scholarly journals Chronic monocytic leukemia with transformation into acute monocytic leukemia. Clinical case

Morphologia ◽  
2021 ◽  
Vol 14 (4) ◽  
pp. 49-57
Author(s):  
L. A. Pesotskaya ◽  
A. S. Korolenko

Background. Chronic myelomonocytic leukemia (CML) is rarely diagnosed and it is 1 per 100 thousand adults annually, in the United States - in 4 per million people, which is about 1100 cases per year. This disease is more common for men over 60. Results. A clinical case of a rare long-term course of myelodysplastic chronic myelomonocytic leukemia (MDCMML) in a middle-aged woman with rapid transformation into acute monocytic leukemia (AMoL-M5v) with atypical fulminant course is presented. Changes in the blood test were identified accidentally during a routine examination. A retrospective analysis of the course of the patient's disease, anamnesis made it possible to draw attention to the severe course of vasculitis of unknown etiology, with a predominant lesion of the skin of the lower limbs, which required inpatient treatment (19 years ago); skin lesions in the form of transient erythema, spotty eruptions for more than 10 years, moderate cervical lymphadenopathy. According to the WHO criteria, the morphological data of the bone marrow puncture corresponded to the MD of the CML. The long course of the disease without an obvious clinical picture, neutrophil dysplasia, myeloid proliferation was atypical, which did not exclude the presence of previous oligomonocytic CML in the patient. A detailed picture of the disease appeared after a viral infection, bronchitis, antibiotic therapy. In the absence of an increase in the number of blasts in the bone marrow, in a few of them normal Auer's sticks were detected, which, according to the literature, is a rarity in CML and an unfavorable prognostic factor of rapid transformation into acute myeloid leukemia. Conclusion. Not typical for the course of acute monocytic leukemia in this case were the absence of significant blastemia and severe suppression of normal hematopoiesis with pronounced extramedular manifestations. There was febrile fever, hyperplasia of the gums, tonsils with ulcerative-necrotic changes in the oral mucosa, an increase in cervical lymph nodes in the form of packets up to 2 cm in diameter with signs of sarcomatous growth. Attention was drawn to the progression of skin lesions, which was prognostically unfavorable. Notable was the development of severe hemorrhagic syndrome without severe thrombocytopenia, significant changes in the coagulogram, as a manifestation of early severe coagulopathy. There was a spread of erythematous elements on the skin with itching, not controlled by antihistamines and corticosteroid drugs (maculopapular rashes of a pink-cyanotic color, in places of a confluent nature, small-point hemorrhages like vasculitis over the entire surface of the skin).

Blood ◽  
2011 ◽  
Vol 118 (21) ◽  
pp. 4288-4288
Author(s):  
Caroline Hamm ◽  
Sindu M. Kanjeekal

Abstract 4288 Seventy year old man presented to Windsor Regional Cancer Centre with fever and pancytopenia on June 24, 2009. Presenting CBC was as follows: WBC 47.8:Ne 0.3, Ly 0.06, Mo 0.62, Meta 0.01, bands 0.01; Hgb 123; Platelets 55,000. A bone marrow biopsy revealed the following: 70% cellularity with sheets of immature blast-like cells; the lesional cells were CD68 and MPO positive and negative for CD34, CD117, CD138, CD20 and CD3. Flow cytometry revealed 46% of the cells positive for CD33, Cd36, CD64, MY4, CD16, HLA-DR, CD13, CD 56, CD10, CD11b, dim CD4, MPO positive, consistent with acute monocytic leukemia. Cytogenetics were 46, XY [24]. He was given the diagnosis of acute monoblastic leukemia and was started on standard induction chemotherapy: 3 + 7 daunorubicin and ara-C. (doses). Repeat bone marrow biopsy on July 17, 2009 revealed lack of remission. This bone marrow revealed sheets of blast-like cells with 30% residual monoblast population and 4% myeloblast population. He was then treated with high dose cytarabine (3 gm / m2 q day 1, 3, 5) for one cycle. Repeat bone marrow biopsy on Aug 20, 2009 revealed non-remission with 20% residual monoblasts. At this time, it was explained to him that he would be treated in a palliative fashion only. He started low dose AraC at this time and received one 21 day course. He showed hematological recovery by September 2009. Repeat bone marrow biopsy on October 5th, 2009 showed 6% residual myeloblast and 40% monocytic population. Because of previous signals of response at our centre to dandelion root tea/ water extract, and because of his current palliative diagnosis, we mentioned dandelion root tea to him. He started this tea on his own and was followed expectantly. Another bone marrow biopsy at the end of November 2009 showed only residual monocytic population of 10 – 12 % with 79% myeloid cells showing dysplastic features consistent with chronic myelomonocytic leukemia type 2. In March 2010, his platelet count started a gradual decline. By December 29, 2010, the platelet count had dropped to 35,000 × 10 9, and a bone marrow biopsy was done to determine etiology. Bone marrow biopsy from January 25, 2011 shows features suggestive chronic myelomonocytic leukemia. This biopsy revealed monocytic cells, as well as occasional promonocytes. There was adequate megakaryoctyes, no Auer rods, no blasts. The monocytes expressed 9% of the total nucleated cells, and did not express CD56. Flow cytometry reported all normal. A diagnosis of idiopathic thrombocytopenic purpura was made and patient was started on prednisone with subsequent improvement in his platelet count. CBC on July 4, 2011, almost two years from his diagnosis of refractory M5 AML, patient’s CBC is almost normal with a white blood count of 7.5 ×10*9/L, hemoglobin of 122 g/L, platelets of 134 ×10*9/L, neutrophils of 3.6 × 10*9/L, monocytes of 1.65 × 10*9/L. His quality of life remains excellent. He continues using dandelion root tea. In view of this very exciting response and the more temporary response seen in chronic myelomonocytic leukemia, as well as the exciting findings we have seen in monocytic cells lines and tolerance in animal models, we moving into a phase I/II clinical trial examining the effect of dandelion root extract in patients with monocytic leukemias. Disclosures: No relevant conflicts of interest to declare.


