Acute lupus hemophagocytic syndrome: report of a case.
Hemophagocytic Syndrome is a clinical condition characterized by the activation of either macrophages or histiocytes with a prominent hemophagocytosis feature in the bone marrow and other reticuloendothelial systems. It leads to the phagocytosis of erythrocytes, leukocytes, platelets and their precursors. The presence of hemophagocytosis can be associated to infections, malignancies, autoimmune diseases, drugs and a variety of other medical conditions. We report a case of a previously healthy 36 year-old woman that developed hemophagocytosis at the same time that fulfilled diagnostic criteria for systemic lupus erythematosus. Lupus related hemophagocytic syndrome is a rare and potentially fatal entity. It offers significant differential diagnosis challenges and requires urgent therapeutic intervention. There are only few cases reported in the literature. However, much is still needed in order to better understand its causes, all the immunopathogenic mechanisms, as well as its clinical and therapeutic aspects.
Botelho
C
C
Department of Nephrology, Centro Hospitalar de Coimbra, Coimbra, Portugal. medmigui@hotmail.com
Ferrer
F
F
Francisco
L
L
Maia
P
P
Mendes
T
T
Carreira
A
A
D000208:Acute Disease; D000328:Adult; D001323:Autoantibodies / Q000097:blood; D001853:Bone Marrow / Q000473:pathology; D003520:Cyclophosphamide / Q000627:therapeutic use; D005260:Female; D005293:Ferritins / Q000032:analysis; D006644:Histiocytes / Q000473:pathology; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007668:Kidney / Q000473:pathology; D008180:Lupus Erythematosus, Systemic / Q000097:blood / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy; D008181:Lupus Nephritis / Q000209:etiology / Q000473:pathology; D051359:Lymphohistiocytosis, Hemophagocytic / Q000097:blood / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology*; D050527:Mesangial Cells / Q000473:pathology; D008775:Methylprednisolone / Q000627:therapeutic use
D001323:Autoantibodies; D007166:Immunosuppressive Agents; D003520:Cyclophosphamide; D005293:Ferritins; D008775:Methylprednisolone