Cases reported "Anemia, Diamond-Blackfan"

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1/2. Central venous catheter thrombosis as a cause of SVC obstruction and cardiac tamponade in a patient with diamond-Blackfan anemia and iron overload.

    cardiac tamponade is an infrequent but potentially lethal complication related to use of central venous catheters (CVC). We present the case of a 16-year-old female with diamond-Blackfan anemia (DBA) who developed pericardial tamponade secondary to superior venous caval obstruction caused by CVC thrombosis. The patient presented 3 months after line placement with vomiting, abdominal pain, and cardiomegaly on chest X-ray (CXR). Her condition quickly decompensated with cardiac arrest and subsequent death despite immediate pericardiocentesis. As a result of this case, our center has developed a protocol for the management of CVC problems as a means of facilitating rapid recognition of central line clots.
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ranking = 1
keywords = iron overload, overload, iron
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2/2. Successful treatment of diamond-Blackfan anemia with metoclopramide.

    diamond-Blackfan anemia (DBA) is a congenital anemia characterized by a low reticulocyte count, the absence or severe reduction of hemoglobin-containing cells in the bone marrow, and normal megakaryocytic and granulocytic differentiation. Although the anemia may initially respond to corticosteroid therapy, many patients require lifelong red blood cell (RBC) transfusion, leading to infectious complications and iron overload. metoclopramide has recently been used to treat DBA. Treatment with metoclopramide induces the release of prolactin from the pituitary and stimulates erythropoiesis. For these reasons, we used metoclopramide to treat a 20-year-old man with DBA refractory to low and high doses of corticosteroids, cyclosporin A, and tacrolimus (FK506). The hemoglobin and hematocrit slowly increased, and he has remained asymptomatic and transfusion-independent for 8 months. metoclopramide therapy should be considered in patients with refractory DBA before treatment-related complications develop.
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ranking = 0.25
keywords = iron overload, overload, iron
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