scholarly journals Acute Myeloid Leukemia Presenting as Myeloid Sarcoma with a Predisposition to the Gynecologic Tract

2019 ◽  
Vol 2019 ◽  
pp. 1-5
Author(s):  
Ryan M. Kahn ◽  
Sushmita Gordhandas ◽  
Eloise Chapman-Davis ◽  
Elizabeth Margolskee ◽  
Cathleen Matrai ◽  
...  

Myeloid sarcoma (MS) is a rare, extramedullary tumor consisting of immature white blood cells of myeloid lineage. MS is usually associated with the concurrent diagnosis of acute myeloid leukemia (AML) but can also present in the absence of bone marrow disease or at relapse of AML. MS of the gynecologic tract is exceedingly rare; however, it is hypothesized that it is likely more prevalent than previously understood given postmortem findings and persistence in preserved ovarian tissue. There is minimal literature surrounding MS and extramedullary relapse with no clear guidelines. This is a case report of a 48-year-old woman with MS involving the uterine corpus, fallopian tubes, and left ovary followed by a literature review. The overall aim is to review data regarding leukemic immune evasion and sanctuary sites in order to raise awareness as this represents an important and underrecognized hematologic malignancy in an often misdiagnosed, underrecognized site.

2020 ◽  
Vol 96 (5) ◽  
pp. 257-260
Author(s):  
Judit Noll ◽  
◽  
Dorottya Asbóth ◽  
Judit Csomor ◽  
Mónika Csóka ◽  
...  

Myeloid sarcoma according to the WHO 2016 version is an independent subgroup of acute myeloid leukemia, characterized by extramedullary tumor-like proliferation of myeloid precursor cells. Myeloid sarcoma can occur without bone marrow disease, associated with myeloid neoplasias or as a relapse of acute myeloid leukemia, too. In this article we describe the case of a 3 week-old newborn, whose myeloid sarcoma presented with skin symptoms and was successfully treated with AML BMF98 chemotherapy following allogeneic hematopoietic stem cell transplantation. Hereby we also summarize the most important current knowledges of the disease.


2019 ◽  
Vol 24 (1) ◽  
pp. 121-126 ◽  
Author(s):  
Shigeo Ishikawa ◽  
Yuichi Kato ◽  
Takanobu Kabasawa ◽  
Chiho Yoshioka ◽  
Kenichiro Kitabatake ◽  
...  

2017 ◽  
Vol 08 (03) ◽  
pp. 466-468 ◽  
Author(s):  
Geetha Narayanan ◽  
Lali V. Soman ◽  
Lakshmi Haridas ◽  
Harish Sugathan

ABSTRACTMyeloid sarcoma (MS) or chloroma is a rare extramedullary tumor composed of extramedullary proliferation of blasts of granulocytic, monocytic, erythroid, or megakaryocytic lineage occurring at sites outside the bone marrow. MS occurs in 2%–8% of patients with acute myeloid leukemia (AML), sometimes it occurs as the presenting manifestation of relapse in a patient in remission. We describe the case of a young male with AML in remission for 6 years presenting with central nervous system symptoms. Magnetic resonance imaging showed an extra-axial altered intensity lesion in the parasagittal parietal region, infiltrating anterosuperiorly into anterior falx, and posterosuperior aspect of the superior sagittal sinus. A biopsy from the lesion was diagnostic of MS which was positive for myeloperoxidase. He did not have any other sites of disease. He has received chemotherapy with FLAG (Fludarabine, Cytosine arabinoside) followed by cranial irradiation and is in complete remission.


2019 ◽  
Vol 18 (14) ◽  
pp. 1936-1951 ◽  
Author(s):  
Raghav Dogra ◽  
Rohit Bhatia ◽  
Ravi Shankar ◽  
Parveen Bansal ◽  
Ravindra K. Rawal

Background: Acute myeloid leukemia is the collective name for different types of leukemias of myeloid origin affecting blood and bone marrow. The overproduction of immature myeloblasts (white blood cells) is the characteristic feature of AML, thus flooding the bone marrow and reducing its capacity to produce normal blood cells. USFDA on August 1, 2017, approved a drug named Enasidenib formerly known as AG-221 which is being marketed under the name Idhifa to treat R/R AML with IDH2 mutation. The present review depicts the broad profile of enasidenib including various aspects of chemistry, preclinical, clinical studies, pharmacokinetics, mode of action and toxicity studies. Methods: Various reports and research articles have been referred to summarize different aspects related to chemistry and pharmacokinetics of enasidenib. Clinical data was collected from various recently published clinical reports including clinical trial outcomes. Result: The various findings of enasidenib revealed that it has been designed to allosterically inhibit mutated IDH2 to treat R/R AML patients. It has also presented good safety and efficacy profile along with 9.3 months overall survival rates of patients in which disease has relapsed. The drug is still under study either in combination or solely to treat hematological malignancies. Molecular modeling studies revealed that enasidenib binds to its target through hydrophobic interaction and hydrogen bonding inside the binding pocket. Enasidenib is found to be associated with certain adverse effects like elevated bilirubin level, diarrhea, differentiation syndrome, decreased potassium and calcium levels, etc. Conclusion: Enasidenib or AG-221was introduced by FDA as an anticancer agent which was developed as a first in class, a selective allosteric inhibitor of the tumor target i.e. IDH2 for Relapsed or Refractory AML. Phase 1/2 clinical trial of Enasidenib resulted in the overall survival rate of 40.3% with CR of 19.3%. Phase III trial on the Enasidenib is still under process along with another trial to test its potency against other cell lines. Edasidenib is associated with certain adverse effects, which can be reduced by investigators by designing its newer derivatives on the basis of SAR studies. Hence, it may come in the light as a potent lead entity for anticancer treatment in the coming years.


