Naslov
Familial Hemophagocytic Lymphohistiocytosis-Therapy with Epipodophyllotoxin

Autori
Krželj, Vjekoslav ; Balarin, Livio ; Rešić, Biserka ; Janković, Stipan ; Čulić, Srđana ; Čulić, Vida

Izvornik
Croatian medical journal (0353-9504) 35 (1994), 4; 229-232

Vrsta, podvrsta i kategorija rada
Radovi u časopisima, članak, stručni

Ključne riječi
epipodophyllotoxin; familial hemophagocytic histiocytosis

Sažetak
To present treatment with epipodophyllotoxin in an infant with rapidly fathal hemophagocytic lymphohistiocytosis. The patient was treated with epipodophyllotoxin(150-250 mg/m2) during one year, combined with corticosteroids, intrathecal methotrexate and cranial irradiation. A signifficant improvement was achieved after the first month of the treatment with epiopodophyllotoxin: Complete remission of the disease lasted for 4-5 months. Signs of relapse appeared during the maintenance therapy. The second intensive treatment approach with epiopodophyllotoxin resulted in good but brief partial remission and patient died on the second birthday with the signs of the disease. Epiopodophyllotoxin seems to be the most effective drug for therapy of rare, malignant and rapidly fatal hemophagocytic lymphohistiocytosis. It should be used as tha first line therapy in the absence of an HLA-identical donor.

Izvorni jezik
Engleski

Znanstvena područja
Kliničke medicinske znanosti

Napomena
Sažetak rada objavljen je u JAMA (Journal of the American Medical Association) : International abstracts (ISSN 0098-7484) 273 (1995) (18) 1470-1470.

POVEZANOST RADA