Table 1.
Clinical and lab parameters of 18 patients with paroxysmal nocturnal hemoglobinuria (PNH)
| Characteristic | N (%) or median (range) |
|---|---|
| Sex | |
| Male | 13 (72%) |
| Female | 5 (28%) |
| Age at diagnosis (years) | 30.5 (18–52) |
| Disease duration (years) prior to diagnosis | 4 (0–15) |
| Presentation | |
| Classical PNH | 17 (95%) |
| PNH-AA | 1 (5%) |
| Signs and symptoms | |
| Fatigue | 18 (100%) |
| Hemoglobinuria | 13 (72%) |
| Abdominal pain | 6 (33%) |
| Splenomegaly | 4 (22%) |
| Erectile dysfunction, N (%)males | 8 (62%) |
| Infections | 1 (5%) |
| Renal failure | 4 (22%) |
| Thrombosis | 3 (17%) |
| Portal vein thrombosis | 1 (5%) |
| Cerebral venous thrombosis | 2 (11%) |
| Diagnostic test | |
| Size of PNH clone % (FLAER) | 85% (52–99.5) |
| LDH, fold above ULN | |
| Median (range) | 11 (2.18–32.66) |
| Peripheral blood abnormalities | |
| Anemia alone | 11 (61%) |
| Anemia and thrombocytopenia | 2 (11%) |
| Pancytopenia at diagnosis | 3 (17%) |
| Bone marrow cellularity (13 patients) | |
| Hypercellular | 7 (54%) |
| Normocellular | 5 (38%) |
| Hypocellular | 1 (8%) |
PNH paroxysmal nocturnal haemoglobinuria, PNH-AA PNH-aplastic aknemia, LDH lactate dehydrogenase, FLAER fluorescently labeled aerolysin, ULN upper limit of normal