[Hemophagocytic syndrome].
A lymph node biopsy sample from a boy aged with fever, pneumonia, hepatosplenomegaly, lymphadenopathy and pancytopenia, showed histiocytosis with erythrophagocytosis, compatible with the haemophagocytic syndrome. Treatment consisted of dexamethasone and etoposide, with cyclosporine added in a later phase. During the subsequent remission phase, bone marrow transplantation was carried out. Haemophagocytic syndrome is a rare condition, characterized by fever, pancytopenia, hepatosplenomegaly and characteristic laboratory findings (including a high interferon-gamma level) and morbid-anatomical findings (haemophagocytic histiocytic cells in bone marrow, lymph nodes, liver and spleen, but also in the CNS, kidneys and lungs). Recent pathophysiological discoveries indicate an enhanced T-cell response, leading to hypercytokinaemia. As a rule the patient dies from multiorgan failure and diffuse intravascular coagulation. Bone marrow transplantation is the treatment of choice.
van der Deure
J
J
Afd. Kindergeneeskunde, Academisch Ziekenhuis Vrije Universiteit. Amsterdam.
Kardos
G
G
von Blomberg-van der Flier
B M
BM
D000893:Anti-Inflammatory Agents / Q000008:administration & dosage; D000972:Antineoplastic Agents, Phytogenic / Q000008:administration & dosage; D001772:Blood Cell Count; D016026:Bone Marrow Transplantation; D002644:Chickenpox / Q000150:complications; D002675:Child, Preschool; D003131:Combined Modality Therapy; D016572:Cyclosporine / Q000008:administration & dosage; D003907:Dexamethasone / Q000008:administration & dosage; D004359:Drug Therapy, Combination; D005047:Etoposide / Q000008:administration & dosage; D015616:Histiocytosis, Non-Langerhans-Cell / Q000097:blood* / Q000150:complications / Q000628:therapy; D006801:Humans; D007166:Immunosuppressive Agents; D008297:Male; D009102:Multiple Organ Failure / Q000209:etiology; D016896:Treatment Outcome
D000893:Anti-Inflammatory Agents; D000972:Antineoplastic Agents, Phytogenic; D007166:Immunosuppressive Agents; D005047:Etoposide; D003907:Dexamethasone; D016572:Cyclosporine