CD123 is a cell surface target expressed on a wide range of myeloid tumors including blastic plasmacytoid dendritic cell neoplasm (BPDCN), certain myeloproliferative neoplasms (MPNs) including chronic myelomonocytic leukemia (CMML
) and myelofibrosis (MF), acute myeloid leukemia (AML) (and potentially enriched in certain AML subsets), myelodysplastic syndrome (MDS), and chronic myeloid leukemia (CML).
Currently, Astex is evaluating guadecitabine (SGI-110) in relapsed and refractory acute myeloid leukemia (R/R AML) as well as relapsed and refractory myelodysplastic syndromes (R/R MDS) and CMML
Disorders altering the myeloid elements include the following disorders: Polycythemia vera (PV), essential thrombocythemia (ET), primary myelofibrosis (PMF), chronic myelogenous leukemia (CML), chronic neutrophilic leukemia (CNL), hypereosinophilic leukemia/CEL, CMML
, and mast cell disease.
Thirteen patients with CMML
(10 males and 3 females) and 10 healthy donors (7 males and 3 females) were included in this study.
He was rehospitalized in September 2016 for a stem cell transplant for his CMML
. 21-OH antibodies were tested during that admission and were negative (<0.1).
Intermediate/highrisk IPSS (International Prognostic Scoring System) patients affected by myeloid dysplastic syndromes(MDS), acute myeloid leukemia (AML), and chronic myelomonocytic leukemia (CMML
) (n = 14) were treated by s.c.
A 68-year-old Caucasian woman recently diagnosed with CMML
presented to our rheumatology office for evaluation of an abnormal chest computed tomography (CT) showing inflammation surrounding the entire thoracic and abdominal aorta.
Recently, SETBP1 mutations have also been identified in aCML and other closely related hematological malignancies, including CNL, CMML
, unclassified MDS, MPNs, and secondary AML evolving from MDS at variable frequencies (1.7-25%) (9-13), as shown in Table 1.
(57,58) On the other hand, SRSF2 mutations are closely associated with CMML
; 28.4% of patients with CMML
harbor SRSF2 mutations.58 EZH2 mutations are more frequently found in MDS patients and are rare in de novo AML patients.
Murine models have also demonstrated that TET2 deletion results in progressive myeloproliferation, extramedullary hematopoiesis, and expansion of undifferentiated myeloid precursors occurring in a pattern highly reminiscent of human CMML
Kulasekararaj et al., "Next-generation sequencing of the TET2 gene in 355 MDS and CMML
patients reveals low-abundance mutant clones with early origins, but indicates no definite prognostic value," Blood, vol.