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. 2015 Sep 1;9(9):OD09–OD10. doi: 10.7860/JCDR/2015/12878.6548

[Table/Fig-3]:

Haematological and molecular characterization of HPFH-3 cases

Parameters IB IIA IIB IIIA IIIB IIIC IIID IIIE IIIF
Age (in years) 73 50 43 26 24 22 18 15 12
Relationship GM F M D1 D2 D3 D4 D5 S1
Haemoglobin fraction by HPLC
HbA0 (%) 65.9 67.4 87.9 88.9 88.3 61.3 65 86.9 61.7
HbA2 (%) 2.0 2.3 3.1 2.7 2.8 2.1 2.2 2.7 2.3
HbF (%) 25.2 21.8 0.4 0.4 0.3 30.5 25.4 1.0 27.7
Red cell indices
WBC (103/μL) 7.6 5.6 4.2 6.5 8.1 7.9 7.8 7.1 6.8
RBC (106/μL) 4.69 6.10 3.33 4.19 3.33 5.55 4.92 5.72 5.64
Haemoglobin (g/dL) 9.8 15 11.8 12.5 13.7 14.1 12.9 12.2 13.3
HCT (%) 35.1 48.3 32 37.9 29 43.7 38.5 40.3 41.4
Platelets (103/μL) 172 266 236 249 222 278 168 283 183
MCV (fL) 79.8 79.2 70.2 90.5 72.6 78.7 78 70 76.4
MCH (pg) 26.2 24.6 22.2 30.1 23.6 25.4 26.1 21.3 23.6
MCHC (g/dL) 31.4 31.1 31.6 33.2 28.9 32.3 33.4 30.2 32.1
Other Investigations
Reticulocyte count (%) 0.34 0.86 0.74 0.54 0.48 0.84 0.72 1.03 0.76
Distribution of HbF Pan Pan - - - Pan Pan - Pan
Alpha thalassemia αα/αα αα/αα -3.7α/αα αα/αα -3.7α/αα αα/αα αα/αα -3.7α/αα αα/αα
HPFH-3 A/HPFH A/HPFH A/A A/A A/A A/HPFH A/HPFH A/A A/HPFH

*WBC, white blood cells; RBC, Red blood cells; HCT, Haematocrit; MCV, mean corpuscular volume; MCH, mean corpuscular Hb; MCHC, mean corpuscular Hb concentration; Pan–pancellular; GM, Grandmother; F, father; M, mother, D, daughter; S, Son. HPFH, High persistence of foetal haemoglobin