Journal of Hematology, ISSN 1927-1212 print, 1927-1220 online, Open Access |
Article copyright, the authors; Journal compilation copyright, J Hematol and Elmer Press Inc |
Journal website https://www.thejh.org |
Original Article
Volume 11, Number 3, June 2022, pages 92-108
Hematological, Biochemical Properties, and Clinical Correlates of Hemoglobin S Variant Disorder: A New Insight Into Sickle Cell Trait
Figures
Tables
Variable, n (%) | Hb S variant (group 1) (n = 55) | Controls (group 2) (n = 60) | P- value | SCD (group 1a) (n = 24) | SCT (group 1b) (n = 31) | P-value |
---|---|---|---|---|---|---|
P value was calculated with unpaired sample t-test. *P value was calculated with the Mann-Whitney test. #P value was calculated with the χ2 test. P value < 0.05 was considered significant. SCD: sickle cell disease; SCT: sickle cell trait; SD: standard deviation; CBC: complete blood cell count; WBC: white blood cell; RBC: red blood cell; Hb: hemoglobin; MCV: mean corpuscular volume; MCH: mean corpuscular hemoglobin; MCHC: mean corpuscular hemoglobin concentration; Hct: hematocrit; MI: Mentzer index; RDW: red cell distribution width; RDWI: red cell distribution width index; GK: Green and King index; EF: England and Fraser index; SL: Shine and Lal index; SI: Srivastava index; RI: Ricerca index; EI: Ehsani index; Sir I: Sirdah index; HH: Huber-Herklotz index. | ||||||
Age (years) | 0.796 | 0.041 | ||||
Mean ± SD | 26.35 ± 9. 85 | 25.93 ± 11.77 | 25.96 ± 10.31 | 30.71 ± 9.73 | ||
Median | 26 | 27 | 22 | 29 | ||
Range | 18 - 52 | 18 - 45 | 18 - 48 | 18 - 52 | ||
Gender | 0.193# | |||||
Male | 31 (56.4) | 26 (43.3) | 14 (58.3) | 17 (54.8) | 0.421 | |
Female | 24 (43.6) | 34 (56.7) | 10 (41.7) | 14 (45.2) | ||
Residence | 0.074# | 0.863# | ||||
Assiut | 18 (32.7) | 21 (35) | 9 (37.5) | 9 (41.9) | ||
El Minia | 1 (1.8) | 4 (6.7) | 1 (4.2) | 0 (0.0) | ||
Sohag | 12 (21.8) | 27 (45) | 6 (25) | 6 (35.5) | ||
Qena | 8 (14.5) | 5 (8.3) | 1 (4.2) | 7 (0.0) | ||
Luxor | 2 (3.6) | 0 (0.0) | 1 (4.2) | 1 (3.2) | ||
Aswan | 3 (5.5) | 1 (1.7) | 1 (4.1) | 2 (3.2) | ||
Red Sea | 5 (9.1) | 2 (3.3) | 2 (8.3) | 3 (6.5) | ||
New Valley | 6 (10.9) | 0 (0.0) | 3 (12.5) | 3 (3.2) | ||
Clinical presentation | 0.000# | |||||
Acute hemolysis | 11 (20) | 9 (37.5) | 2 (6.4) | |||
Painful crisis | 9 (16.3) | 8 (33.3) | 1 (3.2) | |||
Acute abdomen | 4 (7.3) | 4 (16.3) | 0 (0) | |||
Anemic manifestations | 19 (34.5) | 6 (25) | 1 (3.2) | |||
Jaundice | 4 (7.2) | 3 (12.5) | 1 (3.2) | |||
Acute chest syndrome | 2 (3.6) | 2 (8.3) | 0 (0) | |||
Obstructive jaundice | 7 (12.7) | 6 (25) | 1 (3.2) | |||
Accidental discovery | 15 (27.2) | 0 (0.0) | 25 (80.6) | |||
Nephropathy | 3 (5.4) | 2 (8.3) | 1 (3.2) | |||
Aplastic crisis | 2 (3.6) | 2 (8.3) | 0 (0) | |||
CBC | ||||||
WBCs (× 103/L) | 7.72 ± 4.34 | 7.33 ± 1.75 | 0.163 | 8.83 ± 5.30 | 6.86 ± 3.25 | 0.502 |
