"Irony" of managing refractory anemia with transfusional support in hemophagocytic lymphohistiocytosis.
Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening derangement of the immune system in which host macrophages phagocytose the patient's own blood cells. Herein, we present the case of a patient with HLH and associated refractory anemia who developed rapid iron deposition in the liver after transfusion of sixteen units of packed red blood cells (RBCs). Before transfusion, neither a liver biopsy nor computed tomography scan demonstrated iron deposition in the organ parenchyma. After receiving sixteen units of packed RBCs, liver iron concentration rose to 6.7 mg/g dry weight, which is highly unusual in other diseases requiring transfusional support.
Department of Medicine, Elmhurst Hospital Center, Icahn School of Medicine at Mount Sinai, Elmhurst, New York 11373, USA.
Sunyoung
S
Lee
Department of Medicine, Elmhurst Hospital Center, Icahn School of Medicine at Mount Sinai, Elmhurst, New York 11373, USA.
Ilnaz
I
Salehi
Icahn School of Medicine at Mount Sinai, New York, New York 10029, USA.
Michael
M
Chary
Division of Liver Diseases, Department of Medicine, Icahn School of Medicine at Mount Sinai, New York, New York 10029, USA.
Thomas
T
Schiano
Tisch Cancer Institute, Division of Hematology and Oncology, Icahn School of Medicine at Mount Sinai, New York, New York 10029, USA. Electronic address: john.mascarenhas@mssm.edu.
John O
JO
Mascarenhas
D000328:Adult; D000753:Anemia, Refractory / Q000000981:diagnostic imaging / Q000378:metabolism / Q000628:therapy*; D017707:Erythrocyte Transfusion / Q000009:adverse effects*; D005260:Female; D006801:Humans; D019190:Iron Overload / Q000000981:diagnostic imaging / Q000209:etiology*; D051359:Lymphohistiocytosis, Hemophagocytic / Q000000981:diagnostic imaging / Q000378:metabolism / Q000628:therapy*
Hemophagocytic lymphohistiocytosis; Iron overload; Liver iron concentration; Transferrin saturation