Blood ◽  
2007 ◽  
Vol 110 (11) ◽  
pp. 4264-4264
Author(s):  
Chandrika Sreekantaiah

We report a recurrent translocation (X;20)(q13;q13.3) in three patients. The translocation was the sole chromosomal abnormality in all three patients and the number of cells with the abnormality varied from three to seventeen out of twenty metaphases analyzed for each patient. The patients were all female with ages ranging from 66 to 83. The presenting symptoms were variable but all included a history of anemia. Bone marrow aspiration showed acute monocytic leukemia in one patient and normocellular bone marrow with no detectable morphologic or immunophenotypic evidence of neoplasm in the other two. Only eight cases with the translocation have previously been reported. Seven of these cases had either myelodysplastic syndrome or acute myeloid leukemia and one patient had pancytopenia of unknown etiology. Repeated bone marrow evaluations on this patient showed no dyspoietic changes. The t(X;20) has clearly been established as a nonrandom abnormality, however, the clinical significance of the translocation is not clear. Close follow up of these patients is therefore essential. Characterization at the molecular level will also help to determine the genes involved and the mechanism of their action.


2020 ◽  
Vol 51 (1) ◽  
pp. 51-55
Author(s):  
Marta Panz-Klapuch ◽  
Krzysztof Woźniczka ◽  
Anna Koclęga ◽  
Anna Kopińska ◽  
Kinga Boral ◽  
...  

AbstractIntroductionSystemic mastocytosis (SM) with an associated hematological neoplasm (SM-AHN) constitutes about 40% of all patients with SM. AHN commonly includes myeloid neoplasms and chronic myelomonocytic leukemia (CMML) is seen in about 30% of these patients.Case reportA 67-year-old male presented to hematologist with fatigue and significant weight loss. Abdominal ultrasound and computed tomography (CT) detected hepatosplenomegaly, abdominal lymphadenopathy, and ascites. He was anemic with leukocytosis and eosinophilia. Trephine biopsy showed > 30% of spindle-shaped mast cells. The KITD816V mutation was present. Serum tryptase level was elevated to 62 ng/mL. The patient was diagnosed with aggressive SM and received six cycles of cladribine with partial response. Three years later, he developed severe anemia. Eosinophilia and monocytosis (5.6 × 109/L) were demonstrated in blood film. Hepatosplenomegaly and abdominal lymphadenopathy were also present. Trephine biopsy did not demonstrate the presence of spindle-shaped mast cells, but dysplasia in erythroid and myeloid lineages was evident. The histological result of lymph node biopsy as well as blood and bone marrow findings were in line with CMML. He received hydroxyurea, but he transformed soon into fatal acute monocytic leukemia.ConclusionsThe prognosis of SM-AHN depends on AHN component. Leukemic transformation of AHN component may occur in a proportion of patients.


2021 ◽  
Vol 12 ◽  
Author(s):  
Grégoire Martin de Frémont ◽  
Pierre Hirsch ◽  
Santiago Gimenez de Mestral ◽  
Philippe Moguelet ◽  
Yoan Ditchi ◽  
...  

BackgroundMyelodysplastic syndromes (MDS) and chronic myelomonocytic leukemia (CMML) are associated with cutaneous manifestations. Next-generation sequencing (NGS) is a tool capable of identifying clonal myeloid cells in the skin infiltrate and thus better characterize the link between hematological diseases and skin lesions.ObjectiveTo assess whether skin lesions of MDS/CMML are clonally related to blood or bone marrow cells using NGS.MethodsComparisons of blood or bone marrow and skin samples NGS findings from patients presenting with MDS/CMML and skin lesions in three French hospitals.ResultsAmong the 14 patients recruited, 12 patients (86%) had mutations in the skin lesions biopsied, 12 patients (86%) had a globally similar mutational profile between blood/bone marrow and skin, and 10 patients (71%) had mutations with a high variant allele frequency (>10%) found in the myeloid skin infiltrate. Mutations in TET2 and DNMT3A, both in four patients, were the most frequent. Two patients harbored a UBA1 mutation on hematopoietic samples.LimitationsLimited number of patients and retrospective collection of the data. Blood and skin sampling were not performed at the exact same time point for two patients.ConclusionSkin lesions in the setting of MDS/CMML are characterized by a clonal myeloid infiltrate in most cases.


