Cytomegalovirus as a cause of very late interstitial pneumonia after bone marrow transplantation.

Cytomegalovirus (CMV) infection is an important cause of morbidity and mortality after allogeneic transplant. Interstitial pneumonia (IP) is the most common manifestation of CMV in BMT patients, with a 30-48% mortality rate despite adequate treatment. Most CMV infection occurs in the first 100 days. However, prolonged ganciclovir (GCV) prophylaxis has favored the occurrence of late CMV IP, probably by inhibition of the development of CMV-specific T cell lymphocyte responses. We report the case of a patient treated with an allogeneic BMT who received pre-emptive GCV until day +100 because of CMV-positive antigenemia. He developed a CMV IP on day +811 post BMT, which responded to treatment. We intend to alert clinicians that even at long-term (>1 year) post-BMT, CMV is a possible cause of IP in high-risk patients.

[Imerslund-Gräsbeck syndrome: report of two cases] / Síndrome de Imerslund-Gräsbeck: relato de dois casos.

INTRODUCTION: The Imerslund-Gräsbeck syndrome is a rare hereditary autosomal recessive disease, characterized by the onset of megaloblastic anemia and asymptomatic proteinuria during the first 2 years of life. OBJECTIVE: To emphasize the importance of early detection of this disorder, due to high morbidity when not correctly treated, in addition to the necessity of screening and genetic counseling of the asymptomatic family members. METHODS: The authors report two patients, male and female, 8 and 10 years old, respectively. Their past history revealed anemia and multiple blood transfusions since their infancy. They evolved with pancytopenia during childhood and diagnosis of Severe Aplastic Anemia or Fanconi Syndrome was suspected. They were referred to the Bone Marrow Transplantation Section -HC- UFPR. RESULTS: Laboratory investigations revealed pancytopenia in peripheral blood. Bone marrow aspiration showed a marked megaloblastic erythropoiesis. Twenty-four-hour urine collection revealed proteinuria (3.0 and 5.8 g/dl respectively). Cytogenetic analysis was normal. Resolution of symptoms followed replacement therapy with parenteral vitamin B12. CONCLUSIONS: The presence of megaloblastic anemia in children should be followed by investigation of proteinuria, due to the existence of this rare disorder, that has a simple diagnosis and an effective treatment.