Associazione tra trombocitemia essenziale e connettivite indifferenziata: Descrizione di un caso

Translated title of the contribution: Association between essential thrombocytemia and autoimmune disorders: Description of one case

C. Pagliolico, A. Santagostino, P. G. Delvino, R. Mari, G. Camisasca, A. Bosetti, C. Rabascio, V. Bolis, M. Roccella, M. Ferraris

Research output: Contribution to journalArticlepeer-review


Sometime thrombocytosis is observed in patients with active autoimmune diseases, but the mechanisms of thrombocytosis is not clear; perhaps interleukin 3 (IL-3), IL-4, IL-6 and granulocyte-macrophage colony stimulating factor (GM-CSF) may be possible stimulators for thrombopoiesis. Generally the platelet count fluctuates with disease activity, but thrombocytosis of over 600 x 109/L is rare. We report a case of a patient with autoimmune disease complicating with thrombocytosis over 1.000 x 109/L. The patient was a 58 year old man who, in 1989, developed an autoimmune disorder characterized by hip pain, positivity of RA-test, Waaler Rose, ANF and anti-ENA (extractable nuclear antigens). The platelet count at the onset was 640 x 109/L. The patients was treated with 6 mg of deflazacort. In the following years we observed an increase of platelet count, in spite of the remission of autoimmune disease, and mild splenomegaly, so he was admitted to the hospital for workup of thrombocytosis with bone marrow biopsy. The analysis of needle biopsied marrow showed hypercellularity with marked increase in abnormal megacariocyte. Chromosome study of hone marrow cells, performed above all to exclude the presence of Ph' chromosome, didn't show any cytogenetical abnormality. The diagnosis was Essential Thrombocytemia and the patient was treated with pipobromano with good response. It is well known that immunological abnormalities can cause various haematological disorders including malignant lymphoma, aplastic anemia, pure red cell aplasia, in our patient they preceded a myeloproliferative syndrome. The present cases suggest that thrombocytosis inappropriate with disease activity might occur and the careful evaluation of the thrombocytosis is required to confirm a myeloproliferative disorder.

Translated title of the contributionAssociation between essential thrombocytemia and autoimmune disorders: Description of one case
Original languageItalian
Pages (from-to)113-116
Number of pages4
Issue number2
Publication statusPublished - 1997

ASJC Scopus subject areas

  • Rheumatology

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