A Rare Case of Hemophagocytic Lymphohistiocytosis Associated With Myelodysplastic Syndrome and Trisomy 8 in a Pediatric Patient

2019 ◽  
Vol 41 (1) ◽  
pp. e57-e59
Author(s):  
Seh Hyun Kim ◽  
Dae Yong Yi ◽  
Na Mi Lee ◽  
Sin Weon Yun ◽  
Soo Ahn Chae ◽  
...  
HemaSphere ◽  
2021 ◽  
Vol 5 (2) ◽  
pp. e517
Author(s):  
Mehdi Hage-Sleiman ◽  
Jaja Zhu ◽  
Hippolyte Guérineau ◽  
Emily Ronez ◽  
Katayoun Jondeau ◽  
...  

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

2018 ◽  
Vol 19 (10) ◽  
pp. 3269 ◽  
Author(s):  
Simone Feurstein ◽  
Kathrin Thomay ◽  
Winfried Hofmann ◽  
Guntram Buesche ◽  
Hans Kreipe ◽  
...  

Myelodysplastic syndrome (MDS) can easily transform into acute myeloid leukemia (AML), a process which is often associated with clonal evolution and development of complex karyotypes. Deletion of 5q (del(5q)) is the most frequent aberration in complex karyotypes. This prompted us to analyze clonal evolution in MDS patients with del(5q). There were 1684 patients with low and intermediate-risk MDS and del(5q) with or without one additional cytogenetic abnormality, who were investigated cytogenetically in our department, involving standard karyotyping, fluorescence in situ hybridization (FISH) and multicolor FISH. We identified 134 patients (8%) with aspects of clonal evolution. There are two main routes of cytogenetic clonal evolution: a stepwise accumulation of cytogenetic events over time and a catastrophic event, which we defined as the occurrence of two or more aberrations present at the same time, leading to a sudden development of highly complex clones. Of the 134 patients, 61% underwent a stepwise accumulation of events whereas 39% displayed a catastrophic event. Patients with isolated del(5q) showed significantly more often a stepwise accumulation of events rather than a catastrophic event. The most frequent aberrations in the group of stepwise accumulation were trisomy 8 and trisomy 21 which were significantly more frequent in this group compared to the catastrophic event group. In the group with catastrophic events, del(7q)/-7 and del(17p)/-17 were the most common aberrations. A loss of 17p, containing the tumor suppressor gene TP53, was found significantly more frequent in this group compared to the group of stepwise accumulation. This leads to the assumption that the loss of TP53 is the driving force in patients with del(5q) who undergo a sudden catastrophic event and evolve into complex karyotypes.


2018 ◽  
Vol 39 (1) ◽  
pp. 109-113 ◽  
Author(s):  
Danielle McDonald ◽  
Richard Sultan ◽  
Anusha Viswanathan ◽  
Anita Siu

Cureus ◽  
2020 ◽  
Author(s):  
Theano Lagousi ◽  
Paraskevi Korovessi ◽  
Eleni Panagouli ◽  
Vasilis Tsagris ◽  
Stavroula Kostaridou

2021 ◽  
Author(s):  
Mostafa Paridar ◽  
Kazem Zibara ◽  
Seyed Esmaeil Ahmadi ◽  
Abbas Khosravi ◽  
Maral Soleymani ◽  
...  

Abstract Background Myelodysplastic syndrome (MDS), a heterogeneous group of hematopoietic malignancy, has been shown to present different cytogenetic abnormalities, risk factors, and clinico-hematological features in different populations and geographic areas. Herein, we determined the cytogenetic spectrum and clinico-hematological features of Iranian MDS patients for the first time. Methods This prospective cross-sectional study was conducted on 103 patients with MDS in Ahvaz, southwest of Iran, from 2014 to 2018. Clinical presentations, complete blood counts (CBC), and bone marrow (BM) biopsy samples were assessed. Perls' staining was used to evaluate BM iron storage. The cytogenetic evaluation was performed using the conventional G banding method on the BM. Results Patients’ median age was 62.3 (ranged from 50–76), and the majority were male (72.8%). The most common clinical symptom at the time of admission was fatigue (n = 33) followed by pallor (n = 27). The most common subgroup was MDS-Multi Lineage Dysplasia (MDS-MLD) (n = 38, 36.8%), followed by MDS-Single Lineage Dysplasia (MDS-SLD) (n = 28, 18.4%). A normal karyotype was observed in 59 patients (57.3%), while 44 patients (42.7%) had cytogenetic abnormalities. Trisomy 8 (+ 8) was the most common cytogenetic abnormality (n = 14) followed by dell 17p (n = 9) and monosomy 7 (-7) (n = 7). Twelve patients (11.65 %) were transformed to AML. Conclusion Our data betokened that among our MDS patients, Trisomy 8 is the predominant cytogenetic abnormality, and MDS-MLD and MDS-SLD are the most common of subtypes. Noteworthy, the male: female ratio was slightly higher in Iran than in previous reports from other parts of the world. Our study is the first report of the clinical, hematological, and cytogenetic spectrum of MDS patients in Iran


Rheumatology ◽  
2011 ◽  
Vol 50 (7) ◽  
pp. 1342-1344 ◽  
Author(s):  
T. Shigemura ◽  
K. Agematsu ◽  
T. Yamazaki ◽  
K. Sakashita ◽  
Y. Nakayama ◽  
...  

2016 ◽  
Vol 44 (12) ◽  
pp. 505-505
Author(s):  
Arvind Kalyan Sundaram ◽  
Mary Reed

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