1997 ◽  
Vol 15 (2) ◽  
pp. 566-573 ◽  
Author(s):  
F Locatelli ◽  
C Niemeyer ◽  
E Angelucci ◽  
C Bender-Götze ◽  
S Burdach ◽  
...  

PURPOSE To evaluate the role of allogeneic bone marrow transplantation (BMT) in children with chronic myelomonocytic leukemia (CMML). PATIENTS AND METHODS Forty-three children with CMML given BMT and reported to the European Working Group on Myelodysplastic Syndrome in Childhood (EWOG-MDS) data base were evaluated. In 25 cases, the donor was a human leukocyte antigen (HLA)-identical or a one-antigen-disparate relative, in four cases a mismatched family donor, and in 14 a matched unrelated donor (MUD). Conditioning regimens consisted of total-body irradiation (TBI) and chemotherapy in 22 patients, whereas busulfan (Bu) with other cytotoxic drugs was used in the remaining patients. RESULTS Six of 43 patients (14%), five of whom received transplants from alternative donors, failed to engraft. There was a significant difference in the incidences of chronic graft-versus-host disease (GVHD) between children transplanted from compatible/one-antigen-mismatched relatives and from alternative donors (23% and 87%, respectively; P < .005). Probabilities of transplant-related mortality for children given BMT from HLA-identical/one-antigen-disparate relatives or from MUD/ mismatched relatives were 9% and 46%, respectively. The probability of relapse for the entire group was 58%, whereas the 5-year event-free survival (EFS) rate was 31%. The EFS rate for children given BMT from an HLA-identical sibling or one-antigen-disparate relative was 38%. In this latter group, patients who received Bu had a better EFS compared with those given TBI (62% v 11%, P < .01). CONCLUSION Children with CMML and an HLA-compatible relative should be transplanted as early as possible. Improvement of donor selection, GVHD prophylaxis, and supportive care are needed to ameliorate results of BMT from alternative donors.


Oncotarget ◽  
2017 ◽  
Vol 8 (61) ◽  
pp. 103274-103282 ◽  
Author(s):  
Kseniya Petrova-Drus ◽  
April Chiu ◽  
Elizabeth Margolskee ◽  
Sharon Barouk-Fox ◽  
Julia Geyer ◽  
...  

2003 ◽  
Vol 127 (9) ◽  
pp. 1214-1216
Author(s):  
Jason Hyde ◽  
Tsieh Sun

Abstract Chronic myelomonocytic leukemia with eosinophilia is a recently defined rare entity frequently associated with t(5;12)(q33;p13) translocation. It usually shows a peripheral eosinophil count greater than 1500/μL. However, the literature contains a small subset of cases in which the major manifestation is bone marrow eosinophilia. We report a case similar to that subset and discuss our finding that the immature eosinophils are identical to those seen in acute myelomonocytic leukemia with abnormal bone marrow eosinophils.


Blood ◽  
1962 ◽  
Vol 20 (4) ◽  
pp. 453-470 ◽  
Author(s):  
YALE RABINOWITZ ◽  
ROBERT SCHREK

Abstract 1. The study of living blood cells in slide chambers by phase microscopy and cinemicrography adds useful morphologic, developmental and physiologic data complementary to other methods for studying blood cells. 2. Normal monocytes were characterized by flattening in fresh serum with minimal ameboid motility, by ectoplasmic veils waving in the medium, and by transformation to fully developed macrophages in 5-7 days. 3. Cells from four patients with acute monocytic leukemia (Schilling type) showed only slight differences in morphology, development and function from normal monocytes in the slide chamber. 4. Monocytoid cells from four patients with myelomonocytic leukemia (Naegeli type), in the slide chamber, resembled those from two patients with leukemic reticuloendotheliosis in morphology, development and function, but differed from normal monocytes and cells of acute monocytic (Schilling) leukemia. The monocytoid cells were labeled "reticulum" cells with the implication that they were related to the primitive hematopoietic reticulum cell. 5. The "reticulum" cell in the slide chamber differed from the monocyte in having a coarse nuclear chromatin pattern with clumping of chromatin, larger numbers of varying sized cytoplasmic granules, less ectoplasm, development of characteristic star-shaped macrophage forms without ectoplasmic veils, and in exhibiting a considerable ameboid motility as opposed to the sluggish veil waving of the monocyte. 6. Speculation as to the significance of the "reticulum" cell has been presented with the suggestion that this cell precedes the blast cell in hematopoiesis. In Naegeli leukemia, unknown factors may cause alterations in the type of cell released from the marrow. There may also be shifts in emphasis between myeloblast and pre-myeloblast proliferation which become reflected in the cells appearing in the peripheral blood.


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