scholarly journals A Case of Myeloid Sarcoma in the Nasal Cavity Occurred in the Patient with Leukemic Transformation in Myelodysplastic Syndrome

Author(s):  
Dong Hoo Lee ◽  
Sung Yool Park ◽  
Ha Young Park ◽  
Seong Kook Park

Myeloid sarcoma is characterized by the presence of myeloid blasts at an extramedullar site that disrupts the normal architecture of the organ. Many of these cases are associated with acute myeloid leukemia, chronic myeloid leukemia, myelodysplastic syndrome, or occur in de novo. It occurs most commonly in skin, lymph node, gastrointestinal tract, bone, soft tissue but, rarely in head and neck; especially in nasal cavity. Therefore, it is often misdiagnosed as a different disease: most commonly as lymphoma. Here we report a rare case of myeloid sarcoma in the nasal cavity that occurred in a patient with leukemic transformation with myelodysplastic syndrome, provided with literature review.

2006 ◽  
Vol 169 (2) ◽  
pp. 181-183 ◽  
Author(s):  
Ronald Feitosa Pinheiro ◽  
Maria de Lourdes L.F. Chauffaille ◽  
Maria Regina Régis Silva

2018 ◽  
Vol 2018 ◽  
pp. 1-5 ◽  
Author(s):  
T. Couture ◽  
K. Amato ◽  
A. DiAdamo ◽  
P. Li

Jumping translocations of 1q refer to the break-off of chromosome 1q as a donor fusing to two or more recipient chromosomes. We detected jumping translocations of 1q in three patients with initial diagnosis of myelodysplastic syndrome (MDS) and later progression to acute myeloid leukemia (AML). Review of literature found jumping translocations of 1q in 30 reported cases of MDS and AML. The cytogenetic findings from these 33 cases showed that seven cases had a stemline clone and 26 cases had de novo jumping translocations of 1q in which 5% of cell lineages had additional structural rearrangements. In 75% of cases, the 1q donor jumped to the short arm of recipient acrocentric chromosomes. Approximately 82% of the fusions occurred in the telomeric regions of short and long arms and 18% occurred in the pericentric or interstitial regions of recipient chromosomes. Hypomethylation of the donor 1q pericentromeric region and shortened telomeres in recipient chromosomes were associated with the formation of jumping translocations. Jumping translocations of 1q as an indication of chromosomal instability pose high risk for progression of MDS to AML and a poor prognosis. Further understanding of underlying genomic defects and their clinical significance will improve overall treatment and patient care.


Diagnostics ◽  
2019 ◽  
Vol 9 (3) ◽  
pp. 84
Author(s):  
Carla Minoia ◽  
Vincenza de Fazio ◽  
Giovanni Scognamillo ◽  
Anna Scattone ◽  
Nicola Maggialetti ◽  
...  

Myeloid sarcoma (MS) represents a rare disease with an adverse clinical outcome for patients not candidate to acute myeloid leukemia (AML)-like chemotherapies. Here we present the case of an elderly patient affected by a bilateral breast localization of MS treated with the hypomethylating agent decitabine associated to radiotherapy. The association of the two treatment modalities has allowed an optimal and long-lasting disease control.


Blood ◽  
2002 ◽  
Vol 100 (2) ◽  
pp. 427-434 ◽  
Author(s):  
Dorothy R. Barnard ◽  
Beverley Lange ◽  
Todd A. Alonzo ◽  
Jonathan Buckley ◽  
J. Nathan Kobrinsky ◽  
...  

Abstract There has not been a reported series of children with therapy-induced myelodysplastic syndrome/acute myeloid leukemia (tMDS/tAML) who were treated systematically. This paper describes 24 children with tMDS/tAML who were assigned randomly to standard- or intensive-timing induction on protocol CCG 2891. Presenting features and outcomes of those children were compared with those of 960 patients with de novo MDS (62 patients) or AML (898 patients). Children with tMDS/tAML were older at presentation (P = .015), had lower white blood cell counts (P = .01), and were more likely to have MDS (21% vs 7%) (P = .02) and trisomy 8 (P = .06). Fewer had hepatomegaly (P = .02), splenomegaly (P = .03), hepatosplenomegaly (P = .02), or classic AML translocations [t(8;21), t(15;17), 16q22; P = .02]. They had a poorer induction rate (50% vs 72%,P = .016), overall survival (26% vs 47% at 3 years,P = .007), and event-free survival (21% vs 39% at 3 years, P =.023). Disease-free survival after achieving remission was similar (45% vs 53%, P = .868). Children with tMDS/tAML who received intensive-timing induction had better outcomes than those who received standard-timing induction (overall survival 32% vs 0%, P = .54). In this study, the latency period to development of tMDS/tAML was the same for presumed alkylator-induced as for topoisomerase-induced myeloid leukemia. The findings of this study confirm that most children with tMDS/tAML have disease resistant to current therapies. Standard-timing induction appears less effective for this population.


