[Hemophagocytic syndrome]
| Autor: | J, van der Deure, G, Kardos, B M, von Blomberg-van der Flier |
|---|---|
| Jazyk: | Dutch; Flemish |
| Rok vydání: | 1998 |
| Předmět: |
Male
Histiocytosis Non-Langerhans-Cell Multiple Organ Failure Anti-Inflammatory Agents Antineoplastic Agents Phytogenic Combined Modality Therapy Dexamethasone Blood Cell Count Chickenpox Treatment Outcome Child Preschool Cyclosporine Humans Drug Therapy Combination Immunosuppressive Agents Bone Marrow Transplantation Etoposide |
| Zdroj: | Nederlands tijdschrift voor geneeskunde. 141(41) |
| ISSN: | 0028-2162 |
| Popis: | 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. |
| Databáze: | OpenAIRE |
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