Abstract
Th2 dominancy in the peripheral T helper (Th) cell subsets were reported to be involved in the pathogenesis of pure red cell aplasia (PRCA). We encountered a PRCA case secondary to plasma cell dyscrasia that showed Th2 dominancy at the relapse of PRCA. Increased expression of c-maf, a transcriptional factor which induces Th2 differentiation of naive T-cells, and elevated expression of interleukin (IL)-4 were observed in the RNA derived from patient's bone marrow at relapse of PRCA. Following the administration of methylprednisolone which improved PRCA, normalization of Th1/Th2 ratio and decreased expression of c-maf and IL-4 were observed, which suggests that the upregulation of c-maf might have played a role in the pathogenesis of PRCA secondary to plasma cell dyscrasia.
MeSH terms
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Aged
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Bone Marrow / metabolism
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Cell Differentiation
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DNA-Binding Proteins / biosynthesis*
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DNA-Binding Proteins / genetics
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DNA-Binding Proteins / physiology
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Gene Expression Regulation*
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Humans
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Immunoglobulin kappa-Chains / analysis
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Immunoglobulin lambda-Chains / analysis
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Immunosuppressive Agents / therapeutic use
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Interferon-gamma / biosynthesis
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Interferon-gamma / genetics
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Interleukin-4 / biosynthesis
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Interleukin-4 / genetics
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Interleukins / biosynthesis
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Interleukins / genetics
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Male
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Methylprednisolone / therapeutic use
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Paraproteinemias / complications*
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Paraproteinemias / metabolism
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Paraproteins / analysis
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Proto-Oncogene Proteins / biosynthesis*
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Proto-Oncogene Proteins / genetics
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Proto-Oncogene Proteins / physiology
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Proto-Oncogene Proteins c-maf
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RNA, Messenger / biosynthesis
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RNA, Messenger / genetics
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Recurrence
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Red-Cell Aplasia, Pure / drug therapy
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Red-Cell Aplasia, Pure / etiology
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Red-Cell Aplasia, Pure / metabolism*
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Reverse Transcriptase Polymerase Chain Reaction
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Th2 Cells / immunology
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Thrombocytosis / etiology
Substances
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DNA-Binding Proteins
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Immunoglobulin kappa-Chains
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Immunoglobulin lambda-Chains
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Immunosuppressive Agents
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Interleukins
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MAF protein, human
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Paraproteins
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Proto-Oncogene Proteins
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Proto-Oncogene Proteins c-maf
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RNA, Messenger
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Interleukin-4
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Interferon-gamma
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Methylprednisolone