most common acute VOC is the Bone Pain Crisis (BPC). BPC is defined as painful episodes involving one or more sites of bones in the absence of preceding trauma or obvious infection [5]. The most common sites are the long bones of the limbs, lumbar spine, and thoracic cage.

Malaria bacterial and viral infection, are the most common precipitating factors for VOC and leading cause of mortality in SCD [8]. SCA patients are prone to infection because of a defect in the alternative pathway of complement activation and opsonisation [9,10] reduced ability of neutrophils to kill pathogens [9,11] and functional hyposplenism further decreasing macrophage function and immune response to organisms in the blood [9,12].

Humoral immunity is unimpaired in SCA and serum immunoglobulin levels tend to be high in response to frequent infection [9,13,14]. Infection precipitates VOC by increasing the interaction of leucocytes with vascular endothelium [15]. Acute inflammatory reaction to infection or tissue injury increases local and circulating levels of tissue necrotic factor alpha (TNF-α) and interleukin-Iβ (IL-Iβ) which activates leucocytes to express more adhesion molecules (α2Mβ2 & L-selectin) and vascular endothelial cells express more ligands (intercellular adhesion molecule –ICAM, vascular adhesion molecule-VCAM-1) for the adhesion molecule of erythrocytes and leucocytes [15]. This increase aggregation of blood cells to each other and their aggregation to the vascular endothelium leading to vaso-occlusion.