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Sitomegalovirüs (CMV) infeksiyonu tüm dünyada sıkça görülen bir viral infeksiyon olup, gelişmekte olan ülkelerde infeksiyonu geçirme oranı %90'lara çıkmaktadır. İmmünkompetan kişilerde primer CMV infeksiyonu %10 semptomatik olup, nadiren organ spesifik komplikasyonlara neden olur. Biz herditer sferositozlu ve spontan dalak rüptürü olan immünkompetan kritik sepsisli ve kansız ishali olan hastada CMV tedavisine yanıt veren CMV kolitli hastayı bildirdik.

Cytomegalovirus (CMV) infection is a common viral infection worldwide, with a frequency as high as 90% in developing countries. Only 10% of primary CMV infection is symptomatic in immunocompetent patients, and it rarely causes specific complications. We report CMV colitis in an immunocompetent adolescent with hereditary spherocytosis and spontaneous rupture of the spleen, who was critically ill with septicemia and significant non-bloody diarrhoea that responded to specific CMV treatment. [ABSTRACT FROM AUTHOR]

Hereditary spherocytosis (HS) is a relatively common hemolytic anemia; most affected individuals have mild or only moderate haemolysis. It is a heterogeneous group of disorders with regard to clinical severity, protein defects and mode of inheritance. There is usually a family history, and a typical clinical and laboratory picture so that the diagnosis is often easily made without additional laboratory tests. Atypical cases may require measurement of erythrocyte membrane proteins to clarify the nature of the membrane disorder and in the absence of a family history. Mild HS can be managed without folate supplementation and does not require splenectomy. Moderately or severely affected individuals are likely to benefit from splenectomy, which should be performed after the age of 5-6 years. [ABSTRACT FROM AUTHOR]

Hemolytic anemia due to erythrocyte membrane disorder has an important place among hereditary anemia. Hereditary spherocytosis (HS) is one of the most common of them. It is an anemia which is apparent with the spherocyte-shaped erythrocytes and is usually dominantly inherited. Erythrocytes are quickly disintegrated on the periphery because of the membrane defect. Hemolytic, aplastic and megaloblastic crises are its major complications. Human Parvovirus B19 is a virus that can lead to different clinical pictures. It may lead to erythema infection in healthy children and to the aplastic crisis and severe anemia in patients with hemolytic anemia such as hereditary spherocytosis. In this article, two siblings who were diagnosed with hereditary spherocytosis as a result of severe hemolysis induced by the Parvovirus B19 infection are presented. Two brothers, one of them was 4 years old and another one was 5 years old, were brought with jaundice and weakness complaints beginning 3 days after a febril infection period. They had marked pallor, tachycardia, and splenomegaly in the physical examination. The hemoglobin level of one of them was 4.9 gr/dL, and another one's was 2.9 gr/dL. There was a large number of spherocytes in the peripheral smear. Parvovirus B19 Ig M was positive, and their osmotic fragility was increased. It was learned from their history that their father had splenomegaly. The aplastic crisis triggered by the parvovirus B19 infection and anemia due to severe hemolysis may occur as the first symptom of the hematological diseases such as hereditary spherocytosis. Attention should be paid to family history in the early diagnosis for the appropriate follow-up of children with hereditary spherocytosis which is frequently observed in our country, and hereditary spherocytosis should also be considered in the differential diagnosis of jaundice and anemia especially in the neonatal period. In addition, we wanted to remind that parvovirus B19 may lead to serious complications in the presence of hemolytic anemia. [ABSTRACT FROM AUTHOR]