Disappearing Myelodysplastic Syndrome-Associated Hemolytic Anemia in Leukemic Transformation

Abstract

Background: Here we report 2 rare cases of acute myeloid leukemia (AML) complicated with hemolytic anemia limited to the myelodysplastic syndrome (MDS) stage, and disappearing in leukemic transformation. Methods/Results: A 66-year-old man with MDS-RAEB-2 was admitted to hospital for severe anemia with increased reticulocyte counts. Hemolytic anemia was suspected, and it was ameliorated by methylprednisolone pulse therapy. Although anemia grew worse when steroids were tapered off, later improvement coincided with an increase in myeloblasts in the peripheral blood, i.e. with leukemic transformation. In another case, a 68-year-old man was admitted to hospital when laboratory findings showed a white blood cell count of 24,800/µl with increased myeloblasts (62.5%), leading to the diagnosis of AML with multilineage dysplasia. Following a decrease in blasts due to anti-cancer drugs, supporting the MDS-RAEB-2 status, severe anemia with increased reticulocytes and positive direct antiglobulin test was diagnosed, suggesting the existence of autoimmune hemolytic anemia, which was then ameliorated by steroid therapy. Conclusions: The simultaneous loss of autoimmunity and leukemic cell expansion observed in our cases may possibly suggest a common underlying mechanism.

Inflammatory and Immune Disorders Associated with Myelodysplastic Syndromes

“…In a caseseries of five paradigmatic cases of refractory/relapsing autoimmune cytopenias (AIHA, ITP and chronic idiopathic neutropenia) that evolved to idiopathic cytopenia/dysplasia of uncertain significance (ICUS/IDUS) -two recently recognized provisional conditions characterized by isolated unexplained cytopenia/dysplasia in <10% bone marrow cells [106,107] -Barcellini et al hypothesized a shift from autoimmunity against circulating blood cells to bone marrow precursors, leading to an insufficient marrow compensatory response, a progressive/variable degree of bone marrow dysplasia and ultimately overt bone marrow failure [108]. Furthermore, Tabata et al reported two cases of MDS patients with AIHA in which the progression toward AML was paralleled by the disappearance of hemolysis, corroborating the possible pathogenic link between both conditions [109].…”

Autoimmune Complications in Hematologic Neoplasms

“…Myeloproliferative neoplasms (MPN) may be rarely complicated by AICy, with isolated case reports of AIHA and ITP in Philadelphia (Ph)-negative MPN cases [55,60]. A case of steroidresponsive AIHA has been reported in post-MDS acute myeloid leukemia (AML) [56]. Finally, AIN and steroid-refractory ITP has been documented in patients with acute promyelocytic leukemia [61,62].…”

The burden of autoimmunity in myelodysplastic syndromes

“…Some reports focused on responses obtained with specific therapeutic approaches such as intravenous immunoglobulins or mycophenolate mofetil . Finally the possible pathogenetic link between MDS and AHA was further corroborated by 2 cases in which the progression towards over acute myeloid leukaemia (AML) was paralleled by the disappearance of haemolysis …”