Table 1 Clinical and laboratory characteristics of PNH patients at the time of BM examination.

ID	Age (years) /Sex	Diagnosis	Prior IST	Current IST	Time from diagnosis (months)	AA status	Complement inhibitors	RBC transfusion dependance	GPI-AP(−) granulocytes (%)	GPI-AP(−) monocytes (%)	GPI-AP(−) RBC (%)	WBC (4.63 - 6.08 ×10³/µL)	ANC (1.78 - 5.38 ×10³/µL)	Hb (13.7 - 17.5 g/dL)	Reti (26 - 95 ×10³/µL)	Platelets (161 - 347 ×10³/µL)	LDH (125 - 220 U/L)
PNH 3	43/M	AA/PNH	hATG, CsA, EPAG	none	84	CR	none	no	15.99	13.53	type II 0.09, type Ⅲ 5.48	4.54	2.54	14.7	72.8	234	203
PNH 4	60/F	AA/PNH	hATG, CsA	none	83	CR	Ravulizmab, Danicopan	no	93.76	93.44	type II 1.70, type III 88.94	3.21	1.57	12.2	94.1	147	214
PNH 5	67/F	AA/PNH	rATG, CsA, Alemtuzumab	none	175	CR	none	no	22.69	15.51	type II 0.16, type III 4.35	4.47	2.16	13	66.1	217	329
PNH 6	36/F	AA/PNH	hATG, CsA, EPAG	CsA	6, 12	PR	none	no	30.54	24.84	type II 0.57, type III 7.88	5.88	4.86	10	102.3	61	561
PNH 7	21/F	AA/PNH	hATG, CsA, EPAG	none	52	CR	none	no	99.15	93.5	type II 3.78, type III 90.69	5.14	2.84	14.3	47.2	132	234
PNH 8	58/F	AA/PNH	CsA	CsA	20	PR	Ravulizumab	yes	41.48	60.66	type II 1.40, type III 13.71	3.99	2.63	7.8	141.3	93	312
PNH 9	29/F	Classical PNH	none	none	N/A	N/A	Ravulizumab	no	91.0	N/A	N/A	4.21	2.44	8.8	191.2	74	259
PNH 10	67/M	AA/PNH	hATG, CsA, EPAG	CsA	129	PR	Ravulizumab	no	45.62	72.06	type II 0.36, type III 6.06	2.25	2.14	9.3	48.1	84	236

AA aplastic anemia, ANC absolute neutrophil count, BM bone marrow, CsA cyclosporine, CR complete response, EPAG eltrombopag, F female, GPI-AP glycosylphosphatidylinositol anchored protein, hATG horse anti-thymogloblin, Hb hemoglobin, IST immunosuppressive therapy, LDH Lactate Dehydrogenase, M male, N/A not available, rATG rabbit anti-thymogloblin, RBC red blood cells, Reti reticulocytes, PNH paroxysmal nocturnal hemoglobinuria, PR partial response, WBC white blood cells.

Quick links

Search