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Successful treatment of mu-heavy chain disease with fludarabine monophosphate: a case report. 2004

Machi Yanai, and Akinori Maeda, and Naoko Watanabe, and Naoshi Sugimoto, and Akiko Matsushita, and Kenichi Nagai, and Tatsuo Oida, and Takayuki Takahashi
Department of Hematology and Clinical Immunology, Kobe City General Hospital, Kobe, Japan.

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.

UI MeSH Term Description Entries
D008875 Middle Aged An adult aged 45 - 64 years. Middle Age
D011859 Radiography Examination of any part of the body for diagnostic purposes by means of X-RAYS or GAMMA RAYS, recording the image on a sensitized surface (such as photographic film). Radiology, Diagnostic X-Ray,Roentgenography,X-Ray, Diagnostic,Diagnostic X-Ray,Diagnostic X-Ray Radiology,X-Ray Radiology, Diagnostic,Diagnostic X Ray,Diagnostic X Ray Radiology,Diagnostic X-Rays,Radiology, Diagnostic X Ray,X Ray Radiology, Diagnostic,X Ray, Diagnostic,X-Rays, Diagnostic
D005260 Female Females
D006362 Heavy Chain Disease A disorder of immunoglobulin synthesis in which large quantities of abnormal heavy chains are excreted in the urine. The amino acid sequences of the N-(amino-) terminal regions of these chains are normal, but they have a deletion extending from part of the variable domain through the first domain of the constant region, so that they cannot form cross-links to the light chains. The defect arises through faulty coupling of the variable (V) and constant (C) region genes. Franklin Disease,gamma-Chain Disease,mu-Chain Disease,Franklin's Disease,Franklins Disease,Heavy Chain Diseases,gamma Chain Disease,gamma-Chain Diseases,mu Chain Disease,mu-Chain Diseases
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000964 Antimetabolites, Antineoplastic Antimetabolites that are useful in cancer chemotherapy. Antineoplastic Antimetabolites
D001084 Vidarabine Phosphate An adenosine monophosphate analog in which ribose is replaced by an arabinose moiety. It is the monophosphate ester of VIDARABINE with antiviral and possibly antineoplastic properties. Adenine Arabinoside Monophosphate,Ara-AMP,Arabinofuranosyladenine Monophosphate,Vidarabine Monophosphate,9-(5-O-Phosphono-beta-D-arabinofuranosyl)-9H-purin-6-amine,Ara AMP,Monophosphate, Adenine Arabinoside,Monophosphate, Arabinofuranosyladenine,Monophosphate, Vidarabine,Phosphate, Vidarabine
D013163 Splenomegaly Enlargement of the spleen. Enlarged Spleen,Spleen, Enlarged
D013921 Thrombocytopenia A subnormal level of BLOOD PLATELETS. Thrombopenia,Thrombocytopenias,Thrombopenias

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