Author Year No. of cases Age Sex Oral location Other locations Risk factors Treatment Follow-up
Speight et al. [23] 1994 4 NR NR Lipa Stomach NR NR NR
NR NR Lipa Palate NR NR NR
NR NR Lipa Cervical lymph nodes NR NR NR
NR NR Lipa Cervical lymph nodes NR NR NR
Van Mello et al. [24] 2005 3 53 F Lipb Possibly chest area Non-blanching purpuric lesions on the lower limbs, C4 low NR NR
51 F Lipb NR Intermittent swollen parotids, raised levels of total protein,
IgG, IgA, IgM, while C4 levels and white blood cell count were decreased
55 Lipa NR Intermittent swelling of the parotid gland Leukeran (4 mg daily)
and prednisone 20 mg/day
Pijpe et al. [25] 2005 1 42 F Hard palateb Right Parotid 10 years
earlier neck lymph nodes
Increased IgG Weekly, course of 4 infusions of rituximab NS, no recurrence
Sakuma et al. [26] 2006 1 70 F Hard palatea - - Self regression 38 months, no recurrence
Keszler et al. [27] 2012 1 60 F Lipa - - Watch and wait Evaluation performed every 6 months during
the first year and then, once a year, no recurrence
Shwetha et al. [28] 2014 1 62 F Upper and
lower lipb
- - Watch and wait NS, no recurrence
Present case 2017 1 64 F Hard
Stomach; spleen; bone marrow CD4 + T lymphocytopenia, neutropenia, high serum β2 microglobulin, low serum IgM levels Refused treatment for 1 year
(Rituximab + Fludarabine)
2 years, no recurrence

aMALT lymphoma diagnosed after Sjögren’s syndrome initial diagnosis.
bMALT lymphoma diagnosed simultaneously with Sjögren’s syndrome.
NR = not reported; F = female; NS = not specified.