Background.The association of systemic sclerosis (SSc) and haematological cancers was reported in a large number of case reportsand cohort studies, describing SSc patients with highly heterogeneous clinical pictures. Objective. We reviewed the literature tobetter describe SSc patients with haematological malignancies. Methods. SSc cases complicated by haematological malignanciesdescribed in the world literature were collected; other 2 cases referred to our centre were reported. Results. One hundred-thirty SScsubjects were collected from 1954 up to date. The mean age of patients at cancer diagnosis was 56.1 ± 16.7 years; 72% of patientswere females. In 60% of cases, the diagnosis of haematological malignancy was described within 5 years of SSc diagnosis. In 7.8% ofcases, coexistence of Sj¨ogren’s syndrome or other autoimmune disorders was cited. Sixty-six cases with lymphoma (in the majorityof cases B-cell neoplasms), 28 with leukaemia (chronic lymphocytic form in 9), 14 with multiple myeloma plus one solitary IgMplasmocytoma, and 16 with myeloproliferative disorders were found. No specific SSc subsets seem to be related to haematologicalmalignancies. Conclusions. We remarked the importance of clinical work-up in SSc, in order to early diagnose and treat eventualoccult haematological malignancies, especially during the first years of the disease.

Haematological Malignancies in Systemic Sclerosis Patients: Case Reports and Review of the World Literature

COLACI, Michele
2017

Abstract

Background.The association of systemic sclerosis (SSc) and haematological cancers was reported in a large number of case reportsand cohort studies, describing SSc patients with highly heterogeneous clinical pictures. Objective. We reviewed the literature tobetter describe SSc patients with haematological malignancies. Methods. SSc cases complicated by haematological malignanciesdescribed in the world literature were collected; other 2 cases referred to our centre were reported. Results. One hundred-thirty SScsubjects were collected from 1954 up to date. The mean age of patients at cancer diagnosis was 56.1 ± 16.7 years; 72% of patientswere females. In 60% of cases, the diagnosis of haematological malignancy was described within 5 years of SSc diagnosis. In 7.8% ofcases, coexistence of Sj¨ogren’s syndrome or other autoimmune disorders was cited. Sixty-six cases with lymphoma (in the majorityof cases B-cell neoplasms), 28 with leukaemia (chronic lymphocytic form in 9), 14 with multiple myeloma plus one solitary IgMplasmocytoma, and 16 with myeloproliferative disorders were found. No specific SSc subsets seem to be related to haematologicalmalignancies. Conclusions. We remarked the importance of clinical work-up in SSc, in order to early diagnose and treat eventualoccult haematological malignancies, especially during the first years of the disease.
Haematological Malignancies; Blood cancers; Systemic Sclerosis; Review
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/20.500.11769/322062
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