Bone marrow transplantation for familial hemophagocytic lymphohistiocytosis
Ahmed, T.; Mehta, R.; Patel, P.; Kaul, R.; Kaul, A.
Anticancer Research 9(6): 1567-1568
1989
ISSN/ISBN: 0250-7005 PMID: 2627111 Document Number: 331762
A one year old child diagnosed at two months as having familial hemophagocytic lymphohistiocytosis had a partial response to etoposide. He relapsed and underwent a bone marrow transplant after conditioning with etoposide 65 mg/kg, total body irradiation and cyclophosphamide 60 mg/kg X 2 doses. Post transplant sepsis ensued and the patient expired. At autopsy residual disease was noted. Familial hemophagocytic lymphohistiocytosis continues to be a fatal disorder and etoposide is the only agent known so far with any efficacy. Bone marrow transplantation probably should be attempted early in its course and may have a role in the treatment of this disease.