Sickle Cell Disease Clinical Classification

Introduction:

Sickle cell disease (SCD) is one of the most common genetic disorders. The abnormality is due to the substitution of a glutamic acid by a valine at the sixth position on the β-globin chain , resulting in pathologic hemoglobin called hemoglobin S, this results from an autosomal recessive mutation on chromosome 11 [1]. The classic physiopathology of the SCD is based on the polymerization of hemoglobin S, followed by sickling and dehydration of the red blood cells (RBCs). The slowing down of blood circulation and obstruction of the microcirculation by the deformed RBCs is the reason of the manifestations and complications of SCD. [2]. SCD may lead to various clinical acute and chronic complications, which have a high mortality rate . The laboratory genotyping and phenotyping classifications don't indicate the clinical severity of sickle cell disease , many SCD patients have underling serous complicaions , which make SCD clinicaly sever moderate or mild inspite the similiraty in in the HPLC result . Aim: This study is to grade SCD clinical severity based on the number and seriousness of the complications .

Method:

In his on going project 30 SCD patients from both age and gender . The clinical evalation performed along with th following tests complete blood count , kidney fuunction test , liver function test , serum ferritin , coagulation profile , antibody sceerning , high performance liquid chromatography (HPLC) , chest X-Ray , ECG , Echocardiography .The patients also assessed from psychology and social point of view . The grades of the disease are 15 according to the number of the complications the severity is mild , moderate and sever .Mild disease is 1-5 moderate 6-10 and severe 11-15 complications . The severity of the more than 1is sever less than 1 is moderate , less than 0.5 is mild . The factor calculated as any sever X 3 , any moderate multiply by 2 qnd any moderate is multiply by 1 and he normal is 0 table 1 & table 2 .

Results :

In the 30 patients they showed in the 1-kidneynormal ( N ) 17 , mild ( MI ) 2 , moderate ( MO ) 4 , Severe ( SV ) 7; 2-Liver N 4 , MI 6 , MO 16 , SV 4; 3-Spleen N 5, MI 2 , MO 11 , SV 12;

4-Gall bladder ( GB ) N 4, MI 1, MO 10 , SV 15; 5-Bone N 5, MI 5, MO 7 , SV 13; 6-Genitalia ( Gent ) N 15, MI 11, MO 3 , SV 1; 7-Respiratory ( Resp ) N 22, MI 1, MO 3 , SV 4; 8-Cardiovascular system ( CVS ) N 15, MI 3, MO 2 , SV 10; 9-Cental Nervous System ( CNS ) N 20, MI 5, MO 2 , SV 3; 10-TX N O, MI 15, MO 10, SV 5; 11-Antibodies ( Ab ) N 22, MI 3, MO 2 , SV 3; 12-Ferritin N 10, MI 4, MO 5 , SV 11; 13-Psychiatry (Psy) N 5, MI 4, MO 6 , SV 15; 14-Social ( SOC ) N 10, MI 6, MO 5 , SV 9; 15-Pain N 0, MI 5, MO 15 , SV 10;

Conclusion:

The clinical classification for sickle cell disease is mandatory in this systemic disorder. The disease graded clinically according to 15 systemic complicatins each clinical grades from 1-15 are are given SCD1-15. The clinical severity based on the number the severit of the complications .The clinical classification also determind by the severity score of the complications .

References:

1. Akakpo-Numado GK, Boume MA, Mihluedo-Agbolan KA, Sanni YS, Gnassingbe K, Tekou H. Osteoarticular complications of sickle cell disease in

children. Hard Tissue. 2013; 2: 26

2. Al-Jafar HA, Al-Ali NS, Ali A and Alobaid A. Medical and Surgical Treatment Options for Early Osteonecrosis in Sickle Cell Disease. Ann Hematol Oncol. 2016; 3(5): 1092.