Association of bone marrow fibrosis with inferior survival outcomes in chronic myelomonocytic leukemia

Khan, Maliha; Muzzafar, Tariq; Kantarjian, Hagop M.; Badar, Ifra; Short, Nicholas J.; Wang, Xuemei; Chamoun, Kamal; Jain, Preetesh; DiNardo, Courtney D.; Pemmaraju, Naveen; Bose, Prithviraj; Borthakur, Gautam; Cortes, Jorge; Verstovšek, Srđan; Estrov, Zeev

doi:10.1007/s00277-018-3289-6

Cited by 13 publications

(5 citation statements)

References 21 publications

(30 reference statements)

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“…Whenever fibrosis is present, a trichrome stain should be performed to document the presence of collagen fibrosis. Reticulin fibrosis in CMML has been suggested to be a poor prognostic indicator …”

Section: Diagnostic Workup Of Suspected Cases Of Cmmlmentioning

confidence: 99%

How I investigate chronic myelomonocytic leukemia

Sangiorgio

Arber

Orazi

2019

Int J Lab Hematology

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The 2016 revised 4th edition of the World Health Organization classification of hematopoietic neoplasms updated the diagnostic criteria for chronic myelomonocytic leukemia (CMML). Persistent peripheral blood monocytosis of at least 1 × 109/L and a percentage of monocytes ≥10% of the circulating white blood cell count (WBC) are both prerequisite criteria for this diagnosis. CMML represents the prototype of “overlapping” myeloid neoplasms with concurrent myeloproliferative and myelodysplastic features. However, clinical presentation is heterogeneous, with cases showing prevailing “dysplastic” features and others a predominant “proliferative” phenotype. Accounting for this diversity, two variants of CMML are recognized: “dysplastic” CMML defined by WBC < 13 × 109/L and “proliferative” CMML with WBC ≥ 13 × 109/L often showing features mimicking a myeloproliferative neoplasm. Although not an official WHO category, the “oligomonocytic” variant of CMML is defined by relative monocytosis with an absolute monocyte count of 0.5‐0.9 × 109/L. It can be considered a “pre‐phase,” as it frequently anticipates the development of an overt, classic CMML. In an attempt at improving disease prognostication, the blast count based grading system for CMML of the WHO 2008 Classification has been expanded in 2016 to include a new “CMML‐0” category. Lastly, the large body of knowledge on the molecular events occurring in CMML has been used to assist diagnosis and assess prognosis. Despite the step forwards, diagnosis of CMML still remains one of exclusion as no clinical, pathologic or molecular findings are specific for this disease. The current review brings insight into the spectrum of CMML and provides practical advice to approach suspected cases of CMML.

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Section: Diagnostic Workup Of Suspected Cases Of Cmmlmentioning

confidence: 99%

How I investigate chronic myelomonocytic leukemia

Sangiorgio

Arber

Orazi

2019

Int J Lab Hematology

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“…89 The presence of variable degrees of BM fibrosis (usually mild to moderate) has been reported in CMML cases, with several recent studies attempting to determine the prognostic value of this finding. 42,90,91 Indeed, although the data are not yet conclusive, the presence of marrow fibrosis in CMML seems to be of prognostic importance. 42,90,91…”

Section: Introductionmentioning

confidence: 99%

“…42,90,91 Indeed, although the data are not yet conclusive, the presence of marrow fibrosis in CMML seems to be of prognostic importance. 42,90,91…”

Section: Introductionmentioning

confidence: 99%

Proposed diagnostic criteria for classical chronic myelomonocytic leukemia (CMML), CMML variants and pre-CMML conditions

et al. 2019

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Chronic myelomonocytic leukemia (CMML) is a myeloid neoplasm characterized by dysplasia, abnormal production and accumulation of monocytic cells and an elevated risk of transforming into acute leukemia. Over the past two decades, our knowledge about the pathogenesis and molecular mechanisms in CMML has increased substantially. In parallel, better diagnostic criteria and therapeutic strategies have been developed. However, many questions remain regarding prognostication and optimal therapy. In addition, there is a need to define potential pre-phases of CMML and special CMML variants, and to separate these entities from each other and from conditions mimicking CMML. To address these unmet needs, an international consensus group met in a Working Conference in August 2018 and discussed open questions and issues around CMML, its variants, and pre-CMML conditions. The outcomes of this meeting are summarized herein and include diag nostic criteria and a proposed classification of pre-CMML conditions as well as refined minimal diagnostic criteria for classical CMML and special CMML variants, including oligomonocytic CMML and CMML associated with systemic mastocytosis. Moreover, we propose diagnostic standards and tools to distinguish between ‘normal’, pre-CMML and CMML entities. These criteria and standards should facilitate diagnostic and prognostic evaluations in daily practice and clinical studies in applied hematology.

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“…In contrast, CMML patients can demonstrate low levels of JAK2^V617F, MF-1 reticulin fibrosis, and hypolobulated, dysplastic megakaryocytes which were all present in our patient [ 12 ]. CMML patients with any degree of bone marrow fibrosis have been found to have a worse prognosis than those without [ 13 ].…”

Section: Discussionmentioning

confidence: 99%

Coexistence of Chronic Myelomonocytic Leukemia and Ulcerative Colitis With Rapid Progression to Acute Myelomonocytic Leukemia: A Case Report

Pelkey¹,

Graham²,

Zakem³

et al. 2022

Cureus

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Chronic myelomonocytic leukemia (CMML) is a clonal myeloid neoplasm characterized by sustained peripheral blood monocytosis and variable dyspoiesis. We present a case of a 64-year-old male who presented with severe non-bloody diarrhea, peripheral blood neutrophilia, and monocytosis. He was diagnosed with myeloproliferative CMML type 0 and ulcerative colitis (UC). Next-generation DNA sequencing of a bone marrow sample demonstrated mutations of the TET2, ASXL1, NRAS, and SRSF2 genes along with low-level JAK2^V617F mutation. Both TET2 and SRSF2 mutations are associated with systemic inflammatory and autoimmune disease (SIAD), which includes UC. The patient's UC was managed successfully with vedolizumab infusions. The patient’s concurrent CMML was monitored with a “wait and watch” approach. After five months, the patient asymptomatically tested positive for coronavirus disease 2019 (COVID-19). Seven months after his diagnosis of CMML, the patient presented in severe respiratory distress with acute left upper quadrant pain, splenomegaly, and multiorgan failure. A peripheral blood smear demonstrated marked leukocytosis (283 x 10^9 /L) with 39% blasts/promonocytes without Auer rods. The patient was diagnosed with acute myeloid leukemia with myelomonocytic features (AMML). In this report, we discuss the diagnosis of combined CMML and SIAD, mechanisms of immunoregulatory dysfunction that have been suggested to result in CMML progression, and the clinicopathologic significance of the patient’s molecular abnormalities.

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Association of bone marrow fibrosis with inferior survival outcomes in chronic myelomonocytic leukemia

Cited by 13 publications

References 21 publications

How I investigate chronic myelomonocytic leukemia

How I investigate chronic myelomonocytic leukemia

Proposed diagnostic criteria for classical chronic myelomonocytic leukemia (CMML), CMML variants and pre-CMML conditions

Coexistence of Chronic Myelomonocytic Leukemia and Ulcerative Colitis With Rapid Progression to Acute Myelomonocytic Leukemia: A Case Report

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