Anti-c-Mpl (thrombopoietin receptor) autoantibody-induced amegakaryocytic thrombocytopenia in a patient with systemic sclerosis

Yasuhiro Katsumata, Takahiro Suzuki, Masataka Kuwana, Yutaka Hattori, Satoshi Akizuki, Hitoshi Sugiura, Yasuo Matsuoka

Research output: Contribution to journalArticle

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Abstract

Amegakaryocytic thrombocytopenia (AMT) associated with systemic sclerosis (SSc) has been described in several case reports, but the underlying mechanisms have not been identified. Here we describe a rare case of SSc accompanied by thrombocytopenia and megakaryocytic hypoplasia, in which autoantibody against thrombopoietin receptor (c-Mpl) was detected. A 61-year-old woman with limited SSc was admitted to our hospital because of severe thrombocytopenia (platelet count 0.2 × 104/mm3) with gingival bleeding. Her bone marrow was hypocellular with absent megakaryocytes, consistent with AMT. Treatment with corticosteroids and intravenous immunoglobulin infusions resulted in an increased platelet count, and she sustained a remission over a 1-year period, with a platelet count averaging 10.0 × 104/mm3. Her serum was strongly positive for antic-Mpl antibody, and IgG fraction purified from her serum inhibited thrombopoietin-dependent cell proliferation in vitro. Our case report suggests that AMT in patients with SSc could be mediated by the anti-c-Mpl antibody, which functionally blocks an interaction between thrombopoietin and c-Mpl.

Original languageEnglish
Pages (from-to)1647-1651
Number of pages5
JournalArthritis and Rheumatism
Volume48
Issue number6
DOIs
Publication statusPublished - 2003 Jun 1

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Thrombopoietin Receptors
Systemic Scleroderma
Thrombocytopenia
Autoantibodies
Platelet Count
Thrombopoietin
Megakaryocytes
Intravenous Immunoglobulins
Serum
Intravenous Infusions
Adrenal Cortex Hormones
Immunoglobulin G
Bone Marrow
Cell Proliferation
Hemorrhage
Antibodies

ASJC Scopus subject areas

  • Immunology
  • Rheumatology

Cite this

Anti-c-Mpl (thrombopoietin receptor) autoantibody-induced amegakaryocytic thrombocytopenia in a patient with systemic sclerosis. / Katsumata, Yasuhiro; Suzuki, Takahiro; Kuwana, Masataka; Hattori, Yutaka; Akizuki, Satoshi; Sugiura, Hitoshi; Matsuoka, Yasuo.

In: Arthritis and Rheumatism, Vol. 48, No. 6, 01.06.2003, p. 1647-1651.

Research output: Contribution to journalArticle

Katsumata, Yasuhiro ; Suzuki, Takahiro ; Kuwana, Masataka ; Hattori, Yutaka ; Akizuki, Satoshi ; Sugiura, Hitoshi ; Matsuoka, Yasuo. / Anti-c-Mpl (thrombopoietin receptor) autoantibody-induced amegakaryocytic thrombocytopenia in a patient with systemic sclerosis. In: Arthritis and Rheumatism. 2003 ; Vol. 48, No. 6. pp. 1647-1651.
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