"Autoimmune conditions are common in CLL and are often managed in a distinct manner from primary autoimmune disease. Leukemic involvement of other organs is rare in CLL and should be managed in a distinct manner from CLL without these manifestations. The following review summarizes the diagnosis and management of common autoimmune phenomenon related to CLL as well as an organ site specific approach to extranodal involvement in CLL."
This review article by Max J. Gordon and Alessandra Ferrajoli was published in the American Journal of Hematology on 01 May 2022. It's titled "Unusual complications in the management of chronic lymphocytic leukemia".
The article covers the following AUTOIMMUNE COMPLICATIONS:
Autoimmune hemolytic anemia (AIHA) - "Autoimmune hemolytic anemia (AIHA) occurs in approximately 5%–10% of patients with CLL and is most frequently of the IgG warm antibody (wAIHA) subtype, although IgM autoreactive antibodies targeting red blood cells and lead to cold agglutinin disease (CAD) can also be observed."
Immune thrombocytopenia (ITP) - "Immune thrombocytopenia (ITP) occurs in around 1%–5%" with CLL
"Co-occurring AIHA and ITP is called Evans syndrome and occurs in <1% of patients with CLL."
"Hypogammaglobulinemia is observed in ~25% of patients with CLL at diagnosis and subsequently develops in an additional 25% of patients throughout the course of their disease. Hypogammaglobulinemia contributes to the increased risk of infection in patients with CLL. Additionally, hypogammaglobulinemia can exacerbate autoimmune complications, particularly AIHA and ITP."
These rarer conditions are also discussed:
Pure red cell aplasia and autoimmune granulocytopenia - "pure red cell aplasia (PRCA) and autoimmune granulocytopenia (AIG) each occur in less than 1% of patients with CLL."
Acquired von Willebrand disease and acquired hemophilia A
Non-hematologic autoimmune conditions: Paraneoplastic pemphigus, Glomerulonephritis, Angioedema due to acquired C1 esterase inhibitor deficiency ("C1 esterase inhibitor deficiency has been reported in up to 4% of patients with CLL but is rarely symptomatic") and Myasthenia gravis (MG).
Figure 1 in the article provides a table titled "Treatment of autoimmune cytopenias in patients with CLL"
The article also covers EXTRANODAL INVOLVEMENT in the:
skin
central nervous system (CNS)
kidney
lung
heart and
gastrointestinal organ systems
The article concludes:
"The autoimmune phenomenon, especially AIC, complicates the management of the patient with CLL and contribute to the heterogeneous clinical course of the disease. AIHA and ITP are the most common manifestations and may occur at any point in the clinical course of the disease. In patients with CLL who meet IWCLL criteria for treatment and have AIC, CLL-directed therapies are utilized and are often effective in treating both CLL and AIC. In patients who do not meet treatment criteria for CLL, the optimal management of AIC consists of immunosuppression. Usually CLL has the typical leukemia phenotype but can, in rare cases, infiltrate any organ. Symptomatic extranodal involvement with CLL is an indication for treatment. Otherwise unexplained organ dysfunction in a patient with CLL should prompt evaluation of leukemic infiltration. Treatment in these instances should take into consideration sites of disease and the presence of organ function compromise."
onlinelibrary.wiley.com/doi...
Gordon, MJ, Ferrajoli, A. Unusual complications in the management of chronic lymphocytic leukemia. Am J Hematol. 2022; 97( S2): S26- S34. doi:10.1002/ajh.26585
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