Successful treatment of mu-heavy chain disease with fludarabine monophosphate: a case report.
Heavy chain diseases (HCD) are monoclonal lymphoproliferative disorders of B-cells characterized by the synthesis of truncated heavy chains without associated light chains. In patients with mu-HCD, which is a very rare form of HCD, neoplastic cells produce immunoglobulin M heavy chain. The prognosis for patients with mu-HCD is poor, and there is no specific treatment for mu-HCD. In this report, we present a patient with mu-HCD accompanied by splenomegaly and thrombocytopenia. We treated this patient with the fludarabine monophosphate therapy we use for patients with B-cell chronic lymphocytic leukemia. After 5 courses of fludarabine monophosphate treatment, the splenomegaly and thrombocytopenia improved. Fludarabine monophosphate therapy may be a new strategy to improve the prognosis of patients with mu-HCD.
Department of Hematology and Clinical Immunology, Kobe City General Hospital, Kobe, Japan.
Machi
M
Yanai
Akinori
A
Maeda
Naoko
N
Watanabe
Naoshi
N
Sugimoto
Akiko
A
Matsushita
Kenichi
K
Nagai
Tatsuo
T
Oida
Takayuki
T
Takahashi
D000964:Antimetabolites, Antineoplastic / Q000008:administration & dosage*; D005260:Female; D006362:Heavy Chain Disease / Q000150:complications / Q000000981:diagnostic imaging / Q000188:drug therapy*; D006801:Humans; D008875:Middle Aged; D011859:Radiography; D013163:Splenomegaly / Q000000981:diagnostic imaging / Q000209:etiology; D013921:Thrombocytopenia / Q000209:etiology; D001084:Vidarabine Phosphate / Q000008:administration & dosage* / Q000031:analogs & derivatives*
D000964:Antimetabolites, Antineoplastic; D001084:Vidarabine Phosphate; C042382:fludarabine phosphate