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

Abstract

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

Myeloid disorders after autoimmune disease

“…(165) Sporadic case reports have reported on the association between SSc and myeloid malignancies such as CML (166,167) and AML. (168) Colaci et al (169) reviewed SSc patients with HMs who were described in world literature, from 1954 to 2017. One hundred-thirty SSc subjects were identified including 28 with leukemia and 16 with myeloproliferative disorders (4 with myelofibrosis, 1 with polycythemia vera, and 11 with CML).…”

“…One hundred-thirty SSc subjects were identified including 28 with leukemia and 16 with myeloproliferative disorders (4 with myelofibrosis, 1 with polycythemia vera, and 11 with CML). Median time from SSc diagnosis to identification of myelofibrosis and CML was 15 (5-17) and 3 (0-8) years, respectively (169). Notably, majority of the leukemias and CML were diagnosed in the first years of disease, when the immunosuppressors are least likely to have exerted an iatrogenic effect.…”

“…Notably, majority of the leukemias and CML were diagnosed in the first years of disease, when the immunosuppressors are least likely to have exerted an iatrogenic effect. (169) Detailed clinical history was available in only a few patients making it difficult to identify SSc-related myeloid leukemia risk factors.…”

Haematological abnormalities in systemic sclerosis

“…Colaci et al [26], who analysed a group of 130 systemic sclerosis patients with haematological cancers, demonstrated B-cell non-Hodgkin lymphoma in about 50% of them; multiple myeloma, chronic lymphocytic and myeloid leukaemia were found in a high percentage of patients. In single cases, Hodgkin disease, T-cell lymphoma, as well as myelofibrosis and polycythaemia vera, were diagnosed.…”

“…The authors reported that haematological cancers were significantly more common in men than in women (28% of male patients); no correlation between the type of sclerosis and haemato-oncological disease was observed. Furthermore, the highest incidence of haematological cancers was found in the 6 th decade of life; in 60% of cases, the neoplastic disease was diagnosed within 5 years following the diagnosis of systemic sclerosis [26].…”