Clinical and hematologic features of PNH patients
. | . | . | . | . | . | . | Size of PNH population, %* . | . | Absolute counts . | . | . | . | . | . | ||
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| Patient . | Sex/age . | Years from diagnosis . | Hb, g/L . | PMNs, × 109/L . | Plts, × 109/L . | Retics, % . | RBCs . | PMNs . | GPI+ PMNs, × 109/L . | GPI- PMNs, × 109/L . | Hemoglobinuria . | Thrombosis . | Treatment . | Transfusions . | ||
| A | F/24 | 6 | 101 | 2.3 | 130 | 4.5 | 39 | 80 | 0.46 | 1.84 | Yes | No | ATG | Occasional | ||
| B | F/27 | 7 | 108 | 4.2 | 115 | 3.9 | 30 | 90 | 0.42 | 3.78 | Yes | No | ATG | Occasional | ||
| C | M/40 | 2 | 118 | 3.2 | 137 | 3.4 | 13 | 32 | 2.18 | 1.02 | Yes | Cerebral | Supportive | Occasional | ||
| D | M/30 | 3 | 90 | 2.6 | 180 | 14.9 | 43 | 94 | 0.16 | 2.44 | Yes | Splenic | ATG | Occasional | ||
| E | F/37 | 7 | 82 | 1.9 | 115 | 8.2 | 12 | 59 | 0.78 | 1.12 | Yes | No | Supportive | Never | ||
| F | F/24 | 5 | 109 | 3.1 | 103 | 3.0 | 60 | 80 | 0.62 | 2.48 | Yes | Mesenteric | ATG | Occasional | ||
| G | F/34 | 11 | 124 | 2.3 | 74 | 2.1 | 23 | 40 | 1.38 | 0.92 | Yes | Budd-Chiari | Supportive | Never | ||
| H | F/45 | 7 | 97 | 1.3 | 160 | 5.2 | 18 | 77 | 0.30 | 1.00 | Yes | Pulmonar | Supportive | Occasional | ||
| I | F/28 | 10 | 106 | 8.4 | 62 | 5.4 | 72 | 96 | 0.34 | 8.06 | Yes | Budd-Chiari | Supportive | Never | ||
| J | F/60 | 9 | 70 | 3.0 | 229 | ND | 46 | 78 | 0.66 | 2.34 | Yes | No | Supportive | Occasional | ||
. | . | . | . | . | . | . | Size of PNH population, %* . | . | Absolute counts . | . | . | . | . | . | ||
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| Patient . | Sex/age . | Years from diagnosis . | Hb, g/L . | PMNs, × 109/L . | Plts, × 109/L . | Retics, % . | RBCs . | PMNs . | GPI+ PMNs, × 109/L . | GPI- PMNs, × 109/L . | Hemoglobinuria . | Thrombosis . | Treatment . | Transfusions . | ||
| A | F/24 | 6 | 101 | 2.3 | 130 | 4.5 | 39 | 80 | 0.46 | 1.84 | Yes | No | ATG | Occasional | ||
| B | F/27 | 7 | 108 | 4.2 | 115 | 3.9 | 30 | 90 | 0.42 | 3.78 | Yes | No | ATG | Occasional | ||
| C | M/40 | 2 | 118 | 3.2 | 137 | 3.4 | 13 | 32 | 2.18 | 1.02 | Yes | Cerebral | Supportive | Occasional | ||
| D | M/30 | 3 | 90 | 2.6 | 180 | 14.9 | 43 | 94 | 0.16 | 2.44 | Yes | Splenic | ATG | Occasional | ||
| E | F/37 | 7 | 82 | 1.9 | 115 | 8.2 | 12 | 59 | 0.78 | 1.12 | Yes | No | Supportive | Never | ||
| F | F/24 | 5 | 109 | 3.1 | 103 | 3.0 | 60 | 80 | 0.62 | 2.48 | Yes | Mesenteric | ATG | Occasional | ||
| G | F/34 | 11 | 124 | 2.3 | 74 | 2.1 | 23 | 40 | 1.38 | 0.92 | Yes | Budd-Chiari | Supportive | Never | ||
| H | F/45 | 7 | 97 | 1.3 | 160 | 5.2 | 18 | 77 | 0.30 | 1.00 | Yes | Pulmonar | Supportive | Occasional | ||
| I | F/28 | 10 | 106 | 8.4 | 62 | 5.4 | 72 | 96 | 0.34 | 8.06 | Yes | Budd-Chiari | Supportive | Never | ||
| J | F/60 | 9 | 70 | 3.0 | 229 | ND | 46 | 78 | 0.66 | 2.34 | Yes | No | Supportive | Occasional | ||
Hb indicates hemoglobin level; PMNs, granulocytes; Plts, platelets; Retics, reticulocytes; RBCs, red blood cells; Yes, recurrent episodes of macroscopic hemoglobinuria throughout several years of clinical history; F, female; M, male; ATG, antithymocyte globulin; and ND, not done.
The percentage of PNH PMNs reflects the relative size of the PNH cell population more accurately than the percentage of PNH RBCs because the latter will be grossly underestimated as a consequence of selective hemolysis; this underestimation may be further complicated by blood transfusion.