2007 ◽  
Vol 86 (10) ◽  
pp. 763-765 ◽  
Author(s):  
Sebastian Scholl ◽  
Joachim Lüftner ◽  
Lars-Olof Mügge ◽  
Volker Schmidt ◽  
Hans-Jörg Fricke ◽  
...  

2020 ◽  
Vol 154 (Supplement_1) ◽  
pp. S107-S108
Author(s):  
A C Reddy ◽  
K S Reddy

Abstract Introduction/Objective Histiocytic sarcoma (HS) is rare (<1% of hematolymphoid neoplasms), and can present extranodally as disseminated disease. Immunophenotypically, the cells express CD163, CD68, lysozyme and CD45. HS often occurs as a secondary event following B-cell lymphomas, acute lymphoblastic leukemia or acute myeloid leukemia (AML) typically with monocytic differentiation retaining the same molecular/cytogenetic abnormalities as the primary tumor. Results Our patient, a 47 year old male was diagnosed with myeloid sarcoma (MS) following FNA of a new neck mass. A bone marrow biopsy revealed AML without monocytic differentiation. Flow cytometric findings of both marrow and neck mass were similar (positive for CD34, CD117, CD33, CD11b, CD13, CD15, CD64, CD7; negative for CD4, CD14, CD56). Karyotypic and FLT3 ITD mutation analysis were normal. CNS involvement was diagnosed 2 months later, while a marrow biopsy (status post therapy) confirmed resolution of AML. A hypermetabolic left perinephric mass noted by PET CT, when biopsied, showed large epithelioid polygonal cells with amphophilic cytoplasm and atypical vesicular nuclei (positive for CD68, PU.1; negative for LCA, CD163, CD34, CD4, pankeratin). A diagnosis of atypical epithelioid neoplasm suggestive of HS was rendered, although negativity for LCA and CD163 was unusual. No treatment was given for HS. A month later, the patient presented with a cheek mass diagnosed again as being suggestive of HS. His AML also relapsed. Next-generation sequencing (37 genes including BRAF) from both marrow and tissue samples detected the presence of a nonsense mutation in exon 7 of WT1 (p.Ser169). Conclusion Our case appears to be the first reported one of disseminated HS preceded by MS and concomitant AML, lacking monocytic differentiation. The findings overall support the hypothesis of origin as being from a common progenitor cell differentiating along both myeloid and histiocytic/other cell lineages at different time points.


2021 ◽  
Vol 22 (13) ◽  
pp. 6857
Author(s):  
Samantha Bruno ◽  
Manuela Mancini ◽  
Sara De Santis ◽  
Cecilia Monaldi ◽  
Michele Cavo ◽  
...  

Acute myeloid leukemia (AML) is a hematologic malignancy caused by a wide range of alterations responsible for a high grade of heterogeneity among patients. Several studies have demonstrated that the hypoxic bone marrow microenvironment (BMM) plays a crucial role in AML pathogenesis and therapy response. This review article summarizes the current literature regarding the effects of the dynamic crosstalk between leukemic stem cells (LSCs) and hypoxic BMM. The interaction between LSCs and hypoxic BMM regulates fundamental cell fate decisions, including survival, self-renewal, and proliferation capacity as a consequence of genetic, transcriptional, and metabolic adaptation of LSCs mediated by hypoxia-inducible factors (HIFs). HIF-1α and some of their targets have been associated with poor prognosis in AML. It has been demonstrated that the hypoxic BMM creates a protective niche that mediates resistance to therapy. Therefore, we also highlight how hypoxia hallmarks might be targeted in the future to hit the leukemic population to improve AML patient outcomes.


2014 ◽  
Vol 2014 ◽  
pp. 1-4 ◽  
Author(s):  
Pankit Vachhani ◽  
Prithviraj Bose

Myeloid sarcoma represents the proliferation of myeloblasts of acute myeloid leukemia (AML) at extramedullary sites. While extramedullary involvement in AML is uncommon in itself, isolated myeloid sarcomas, that is, myeloid sarcomas without any bone marrow involvement, are extremely rare and pose a diagnostic and therapeutic challenge. Here, we present the case of a middle-aged woman with isolated myeloid sarcoma in the stomach—an organ seldom involved by this disease. Additionally, the literature on the epidemiology, diagnosis, pathology, prognosis, and therapeutic options in myeloid sarcomas has been reviewed.


2014 ◽  
Vol 53 (11) ◽  
pp. 1159-1164 ◽  
Author(s):  
Yusuke Isshiki ◽  
Chikako Ohwada ◽  
Emi Togasaki ◽  
Ryoh Shimizu ◽  
Nagisa Hasegawa ◽  
...  

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