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 mye...

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Bibliographic Details
Published in:British journal of haematology 2001-08, Vol.114 (2), p.358-361
Main Authors: Shimamoto, Takashi, Iguchi, Tomotaka, Ando, Keiko, Katagiri, Tomoko, Tauchi, Tetsuzo, Ito, Yoshikazu, Yaguchi, Makoto, Miyazawa, Keisuke, Kimura, Yukihiko, Masuda, Michihiko, Mizoguchi, Hideaki, Ohyashiki, Kazuma
Format: Article
Language:English
Subjects:
Aged
Anemia, Aplastic - drug therapy
Anemia, Aplastic - genetics
Biological and medical sciences
Blotting, Southern
cyclosporin A
Cyclosporine - therapeutic use
Female
Gene Rearrangement, beta-Chain T-Cell Antigen Receptor
Gene Rearrangement, gamma-Chain T-Cell Antigen Receptor
Gene Rearrangement, T-Lymphocyte
Hematologic and hematopoietic diseases
Hematology
Humans
Immunomodulators
Immunosuppressive Agents - therapeutic use
inverted CD4/8 ratio
Leukemias. Malignant lymphomas. Malignant reticulosis. Myelofibrosis
Male
Medical sciences
Middle Aged
myelodysplastic syndrome
Myelodysplastic Syndromes - drug therapy
Myelodysplastic Syndromes - genetics
Pharmacology. Drug treatments
Polymerase Chain Reaction
pure red cell aplasia
TCR rearrangements
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Summary: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)‐β and ‐γ 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.
ISSN:0007-1048
1365-2141
DOI:10.1046/j.1365-2141.2001.02925.x