Table 1.

Autoimmune cytopenias (AICy) complicating lymphoproliferative disorders.

AICy	Lymphoproliferative Disorder	Frequency of AICy	Key Findings	References
AIHA	CLL	7 to 15%	The commonest AICy correlating with advanced disease and high biologic risk (del 11q, del17p, unmutated IGHV)	[1,11]
	NHL	2 to 50%	Frequency is maximal in angioimmunoblastic T-cell lymphoma and in marginal zone lymphoma	[5,12]
	HL	0.2%	Very rare association, but may increase after HSCT or therapy with CPIs.	[4,5,13,14]
	LGLL	Case reports	May be concomitant, precede or follow LGL diagnosis; may be concomitant to ITP and AIN and be multi-refractory (even require splenectomy)	[15]
	CD	Case reports to 6%	May revert after anti-IL6 therapy with tocilizumab	[16]
	ALL	Case reports	Mainly B-ALL in the pediatric setting and post-HSCT	[17]
	MM	Case reports	May rarely complicate MM and also precede the diagnosis.	[18]
ITP	CLL	1 to 5%	Even in association with AIHA (ES). Difficult to distinguish from infiltrative cytopenia; response to steroids and IVIG may confirm the diagnosis	[11]
	NHL	Case reports	Mainly in Waldenström macroglobulinemia and marginal zone NHL; may also follow fludarabine treatment and HSCT.	[12]
	HL	0.2 to 1%	May precede or follow HL diagnosis and be observed even after remission; ITP risk may increase after HSCT or CPIs.	[13,14,19]
	LGLL	1 to 20%	May respond to steroids, IVIG and cytotoxic immunosuppressants used for LGL	[20]
	CD	Case reports	Case reports of ITP and ES during CD progression, may respond to rituximab or be refractory	[21]
	ALL	Case reports	Mostly during chemotherapy. Some require more than 3 lines including splenectomy	[22]
	MM	Case reports	MM may be complicated by ITP and ES; lenalidomide may increase the risk.	[23,24]
AA/PRCA/AIN	CLL	<1%	AIN has been reported in only 3 out of 1750 patients (0.17%); anti-neutrophil autoantibodies may be positive	[11]
	NHL	Case reports	Up to 21 cases of PRCA and rarely AA reported; may develop at onset, during remission, or after chemotherapy and/or HSCT; may be associated with EBV infection and AIHA	[12]
	HL	Case reports	16 patients reported in literature. Case reports of AIN, even years after remission, successfully treated with IVIG	[14,25]
	LGLL	Case reports	Three patients with concomitant amegakaryocytic thrombocytopenia and PRCA and 1 with concomitant AIHA, ITP and AIN	[26]
	ALL	Case reports	Two patients with T-ALL developed PRCA, possibly associated with ALL therapy; 1 patient with T-lymphoblastic lymphoma presented as AA and hypercalcemia	[27]
	MM	Case report	Association of AIN and anti-thyroid autoantibodies	[28]

AIHA autoimmune hemolytic anemia, ITP immune thrombocytopenia, AA aplastic anemia, PRCA pure red cell aplasia, AIN autoimmune neutropenia, CLL chronic lymphocytic leukemia, NHL non-Hodgkin lymphoma, HL Hodgkin lymphoma, LGLL large granular lymphocyte leukemia, CD Castleman disease, ALL acute lymphoblastic leukemia, MM multiple myeloma; HSCT hematopoietic stem cell transplant; CPIs checkpoint inhibitors, ES, Evans syndrome, IVIG intravenous immunoglobulins, EBV Epstein Barr virus.