Immunologic Abnormalities in Myelodysplastic Syndromes: Clinical Features and Characteristics of the Lymphoid Population

Abstract

It has been recognized that some patients with myelodysplastic syndromes (MDS) develop immunologic abnormalities, but little is known of its correlations to MDS-specific disease features. In a retrospective study of 284 MDS patients, we identified 32 patients (11.3%) with clinical or serologic immunological abnormalities (group A) and compared them to the remaining 252 cases (group B). Group A consisted of 20 patients with clinical signs of autoimmune disease and 12 asymptomatic patients with serologic immunological abnormalities only. Apart from significant female predominance in group A (M/F = 2.5 vs M/F = 0.7, p = 0.001), the other clinical and biological features such as median age, distribution of MDS subtypes, incidence of karyotyopic abnormalities, "abnormal" in vitro growth of GM-progenitors and survival times were similar in the two groups. Autoimmune manifestations partially responded to immunosuppressive therapy, with moderate improvement of peripheral cytopenia. In addition, CD3(+), CD4(+), CD8(+), CD19(+), and CD56(+) cells were quantified in peripheral blood of 38 patients. Matched with similarly aged healthy control group, most MDS patients showed significant lymphocytopenia, mainly due to the reduction of T-helper series (in both absolute numbers and percentage). B-cells were reduced in absolute numbers, but their percentage still overlapped with the control. No major abnormalities of natural killer cells (CD56(+)) were seen. We conclude that autoimmune diseases and asymptomatic immunologic abnormalities are common in patients with MDS, but except for female predominance, no correlation between these abnormalities and MDS-specific disease features were found.

Disappearing Myelodysplastic Syndrome-Associated Hemolytic Anemia in Leukemic Transformation

“…The most plausible explanation is that plasma cells producing autoreactive antibodies are replaced by leukemic cells after transformation, which leads to the hypogammaglobulinemia commonly observed in leukemic diseases. Indeed, a decrease in B cells and hypogammaglobulinemia is mostly observed in high-risk patients with MDS [7]. …”

An Immune Thrombocytopenic Purpura-Like Picture Preceding Chronic Myelomonocytic Leukemia: A Case Series and Review of the Literature

“…Autoimmune hemolytic anemia, Sweet's syndrome and Sjögren's sicca syndrome were diagnosed in 2 patients each and myasthenia gravis, temporal arteritis, pyoderma gangrenosum (PG), livedo reticularis with Raynoud syndrome and necrotizing panniculitis were diagnosed in 1 patient each. 7 There were 12 (4.2%) additional patients with asymptomatic serologic immunological abnormalities. In a study including 221 MDS patients, Enright et al 2 found that 30 (13.6%) cases had associated autoimmune disorders.…”

“…The prognostic worth of immune abnormalities in MDS is still unclear. 7 We performed an English literature review on the relationships of MDS and CMML with autoimmunity and ITP. PubMed (http://www.ncbi.nlm.nih.gov/pubmed) and Web of Science (Web of KnowledgeSM [v5.12], Thomson Reuters, http://apps.webofknowledge.com/ WOS) databases were used.…”

Myelodysplastic Syndromes: Review of Pathophysiology and Current Novel Treatment Approaches

“…Irregularities in B cell function have also been implicated in MDS based upon epidemiologic data revealing increased frequency of autoimmune disorders and immune-mediated complications in these patients [134,135]. Studies evaluating this correlation revealed the presence of either clinical or serologic manifestation of autoimmunity in 10-12% of MDS patients with a higher prevalence in younger patients, those with treatment-related MDS, or those with cytogenetic abnormalities [135,134].…”

“…Studies evaluating this correlation revealed the presence of either clinical or serologic manifestation of autoimmunity in 10-12% of MDS patients with a higher prevalence in younger patients, those with treatment-related MDS, or those with cytogenetic abnormalities [135,134]. Just as T cell repertoire skewing has been noted, clonality of B cells within MDS is also under investigation.…”