Cutaneous mucosa-associated lymphoid tissue lymphoma complicating Sjögren's syndrome: A case report and review of literature

Table 1 Clinical characteristics of the 142 Sjögren's syndrome patients with mucosa-associated lymphoid tissue lymphoma reported in the literature

Patients characteristics	SS with MALT lymphoma (n = 142)
Age of SS diagnosis, mean year	45
Age of lymphoma onset, mean year	54
Female, n (%)	133/142 (93.7)
Dry eye, n (%)	45/108 (41.7)
Dry mouth, n (%)	45/108 (41.7)
Parotid gland swelling, n (%)	51/102 (50.0)
Fatigue, decreased appetite, or weight loss, n (%)	26/96 (27.1)
Cryoglobulinemia or cryoglobulinemic vasculitis, n (%)	10/77 (13.0)
ANA positive, n (%)	104/113 (92.0)
Anti-Ro/SSA positive, n (%)	100/113 (88.5)
Anti-La/SSB positive, n (%)	41/107 (38.3)
Monoclonal gammopathy, n (%)	37/82 (45.1)
RF positive, n (%)	87/102 (85.3)
Low C4, n (%)	31/77 (40.3)
Positive SG biopsy, FS ≥ 1, n (%)	36/41 (87.8)
Lymphoma location Parotid gland, n (%)	110 (77.5)
Lung, n (%)	21 (14.8)
Thymus, n (%)	8 (5.6)
Lymph nodes, n (%)	6 (4.2)
Bone marrow, n (%)	5 (3.5)
Submandibular glands, n (%)	3 (2.1)
Lacrimal gland, n (%)	2 (1.4)
Other sites, n (%) (stomach, breast, tongue, spleen, liver, kidney, mouth, lip)	9 (6.3)

SS: Sjögren's syndrome; MALT: Mucosa-associated lymphoid tissue; RF: Rheumatoid factor; SG: Salivary gland.

Table 2 Literature reviews of 26 published cases or case series

Ref.	n	Age of SS diagnosis	Age of lymphoma onset	Sex	Sicca symptom	Risk factor	Anti-SSA/SSB	Lymphoma location	Stage (Ann Arbor)	Treatment	Prognosis
Xu et al[3]	4	NA	42	M	—	PGS	SSA/SSB	Parotid gland, thymus	IVA	Surgery	Improved
		NA	49	F	—	PGS, Monoclonal gammopathy, RF	SSA/SSB	Parotid gland, thymus	IVA	Surgery	Improved
		NA	49	F	—	Monoclonal gammopathy	SSA/SSB	Thymus	IVA	Surgery	Improved
		NA	38	F	—	RF	SSA/SSB	Thymus	IA	Surgery	Improved
Belfeki et al[6]	1	60	65	F	Dry eye, dry mouth	PGS, Monoclonal gammopathy, low C3, low C4	SSA	Breast	NA	NA	NA
Xian et al[7]	1	NA	45	F	NA	NA	NA	Lung	NA	Chemotherapy	Deceased
Arai et al[12]	1	NA	66	F	NA	RF	SSA	Thymus	NA	Surgery	Improved
Momoi et al[13]	1	52	58	F	NA	RF	SSA	Thymus	NA	Surgery, chemotherapy	Improved
Yoshida et al[14]	1	32	NA	F	NA	Monoclonal gammopathy	SSA	Thymus	NA	Surgery, chemotherapy	Improved
Hsu et al[15]	1	NA	54	F	Dry eye, dry mouth	NA	SSA	Mouth	IE	Surgery	Improved
De Vita et al[16]	1	41	46	F	Dry eye, dry mouth	PGS, cryoglobulins,RF, low C4	SSA/SSB	Parotid gland	IE	Belimumab, RTX	Improved
Kobayashi et al[17]	1	31	39	F	Dry eye	NA	SSA/SSB	Lung	I	GS	NA
Taylor et al[18]	1	NA	67	F	NA	NA	NA	Lung	NA	Surgery	NA
Baqir et al[19]	3	NA	74	F	Dry eye, dry mouth	NA	SSA/SSB	Lung	NA	NA	NA
		NA	48	F	Dry eye, dry mouth	NA	SSA/SSB	Lung	NA	NA	NA
		NA	53	F	Dry eye, dry mouth	NA	SSA/SSB	Lung	NA	NA	NA
Kluka et al[20]	1	33	53	F	Dry eye, dry mouth	Monoclonal gammopathy	SSA/SSB	Lung	I	HCQ, GS	NA
Keszler et al[21]	1	60	62	F	Dry eye, dry mouth	RF	—	Minor labial salivary gland	I	—	Improved
Watanabe et al[22]	1	29	49	F	Dry eye, dry mouth	NA	SSA/SSB	Lung	IV	Chemotherapy	Improved
De Vita et al[23]	1	45	51	F	Dry eye, dry mouth	PGS, cryoglobulins, RF, low C4	SSA/SSB	Parotid gland	IE	RTX	Improved
Covelli et al[25]	2	32	35	F	Dry eye, dry mouth	PGS, RF, low C4	SSA	Parotid gland	NA	RTX	NA
		42	44	F	Dry eye, dry mouth	Monoclonal gammopathy, RF	SSA/SSB	Lung	NA	GS, RTX	NA
Ornetti et al[26]	1	73	73	F	Dry eye, dry mouth	PGS	SSA	Parotid gland	NA	RTX, chemotherapy	Improved
Movahed et al[27]	1	36	41	F	Dry mouth	PGS	NA	Submandibular glands	III	—	Improved
Zenone et al[28]	2	55	56	F	Dry eye, dry mouth	PGS, cryoglobulins, RF	—	Parotid gland	NA	RTX, chemotherapy, radiotherapy	Progressive
		31	42	F	Dry eye, dry mouth	PGS, cryoglobulins, Monoclonal gammopathy	SSA/SSB	Parotid gland	NA	HCQ, RTX	Improved

PGS: Parotid gland swelling; RF: Rheumatoid factor; RTX: Rituximab; GS: Glucocorticoids; HCQ: Hydroxychloroquine; NA: Not available; “-”: Negative.