RBCs (× 106/L) | 5.01 ± 4.93 | 5.92 ± 0.53 | < 0.001 | 3.78 ± 1.31 | 5.96 ± 6.34 | 0.050 |
Hb (g/dL) | 10.65 ± 3.31 | 14.22 ± 1.54 | < 0.001 | 9.10 ± 2.20 | 11.85 ± 3.55 | < 0.001 |
MCV (fL) | 74.67 ± 17.79 | 85.72 ± 4.32 | < 0.001 | 76.48 ± 9.86 | 73.27 ± 22.14 | 0.830 |
MCH (pg) | 25.10 ± 5.43 | 28.27 ± 1.20 | < 0.001 | 26.31 ± 4.59 | 24.17 ± 5.91 | 0.391 |
MCHC (g/dL) | 36.21 ± 10.61 | 32.59 ± 1.26 | 0.518 | 31.29 ± 2.14 | 40.02 ± 12.84 | < 0.001 |
Hct (%) | 35.42 ± 11.77 | 43.82 ± 3.03 | < 0.001 | 27.23 ± 3.84 | 41.76 ± 11.96 | < 0.001 |
Reticulocytes (%) | 3.74 ± 2.54 | 0.9 ± 0.37 | < 0.001 | 4.72 ± 3.12 | 2.97 ± 1.67 | 0.045 |
Platelets (× 103/L) | 300.67 ± 93.84 | 316.73 ± 60.11 | 0.149 | 316.13 ± 82.53 | 300.67 ± 93.84 | 0.247 |
Targeted indices | ||||||
MI | 20.56 ± 11.14 | 16.37 ± 2.03 | 0.046 | 23.37 ± 10.91 | 18.09 ± 3.11 | 0.086 |
RDW (%) | 17.01 ± 3.86 | 13.26 ± 0.80 | < 0.001 | 17.01 ± 3.86 | 16.19 ± 4.22 | 0.030 |
RDWI | 353.43 ± 24.80 | 216.71 ± 26.56 | < 0.001 | 424.99 ± 201.77 | 298.03 ± 229.09 | 0.040 |
GK | 108.37 ± 63.09 | 69.19 ± 8.72 | 0.260 | 127 ± 55.91 | 93.20 ± 6.49 | 0.009 |
EF | 15.5 ± 22.30 | 5.94 ± 8.26 | 0.076 | 23.79 ± 11.9 | 9.13 ± 22.67 | 0.008 |
SL | 1,569.90 ± 656.78 | 2,089.21 ± 280.90 | < 0.001 | 1,608.6 ± 638.10 | 1,539.8 ± 679.80 | 0.773 |
SI | 6.90 ± 3.54 | 5.39 ± 0.60 | 0.023 | 8.03 ± 3.94 | 6.01 ± 2.96 | 0.017 |
RI | 4.81 ± 2.55 | 2.52 ± 0.28 | < 0.001 | 5.43 ± 2.25 | 4.32 ± 2.68 | 0.018 |
EI | 36.06 ± 19.90 | 32.77 ± 7.34 | 0.799 | 38.60 ± 20.46 | 34.09 ± 19.56 | 0.164 |
Sir I | 40.59 ± 17.11 | 37.78 ± 5.87 | 1.000 | 45.39 ± 15.45 | 36.88 ± 17.64 | 0.018 |
HH | 28.90 ± 9.29 | 20.40 ± 1.26 | < 0.001 | 32.66 ± 8.64 | 26.00 ± 8.85 | 0.003 |
Participant group | Mean | SD | P value | |
---|---|---|---|---|
P value was calculated with unpaired sample t-test. SCT: sickle cell trait; SD: standard deviation; WBC: white blood cell; RBCs: red blood cells; Hb: hemoglobin; MCV: mean corpuscular volume; MCH: mean corpuscular hemoglobin; MCHC: mean corpuscular hemoglobin concentration; Hct: hematocrit; MI: Mentzer index; RDW: red cell distribution width; RDWI: red cell distribution width index; GK: Green and King index; EF: England and Fraser index; SL: Shine and Lal index; SI: Srivastava index; RI: Ricerca index; EI: Ehsani index; Sir I: Sirdah index; HH: Huber-Herklotz index. | ||||
WBCs (× 103/L) | Controls | 7.329 | 1.7476 | 0.138 |
SCT | 7.048 | 2.9781 | ||
RBCs (× 106/L) | Controls | 5.296 | 0.5288 | < 0.001 |
SCT | 4.368 | 1.2013 | ||
Hb (g/dL) | Controls | 14.218 | 1.5449 | 0.007 |
SCT | 12.174 | 3.0627 | ||
MCV (fL) | Controls | 85.732 | 4.3223 | < 0.001 |
SCT | 77.774 | 11.6808 | ||
MCH (pg) | Controls | 28.273 | 1.2038 | < 0.001 |
SCT | 24.168 | 5.9053 | ||
MCHC (g/dL) | Controls | 32.592 | 1.2607 | 0.008 |
SCT | 40.023 | 12.8395 | ||
Platelets (× 103/L) | Controls | 316.73 | 60.106 | 0.027 |
SCT | 288.71 | 101.439 | ||
