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Immune thrombocytopenic purpura presenting as paraneoplastic syndrome in a dedifferentiated liposarcoma: A case report

Juliana Magalhães, Marina Coelho, André Nunes, Inês Antunes, Ana Costa.

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Introduction: Immune thrombocytopenia (ITP) is an autoimmune bleeding disorder characterized by isolated low platelet counts, that occurs because of increased platelet destruction by the production of autoantibodies against platelet antigens. ITP secondary to solid neoplasia is rare. The treatment consists of pharmacological treatment to improve thrombocytopenia and the treatment of the underlying malignancy.
Case Report: A 72-year-old woman was admitted in the emergency department with petechial lesions and spontaneous ecchymosis with about three weeks of evolution. Analytically confirmed severe thrombocytopenia, without anemia and normal leukogram, and positive direct antigliadin test, admitting thrombocytopenic purpura.
In the subsequent etiological study, a nodular formation was identified in the right para renal area, later biopsied confirming the diagnosis of dedifferentiated retroperitoneal liposarcoma. Despite an initial favorable response to corticosteroid therapy, there was persistence of low platelet count despite first-line therapy (corticosteroids and immunoglobulin), with the need to associate second-line drugs (rituximab and Eltrombopag). There was remission of ITP after complete tumor excision.
Conclusion: The association of immune thrombocytopenia with dedifferentiated liposarcoma is very rare, with only one previous case described in the medical literature.
The treatment of an underlying malignancy may lead to resolution of thrombocytopenia, which is why, although the association of ITP with solid tumors is very uncommon, it is important to screen for solid tumor in the etiological study of ITP.

Key words: Immune thrombocytopenia, dedifferentiated retroperitoneal liposarcoma, paraneoplastic syndrome

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