ABSTRACT
Paroxysmal nocturnal hemoglobinura is a very rare acquired clonal hematopoietic cell disorder leading to chronic intravascular hemolysis caused by abnormal complement activation. Pregnancy in a patient with paroxysmal nocturnal hemoglobinuria is often complicated with thrombosis resulting serious materno-fetal morbidity and mortality. A case of successful maintenance of the pregnancy, delivery and postpartum in a woman at gestational age of 37 weeks and 2 days with paroxysmal nocturnal hemoglobinuria managed with prophylactic transfusions and anticoagulation therapy by low molecular weight heparin is presented with a review of literature.
Subject(s)
Female , Humans , Pregnancy , Complement Activation , Gestational Age , Hemoglobinuria, Paroxysmal , Hemolysis , Heparin, Low-Molecular-Weight , Mortality , Postpartum Period , ThrombosisABSTRACT
Hypoglycemia due to non-islet cell tumor is usually associated with hypersecretion of big insulin-like growth factor II (IGF-II). This big IGF-II cannot form ternary IGF complex, and is biologically more active in peripheral tissue, inducing increased glucose utilization and hypoglycemia. A 57-year-old man developed severe hypoglycemia due to hepatocellular carcinoma. To control hypoglycemia, the patient required continuous glucose infusion. The circulating levels of cortisol and free T4 were in the normal range. The plasma levels of insulin, C-peptide, IGF-I, IGF binding protein-3 (IGFBP-3), and total IGF-II levels were decreased. Radioimmunoassay of IGF-II revealed that big IGF-II immunoreactivity markedly increased compared to that of normal control. In this patient, it was strongly suggested that big IGF-II might be a cause of severe intractable hypoglycemia.