Hct (%) | Controls | 43.82 | 3.037 | 0.805 |
SCT | 40.44 | 9.655 | ||
Reticulocytes (%) | Controls | 0.873 | 0.3654 | < 0.001 |
SCT | 2.974 | 1.6733 | ||
Mentzer index | Controls | 16.3668 | 2.02790 | 0.779 |
SCT | 20.3172 | 10.53331 | ||
RDW index | Controls | 216.71 | 26.55909 | 0.49 |
SCT | 345.52 | 246.46697 | ||
RDW | Controls | 13.2650 | 0.80398 | 0.002 |
SCT | 16.1903 | 4.22164 | ||
GK | Controls | 69.1987 | 8.71872 | 0.514 |
SCT | 93.2031 | 64.98447 | ||
EF | Controls | 5.9440 | 8.26077 | 0.233 |
SCT | 9.1348 | 22.67161 | ||
SL | Controls | 2,089.21 | 280.97225 | < 0.001 |
SCT | 1,539.93 | 679.80101 | ||
SI | Controls | 5.3922 | 0.60189 | 0.763 |
SCT | 6.0194 | 2.96136 | ||
RI | Controls | 2.5274 | 0.27603 | < 0.001 |
SCT | 4.3260 | 2.68780 | ||
EI | Controls | 32.7717 | 7.34526 | 0.293 |
SCT | 34.0903 | 19.56032 | ||
Sir I | Controls | 37.7807 | 5.87379 | 0.016 |
SCT | 36.8832 | 17.64508 | ||
HH | Controls | 20.4038 | 1.25761 | 0.001 |
SCT | 26.0023 | 8.85158 |
Hb | Groups | Mean | Std. deviation | Std. error mean | P-value |
---|---|---|---|---|---|
Hb: hemoglobin; SCT: sickle cell trait. | |||||
Hb A | SCT | 62.45 | 11.45 | 2.0570 | 0.000 |
Controls | 96.770 | 0.5097 | 0.0658 | ||
Hb A2 | SCT | 3.37 | 0.47 | 0.9562 | 0.009 |
Controls | 2.623 | 0.2619 | 0.0338 | ||
Hb F | SCT | 1.400 | 2.35 | 0.4229 | 0.014 |
Controls | 0.61 | 0.51 | 0.0650 | ||
Hb S | SCT | 34.78 | 10.59 | 1.9412 | 0.000 |
Controls | 0.000 | 0.0000 | 0.0000 |
Variable | AUC (95%) | Cut-off | Sens (%) | Spec (%) | PPV (%) | NPV (%) | % Correctly identified | YI | P value |
---|---|---|---|---|---|---|---|---|---|
AUC: area under the curve; Sens: sensitivity; Spec: specificity; PPV: positive-predictive value; NPV: negative-predictive value; YI: Youden’s index; SC: sickle cell; SCT: sickle cell trait; RBCs: red blood cell; Hb: hemoglobin; MCHC: mean corpuscular hemoglobin concentration; Hct: hematocrit; RDW: red blood cell distribution width; RDWI: red blood cell distribution width index; GK: Green and King index; EF: England and Frazer index; SL: Shine and Lal index; SI: Srivastava index; RI: Ricerca index; EI: Ehsani index; Sir I: Sirdah index; HH: Huber-Herklotz index. | |||||||||
MCHC | 0.67 (0.52 - 0.81) | SCT > 33.5 | 61% | 81% | 63% | 80% | 75% | 0.42 | 0.009 |
Retics (%) | 0.98 (0.96 - 1.02) | SCT > 2% | 70% | 100% | 100% | 87% | 66% | 0.70 | < 0.001 |
RDW (%) | 0.70 (0.56 - 0.83) | SCT > 14% | 54% | 85% | 65% | 78% | 74% | 0.39 | 0.002 |
RDWI | 0.63 (0.49 - 0.76) | SCT > 240% | 54% | 80% | 59% | 77% | 71% | 0.29 | 0.049 |
SL | 0.82 (0.70 - 0.93) | SCT < 1,780 | 88% | 71% | 58% | 76% | 82% | 0.59 | < 0.001 |
RI | 0.79 (0.68 - 0.90) | SCT > 2.8 | 68% | 83% | 68% | 83% | 78% | 0.51 | < 0.001 |
Sir I | 0.35 (0.19 - 0.50) | SCT > 35 | 26% | 52% | 22% | 58% | 43% | -0.22 | 0.016 |
HH | 0.71 (0.58 - 0.84) | SCT > 21.5 | 55% | 78% | 56% | 77% | 70% | 0.33 | 0.001 |