Table 4.

Autoimmune diseases (AID) complicating myeloid neoplasms.

AID	Myeloid Neoplasm	Frequency of AID	Key Findings	References
Systemic autoimmune disorders	MDS	20–30%	Mainly vasculitis including polyarteritis nodosa, giant cell arteritis, Behçet’s-like vasculitis and other less common types.Discrepancy about the impact of AID on MDS prognosis and survival. Biologics and azacytidine more effective than classic immunosuppressants (steroids and cytotoxic drugs).	[50,68,69,70,71,72,73,74,75]
	CMML	15–25%	AID-CMML patients are younger and mostly CMML-1, show similar AML progression and slightly longer overall survival than non-AID-CMML.Response to steroid is high, but 40–60% of cases need second-line treatment.	[57,68,76]
	Ph-negative MPN	Case reports	Case of polyarteritis nodosa, arthritis, Sjögren syndrome, intestinal autoimmune disorders, dermatomyositis, and multiple sclerosis mainly in MF.	[77,78,79,80]
Other hematologic AIDs	MDS	Case reports	Acquired HA and TTP.	[81,82]
	CMML	Case reports	Acquired HA, TTP, and APS, even catastrophic, either preceding or following CMML diagnosis.	[83,84,85]
	CML	Case reports	TTP and aHUS developed in imatinib- and dasatinib-treated patients.	[86,87]
	Ph-negative MPN	Case reports	Acquired HA, APS, and TTP (in a polycythemia vera patient treated with pegylated interferon).	[88,89]
	AML	Case reports	Acquired HA, acquired factor VII deficiency, fatal catastrophic APS (adult and paediatric, refractory to anticoagulation, plasma exchange and chemotherapy), and TTP.	[90,91,92,93]

AID autoimmune diseases, MDS myelodysplastic syndromes, CMML chronic myelomonocytic leukemia, Ph-negative MPN Philadelphia-negative myeloproliferative neoplasms, AML acute myeloid leukemia, CML chronic myeloid leukemia, MF myelofibrosis, HA hemophilia A, TTP thrombotic thrombocytopenic purpura, APS antiphospholipid syndrome, aHUS atypical hemolytic uremic syndrome.