Chronic myelomonocytic leukemia: 2016 update on diagnosis, risk stratification, and management

Abstract

Chronic myelomonocytic leukemia (CMML) is a clonal hematopoietic stem cell disorder characterized by overlapping features of myelodysplastic syndromes and myeloproliferative neoplasms. Diagnosis is based on the presence of persistent (>3 months) peripheral blood monocytosis (>1 × 109/L), along with bone marrow dysplasia. Clonal cytogenetic abnormalities occur in ∼20–30% of patients, while >90% have gene mutations. Mutations involving TET2 (∼60%), SRSF2 (∼50%), ASXL1 (∼40%), and RAS (∼30%) are frequent; with only ASXL1 mutations negatively impacting overall survival. Two molecularly integrated, CMML‐specific prognostic models include; the Groupe Français des Myélodysplasies (GFM) and the Molecular Mayo Model (MMM). The GFM model segregates patients into 3 groups based on: age >65 years, WBC >15 × 109/L, anemia, platelets <100 × 109/L, and ASXL1 mutation status, with respective median survivals of 56 (low), 27.4 (intermediate), and 9.2 (high) months. The MMM is based on ASXL1 mutational status, absolute monocyte count >10 × 109/L, hemoglobin <10 g/dL, platelets <100 × 109/L and circulating immature myeloid cells. This model stratifies patients into four groups; high (≥3 risk factors), intermediate‐2 (2 risk factors), intermediate‐1 (1 risk factor) and low (no risk factors), with median survivals of 16, 31, 59, and 97 months, respectively. Hypomethylating agents such as 5‐azacitidine and decitabine are commonly used, with overall response rates of ∼30–40% and complete remission rates of ∼7–17%. Allogeneic stem cell transplant is the only potentially curative option, but is associated with significant morbidity and mortality. Individualized therapy, including epigenetic modifiers and small molecule inhibitors, are exciting prospects. Am. J. Hematol. 91:632–642, 2016. © 2016 Wiley Periodicals, Inc.

Adult Chronic Myelomonocytic Leukemia with Trisomy 11: A Case Report

“…In most cmml patients, the disease course is indolent. However, because 15%-20% of patients can progress to acute myeloid leukemia 3 , cmml patients must be followed and managed carefully. Of all cmml patients, 30% have cytogenetic abnormalities.…”

“…Although most cmml patients have genetic mutations occurring in the TET2, SRSF2, ASXL1, or RAS genes, up to 30% of patients present with cytogenetic abnormalities 3 . The most common alteration is trisomy 8, followed by the absence of chromosome Y, chromosome 7 abnormalities, complex and monosomal karyotypes, and trisomy 21 4 .…”

Association of the Rares: the Occurrence of Chronic Myelomonocytic Leukemia During the Course of Necrotising Sarcoid Granulomatosis

“…Hastamızda splenomegali yoktu ancak belirtilen tam kan sayımı bulguları mevcuttu. Dünya Sağlık Örgütü (DSÖ) KMML 2008 tanı ölçütleri; çevre kanında monositoz varlığı (monosit > 1.000/mm 3 ), t(9;22) ve BCR-ABL negatifliği, PDGFRA ve PDGFRB rearanjmanlarının negatif olması (özel-likle eozinofili varlığında), çevre kanı ve kemik iliğinde blast yüzdesinin %20'nin altında bulunması (miyeloblast, monoblast ve promonositlerin sayılmasıyla) ve bir ya da daha çok seride displazi bulunmasıdır (6). KMML tanısı olan hastalar çevre kanı ve kemik iliğindeki blast veya promonosit sayısı-na göre ikiye ayrılırlar.…”

“…Kronik miyelomonositer lösemi (KMML) çevre kanında şiddetli ve inatçı monositoz ve kemik iliğinde miyelodisplastik ve miyeloproliferatif özelliklerin görüldüğü, nadir bir klonal hematopoietik kök hücre hastalığıdır (6). Monositozu bulunan bir hastada hematolojik tanılardan önce reaktif monositoz nedenlerinin dışlanması gerekmektedir (6).…”

“…Monositozu bulunan bir hastada hematolojik tanılardan önce reaktif monositoz nedenlerinin dışlanması gerekmektedir (6). Bu yazıda biri inflamatuvar diğeri neoplastik iki nadir durumun birlikte görüldüğü bir olgu sunulmaktadır.…”