Autoimmune Syndromes Presenting as a Paraneoplastic Manifestation of Myelodysplastic Syndromes: Clinical Features, Course, Treatment and Outcome

Abstract

Autoimmune manifestations (AIM) are reported in up to 10-30% of myelodysplastic syndromes (MDS) patients; this association is not well defined. We present herein a retrospective chart review of single center MDS patients for AIM, a case discussion and a literature review. Of 252 MDS patients examined, 11 (4.4%) had AIM around MDS diagnosis. International Prognostic Scoring System scores were: low or intermediate (int)-1 (n=7); int-2 or high (n=4). AIM were: culture negative sepsis (n=7); inflammatory arthritis (n=3); vasculitis (n=4); sweats; pericarditis; polymyalgia rheumatica (n=2 each); mouth ulcers; pulmonary infiltrates; suspicion for Behcet’s; polychondritis and undifferentiated (n=1 each). AIM treatment and outcome were: prednisone +/- steroid sparing agents, n=8, ongoing symptoms in 5; azacitidine (n=3), 2 resolved; and observation, n=1, ongoing symptoms. At a median follow up of 13 months, seven patients are alive. In summary, 4.4% of MDS patients presented with concomitant AIM. MDS should remain on the differential diagnosis of patients with inflammatory symptoms.

The Pulmonary Complications of Paraneoplastic Autoimmune Vasculitis in a Patient With Myelodysplastic Syndrome

“…This case of paraneoplastic autoimmune vasculitis in a patient with MDS highlights diffuse alveolar hemorrhage as a rare but fatal complication. Autoimmune phenomena are demonstrable in up to 30% of MDS patients with varied manifestations [4]. In this patient, a broad differential initially included copper deficiency, HLH, acute leukemia, MDS, and septic shock secondary to pneumonia.…”

“…The authors have also noted that vasculitides associated with MDS resulted in more frequent renal involvement (p = 0.02) and steroid dependence (p = 0.04); remission was less frequently achieved in MDS-mediated vasculitis compared to vasculitides associated with other malignancies (p = 0.04) [ 3 ]. Immune-mediated hematologic presentations, such as anemia and thrombocytopenia from an MDS-associated paraneoplastic syndrome, is uncommon [ 4 ]. This report discusses the case of a 55-year-old male patient who developed an autoimmune vasculitis secondary to myelodysplasia.…”

Tuning of the Hematopoietic Stem Cell Compartment in its Inflammatory Environment

“…Metabolic status, age, hygiene conditions and immunological history are some of the multiple parameters that alter the basal inflammatory state and influence HSC homeostasis. Interestingly these factors have been associated with altered immunological functions but also with hematological pathologies including myelodysplastic syndromes (MDS), myeloproliferative neoplasms (MPNs) and acute myeloid leukemia (AML) [72–76]. Whether the lone effect of inflammation on HSCs is able to promote hematological diseases remains to be formally established.…”

Stress and Non-Stress Roles of Inflammatory Signals during HSC Emergence and Maintenance

“…Although the precise connection is still being elucidated, several studies have correlated MDS with inflammatory disorders. In a recent study, 4.4% of MDS patients presented also autoimmune manifestations (106). In addition, it was reported that inflammatory bowel disease patients were diagnosed with MDS.…”