2021 ◽  
Vol 15 (1) ◽  
Author(s):  
Bahaa Razem ◽  
Mohamed Raiteb ◽  
Sanaa El Mrini ◽  
Faiçal Slimani

Abstract Background Myeloid sarcoma is a solid tumor that consists of immature myeloid cells occurring at an extramedullary site. It can present before, concurrent with, or after the diagnosis of acute myeloid leukemia or other myeloproliferative diseases, and a proportion of patients never develop bone marrow infiltration. Only a few isolated cases of pediatric orbital myeloid sarcoma have been reported, and they are often associated with a high misdiagnosis rate. Case report We report a rare case of pediatric orbital myeloid sarcoma associated with blunt trauma in a 3-year-old Caucasian male patient, which was clinically and radiologically misdiagnosed for orbital subperiostal hematoma. The patient underwent a surgical intervention to drain the hematoma when an orbital mass was found. The microscopic, immunologic, and genetic features of the tumor and the myelogram were in favor of LAM2, and the patient was started with chemotherapy with a favorable evolution within 18 months follow-up. Conclusion Orbital myeloid sarcoma usually exhibits clinical and radiological features that can be easily misleading, especially if it happens de novo or as the first manifestation of acute myeloid leukemia. Only a few isolated cases have reported and proposed trauma as a trigger event of the onset of this type of tumor proliferation, but further investigations and evidence are needed to support this hypothesis.


Amino Acids ◽  
2010 ◽  
Vol 40 (3) ◽  
pp. 943-951 ◽  
Author(s):  
Maria Braoudaki ◽  
Fotini Tzortzatou-Stathopoulou ◽  
Athanasios K. Anagnostopoulos ◽  
Chrisa Papathanassiou ◽  
Konstantinos Vougas ◽  
...  

2021 ◽  
Vol 9 (1) ◽  
Author(s):  
Minghua Hong ◽  
Junqing Wu ◽  
Lifeng Ma ◽  
Xiaoping Han ◽  
Ting Lu ◽  
...  

AbstractMyelodysplastic syndrome with myelofibrosis (MDS-MF) has been associated with an inferior prognosis compared with MDS without MF. However, MDS-MF is not listed independently as a subtype of MDS, and its clinical and genetic characteristics remain poorly understood. We retrospectively compared 53 patients with MDS-MF (44 MF grade 1/MF1; 9 MF grade 2–3/MF2 − 3) and 31 with de novo MDS without MF (MDS). The leukemic transformation risks of both MDS-MF2 − 3 and MDS-MF1 were increased compared with the MDS group. To identify the potential mechanisms responsible for the leukemic transformation of MDS-MF, we performed single-cell sequencing for one MDS-MF2 − 3 patient before and after leukemic transformation to explore the variations in gene expression levels. In addition to upgraded expression levels of acute myeloid leukemia-related genes during leukemic transformation, expression levels of some inflammation-related genes (such as S100s, RNASE3, and CYBB) were also increased, and inflammation-related pathways were up-regulated. These results suggest that inflammation-related genes and pathways may play an important role in the leukemic transformation of MDS-MF.


Blood ◽  
2008 ◽  
Vol 112 (11) ◽  
pp. 5068-5068
Author(s):  
Dolly G Aguilera ◽  
Christos Vaklavas ◽  
Apostolia-Maria Tsimberidou ◽  
Sijin Wen ◽  
L. Jeffrey Medeiros ◽  
...  

Abstract Background: Therapy-related myelodysplastic syndrome/acute myeloid leukemia (t-MDS/AML) is a long-term complication of pediatric cancer and it carries a poor prognosis. Patients and Methods: We retrospectively studied pediatric t-MDS/AML patients treated at M. D. Anderson from 1975 to 2007. We also compared these patients to pediatric patients with de novo MDS/AML during this time interval. Results: Among 2589 children with cancer treated at M. D. Anderson, we identified 22 (0.85%) patients with t-MDS/AML and 141 (5.4%) patients with de novo MDS/AML. Patients with t-MDS/AML had a median age of 14 years (range, 3–20). There was a male and Hispanic predominance. The most common primary malignancies were osteosarcoma and Hodgkin lymphoma. The median latency period was 4.1 years. Fourteen patients received AML-type chemotherapy, 5 underwent allogeneic stem cell transplantation (SCT) as induction therapy, and 3 received supportive care. Fourteen patients underwent SCT as induction (n=5), post-remission (n=5), or salvage therapy (n=4). Their respective 2-year survival rates were 20%, 40%, and 25% (p= 0.85). Patients with de novo AML were younger (p=0.001), and had higher rates of CR (p=0.03), and survival (p<0.0001). Independent factors predicting shorter survival were poor/intermediate-risk cytogenetics (p=0.01), lower hemoglobin level (p=0.0001), and t-MDS/AML (vs. de novo) (p=0.003). Conclusion: Childhood t-MDS/AML has a poor prognosis. Although patients benefited from AML-type induction chemotherapy followed by SCT as post-remission therapy, effective therapies are needed.


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