The findings come from the group’s review of over 30 years’ worth of research of the presence of autoimmune cytopenias and autoimmune diseases in lymphoid and myeloid neoplasms.
Autoimmune complications occur frequently in both lymphoid and myeloid neoplasms, say researchers, who have also found that the complications are sometimes hard to diagnose and are often severe and life-threatening.
The findings come from the group’s review of over 30 years’ worth of research of the presence of autoimmune cytopenias (AICy) and autoimmune diseases (AID) in lymphoid and myeloid neoplasms.
Complications include not just the well-known complications, such as autoimmune hemolytic anemia (AIHA) and immune thrombocytopenia (ITP), but also the poorly recognized AICy, such as autoimmune neutropenia (AIN) and aplastic anemia (AA), and poorly recognized AID, such as systemic lupus erythematosus and rheumatoid arthritis.
The review showed that nearly 10% of chronic lymphocytic leukemia (CLL) cases are impacted by AICy and are common in certain subtypes of non-Hodgkin lymphoma. Meanwhile, they are uncommon in low-risk myelodysplastic syndrome and chronic myelomonocytic leukemia, present in less than 1% of cases.
“Whilst AIHA and ITP are known complications, the rarer AIN, [pure red cell aplasia], and AA should be considered in the diagnostic algorithm of the cytopenic patient, taking into account bone marrow infiltration and/or peripheral destruction,” wrote the researchers. “Pitfalls for the latter include DAT-negative AIHA and the low sensitivity of anti-platelet and anti-neutrophil autoantibodies, whereas the kinetics of clinical presentation and response to steroid/[intravenous immunoglobulins] may help the diagnosis. CLL/lymphoma-specific therapy is generally required in relapsed/refractory AICy cases or in patients with progressive [lymphoproliferative disorders]-disease.”
AID are documented in nearly 1 in 3 lymphoid and myeloid cases, according to the literature, which found that these complications include those that may be severe and fatal.
When it comes to identifying AID, the researchers explained that identifying the complication and determining treatment has proven difficult as a result of their high heterogeneity and variable organ involvement, as well as a lack of specific diagnostic tests and low awareness from hematologists.
“AICy are generally related to LPD activity and may worsen the prognosis of the underlying disease, whereas AID may develop before the hematologic neoplasm or during its course, not clearly affecting its prognosis,” explained the researchers. “Likewise, AID-specific treatment may be required independently from the hematologic therapy. It is even more difficult to attribute a pathogenetic, clinical and prognostic value to the several autoimmune serological positivities reported in hematologic neoplasms, as they are not predictive of overt AICy/AID.”
The researchers also drew attention to emerging autoimmune complications, such as AICy in hematopoietic stem cell transplant and in the context of chimeric antigen receptor-T cell treatment, as well as AID during treatment with checkpoint inhibutors.
Barcellini W, Giannotta J, Fattizzo B. Autoimmune complications in hematologic neoplasms. Cancers. 2021;13(7):1532.