[Natural history of paroxysmal nocturnal hemoglobinuria in adolescents,adults, and children: the Mexican experience].
OBJECTIVE
To compare the clinical patterns and survival of young and adult (AP) versus paediatric (PP) patients with paroxysmal nocturnal haemoglobinuria (PNH).


METHODS
The clinical records of 117 patients (82% AP, 18% PP) seen in four cities of the Mexican Republic were analysed, the clinical course and survival of both groups being compared.


RESULTS
No sex difference was found in the two patient-groups: 51% and 52% males, 49% and 48% females in AP and PP, respectively. The onset of PNH had similar distribution for the two groups of patients: aplastic form, 45% in AP and 62% in PP; cytopenias, 24% in AP versus 27% in PP; haemolysis, 28% in AP and 9% in PP, and thrombosis, 3% in AP versus 0% in PP. The clinical features with significant difference were: anaemic+haemorrhagic syndrome (39 AP (40%) vs 14 PP (67%), p = 0.02), initial diagnosis of immunologic thrombocytopenic purpura (7 AP (7%) vs 7 PP (33%), p = 0.003), and death rate (17 AP (18%) vs 8 PP (38%), p = 0.04). The actuarial survival curves showed significant differences between both groups (p = 0.045, Cox-Mantel), with estimated 10-year survival of 81% for AP and 55% for PP, and 15-year survivals of 64% for AP and 55% for PP.


CONCLUSIONS
Seemingly, PNH in paediatric age has poorer prognosis than in adults, which is associated to higher incidence of fatal haemorrhages due to thrombocytopenia.
Góngora-Biachi
R A
RA
Centro de Investigaciones Regionales Dr. Hideyo Noguchi, Universidad Autónoma de Yucatán, Mérida, México. gbiachi@tunku.uady.mx
González-Martínez
P
P
Sosa-Muñoz
J
J
Castro-Sansores
C
C
Delgado-Lamas
J L
JL
Vázquez-Villegas
V
V
Rico-Balzadua
G
G
Herrera-Sánchez
P
P
Selva-Pallares
J
J
González-Llaven
J
J
D000208:Acute Disease; D000293:Adolescent; D000328:Adult; D000740:Anemia / Q000209:etiology; D000754:Anemia, Refractory, with Excess of Blasts / Q000209:etiology; D001853:Bone Marrow / Q000473:pathology; D002648:Child; D002675:Child, Preschool; D003937:Diagnosis, Differential; D005260:Female; D006457:Hemoglobinuria, Paroxysmal / Q000145:classification / Q000150:complications / Q000453:epidemiology* / Q000473:pathology; D006470:Hemorrhage / Q000209:etiology; D006801:Humans; D015994:Incidence; D007223:Infant; D007951:Leukemia, Myeloid / Q000209:etiology; D015993:Life Tables; D008297:Male; D008800:Mexico / Q000453:epidemiology; D011379:Prognosis; D016553:Purpura, Thrombocytopenic, Idiopathic / Q000175:diagnosis; D012189:Retrospective Studies; D016019:Survival Analysis; D013927:Thrombosis / Q000209:etiology