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2‐Chlorodeoxyadenosine treatment after splenectomy in patients who have myelofibrosis with myeloid metaplasia
Author(s) -
Tefferi Ayalew,
Silverstein Murray N.,
Li ChinYang
Publication year - 1997
Publication title -
british journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.907
H-Index - 186
eISSN - 1365-2141
pISSN - 0007-1048
DOI - 10.1046/j.1365-2141.1997.3843198.x
Subject(s) - myelofibrosis , splenectomy , medicine , metaplasia , myeloid , gastroenterology , immunology , spleen , bone marrow
2‐Chlorodeoxyadenosine (2‐CdA) is a purine nucleoside analogue with therapeutic activity in low‐grade lymphoproliferative disorders. In addition, 2‐CdA has a potent myelosuppressive effect, and it has been shown to be toxic to malignant myeloid cells both in vitro and in vivo . In this pilot study we treated nine patients who had advanced myelofibrosis with myeloid metaplasia (MMM) and progressive hepatomegaly or symptomatic thrombocytosis after therapeutic splenectomy. 2‐CdA was administered at 0.05–0.1 mg/kg/d for 7 d for one to five treatment cycles. A reduction in liver size associated with marked improvement in fatigue and control of thrombocytosis and leucocytosis was achieved in seven of the nine patients (78% response rate). In four of the seven responding patients the reduction in liver size was durable (4–28 months) and was associated with a decrease in serum alkaline phosphatase levels. However, no patient had improvement in anaemia, and two of the seven initially responding patients have since died of acute leukaemia or progressive disease. Improvement in bone marrow fibrosis was noted in two of five available post‐treatment marrow examinations. Toxicity was mainly myelosuppression, which was severe in two patients. 2‐CdA may be considered a palliative therapeutic agent after splenectomy in noncytopenic patients with MMM who have progressive hepatomegaly or extreme thrombocytosis.