RESUMO
Paroxysmal nocturnal hemoglobinuria (PNH) is a clonal nonneoplastic hematopoietic stem cell disease characterized by an acquired mutation of the PIG-A gene with reduction or absence of CD55 and CD59. The absence of these proteins renders PNH erythrocytes susceptible to complement-mediated hemolysis. We report the case of a PNH patient before and during pregnancy until delivery. We observed and treated some postpartum thrombotic complications. Eculizumab should be used with caution in pregnancy. There are several reports supporting its use in these patients. This case should be considered paradigmatic of a series of clinical situations that may occur in the course of a pregnancy in patients with PNH: increased need for transfusion, need to increase the dose of Eculizumab, and insurgence of fetal sufferance. Moreover, after delivery, the patient, despite adequate prophylaxis with low-molecular-weight heparins, presented severe complications: development of pleural and peritoneal effusion, pulmonary embolism, bilateral upper limbs thrombophlebitis, and a possible abdominal angina with a transient paralytic ileus. All these complications were overcome and now the baby is healthy and the mother has returned to the usual therapeutic regimen.
Assuntos
Hemoglobinúria Paroxística/terapia , Complicações Hematológicas na Gravidez/terapia , Adulto , Anticorpos Monoclonais Humanizados/uso terapêutico , Transfusão de Sangue , Feminino , Hemoglobinúria Paroxística/sangue , Hemoglobinúria Paroxística/complicações , Hemoglobinúria Paroxística/genética , Humanos , Recém-Nascido , Período Pós-Parto , Gravidez , Complicações Hematológicas na Gravidez/sangue , Complicações Hematológicas na Gravidez/genética , Trombofilia/epidemiologiaRESUMO
BACKGROUND: Blood donors positive only for anti-HBc may have a resolved hepatitis B virus (HBV) infection, low grade chronic infection or infection with variant strains of HBV. We aimed to assess the significance of this serological pattern after hepatitis B vaccination in such cases. MATERIALS AND METHODS: Twenty-four anti-HBc only blood donors were vaccinated with the Engerix HBV vaccine and a serological and virological evaluation was performed before HBV vaccination and 7-10 days after each dose. Subjects were classified as non-responders if their anti-HBs levels stayed below 10 IU/L after full vaccination, while the response was considered secondary (anamnestic) if anti-HBs levels rose over 10 IU/L after the first vaccine dose, and primary if anti-HBs levels rose over 10 IU/L only after the second or third vaccine dose. RESULTS: Of the 21 fully evaluable donors, six had no response, eight showed a primary response and seven had an anamnestic response. One non-responder had transient positivity for HBV-DNA at low levels (12 IU/mL) with persistent negativity for HBsAg. DISCUSSION: Anti-HBc-only positive blood donors are a heterogeneous population including HBV naïve subjects with a likely false-positive anti-HBc reactivity, subjects with a resolved HBV infection, and subjects with persistent low-level HBV replication. The analysis of the anti-HBs response after a dose of HBV vaccine may help to distinguish among the different causes of the isolated anti-HBc positivity, thereby enabling proper counselling and potential readmission to blood donation.