Successful treatment with cyclosporin A for myelodysplastic syndrome with erythroid hypoplasia associated with T-cell receptor gene rearrangements.
Myelodysplastic syndrome (MDS) with erythroid hypoplasia, a rare form of MDS, has not yet been clearly defined. We report four patients with MDS with erythroid hypoplasia who received immunosuppressive therapy. All were elderly, had severe transfusion-dependent anaemia, morphological evidence of myelodysplasia and a low percentage (3.2-13.6%) of erythroid precursors. Administration of cyclosporin A (CsA) improved their anaemia; all transfusion-dependent patients achieved transfusion-independence. An inverted CD4/8 ratio was seen in three patients who also demonstrated T-cell receptor (TCR)-beta and -gamma gene rearrangements by Southern blotting and clonality by polymerase chain reaction. Treatment with CsA can be an attractive alternative treatment for patients with MDS with erythroid hypoplasia, which may be associated with a clonal abnormality in T cells.
Shimamoto
T
T
First Department of Internal Medicine, Tokyo Medical University, Japan. takashis@cb3.so.net.jp
Iguchi
T
T
Ando
K
K
Katagiri
T
T
Tauchi
T
T
Ito
Y
Y
Yaguchi
M
M
Miyazawa
K
K
Kimura
Y
Y
Masuda
M
M
Mizoguchi
H
H
Ohyashiki
K
K
D000368:Aged; D000741:Anemia, Aplastic / Q000188:drug therapy* / Q000235:genetics; D015139:Blotting, Southern; D016572:Cyclosporine / Q000627:therapeutic use*; D005260:Female; D015329:Gene Rearrangement, T-Lymphocyte; D015333:Gene Rearrangement, beta-Chain T-Cell Antigen Receptor; D015334:Gene Rearrangement, gamma-Chain T-Cell Antigen Receptor; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D008297:Male; D008875:Middle Aged; D009190:Myelodysplastic Syndromes / Q000188:drug therapy* / Q000235:genetics; D016133:Polymerase Chain Reaction
D007166:Immunosuppressive Agents; D016572:Cyclosporine