Patients with primary dry syndrome can present with a variety of hematologic abnormalities, including anemia, leukopenia, thrombocytopenia, hypergammaglobulinemia, and monoclonal immunoglobulinopathy. In addition, patients with primary dry syndrome are also prone to malignant lymphoproliferative diseases, mainly B-cell-derived non-Hodgkin’s lymphoma. 1.Anemia According to most literature reports, about one-fourth of patients with primary dry syndrome have anemia, mostly mild orthocytic orthochromic anemia. Those with primary dry syndrome with anemia are more likely to have kidney involvement, skin vasculitis, peripheral neuropathy, antinuclear antibody, anti-SSA, anti-SSB, rheumatoid factor, cryoglobulinemia and hypocomplementemia than those without anemia. Patients are also often first seen in hematology clinics for anemia. 2. Leukopenia Literature reports that 30% of patients with primary dry syndrome have lower than normal white blood cells, and 25% of patients with primary dry syndrome have increased eosinophils or lymphocytes. Some studies have shown that the incidence of peripheral neuropathy, anti-SSA, anti-SSB, rheumatoid factor, cryoglobulinemia and hypocomplementemia is higher in patients with primary dry syndrome with leukopenia compared with those without leukopenia, but multi-factor analysis found that only anti-SSA and rheumatoid factor are meaningful independent variables. 3. Thrombocytopenia The hematology department of the author often has patients who are hospitalized for thrombocytopenic purpura, and the examination reveals high titers of positive SSA and SSB antibodies, and later the diagnosis of primary desiccation syndrome is confirmed after lipstick biopsy, etc. Some research analysis found that primary dry syndrome with platelet hypoplasia is more likely to have renal involvement and anti-SSB positivity than those with normal platelets, and multi-factor analysis also confirmed that both are meaningful independent variables. 4.Monoclonal immunoglobulin disease 50% of patients with primary dry syndrome have reduced albumin and increased polyclonal globulin, and all three major immunoglobulins can be increased, with IgG being the most obvious, and IgA and IgM can also be increased, but it is less common and less severe. Macroglobulin or mixed cryoglobulinemia is less common, and such patients often have a clinical hyperviscosity syndrome. 5. Lymphoproliferative diseases Among autoimmune diseases, primary dry syndrome has the highest chance of malignant lymphoproliferative diseases, so SS is considered a crossroads between autoimmune and lymphoproliferative diseases. In the latest meta-analysis of a large series of studies on the incidence of non-Hodgkin’s lymphoma in autoimmune diseases, which included 8700 cases of SLE (6 studies), 95104 cases of rheumatoid arthritis (9 studies) and 1300 cases of pSS (5 studies), the highest incidence of non-Hodgkin’s lymphoma in primary dry syndrome was found to be 18.8%, compared with SLE lupus erythematosus was 7.4% and rheumatoid arthritis was only 3.9%. The incidence and risk of lymphoma in each study are different due to different diagnostic criteria and different follow-up time. 6.Other hematological malignancies Multiple myeloma is rare in primary dry syndrome. There is a case report of extramedullary IgG and IgA plasmacytoma of salivary gland and primary junctional plasmacytoma. Other hematologic malignancies such as T-cell large granular lymphocytic leukemia, angioimmunoblastomatous lymphadenopathy with abnormal proteinemia, and multicentric Castleman disease are also seen in patients with SS.