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Asian Journal of Surgery Mar 2024
Topics: Male; Humans; Sarcoma, Myeloid; Prostate; Sarcoma; Soft Tissue Neoplasms; Pelvis
PubMed: 38161133
DOI: 10.1016/j.asjsur.2023.12.076 -
Cell Reports. Medicine Dec 2023Chronic myelomonocytic leukemia (CMML) is frequently associated with mutations in the rat sarcoma gene (RAS), leading to worse prognosis. RAS mutations result in active...
Chronic myelomonocytic leukemia (CMML) is frequently associated with mutations in the rat sarcoma gene (RAS), leading to worse prognosis. RAS mutations result in active RAS-GTP proteins, favoring myeloid cell proliferation and survival and inducing the NLRP3 inflammasome together with the apoptosis-associated speck-like protein containing a caspase recruitment domain (ASC), which promote caspase-1 activation and interleukin (IL)-1β release. Here, we report, in a cohort of CMML patients with mutations in KRAS, a constitutive activation of the NLRP3 inflammasome in monocytes, evidenced by ASC oligomerization and IL-1β release, as well as a specific inflammatory cytokine signature. Treatment of a CMML patient with a KRAS mutation using the IL-1 receptor blocker anakinra inhibits NLRP3 inflammasome activation, reduces monocyte count, and improves the patient's clinical status, enabling a stem cell transplant. This reveals a basal inflammasome activation in RAS-mutated CMML patients and suggests potential therapeutic applications of NLRP3 and IL-1 blockers.
Topics: Humans; Inflammasomes; NLR Family, Pyrin Domain-Containing 3 Protein; Proto-Oncogene Proteins p21(ras); Leukemia, Myelomonocytic, Chronic; Symptom Burden; Interleukin-1
PubMed: 38118408
DOI: 10.1016/j.xcrm.2023.101329 -
ACG Case Reports Journal Dec 2023A female patient in her mid-70s, with a history of diverticulosis, presented with a 2-month history of severe diarrhea, left lower quadrant abdominal pain, decreased...
A female patient in her mid-70s, with a history of diverticulosis, presented with a 2-month history of severe diarrhea, left lower quadrant abdominal pain, decreased appetite, and fever. She was treated for diverticulitis, but did not improve. Subsequent workup revealed leukocytosis and circulating myeloblasts on a peripheral blood smear. Bone marrow evaluation and flow cytometry confirmed the diagnosis of acute myeloid leukemia. Abdominal computed tomography and sigmoidoscopy were performed for her persistent diarrhea. While both failed to show an obvious mass or anatomical abnormality, pathology from the colorectum showed infiltration by leukemic cells consistent with myeloid sarcoma. The diarrhea improved with acute myeloid leukemia chemotherapy.
PubMed: 38089536
DOI: 10.14309/crj.0000000000001213 -
Indian Journal of Pathology &... 2023Biliary obstruction secondary to malignancy is a common clinical problem. Rarely, biliary obstruction is due to leukemia, and obstructive jaundice in these patients...
Biliary obstruction secondary to malignancy is a common clinical problem. Rarely, biliary obstruction is due to leukemia, and obstructive jaundice in these patients usually presents late in the course of the disease. We present a rare case of a patient who presented with fever, jaundice, and pruritus with multiple nodular swellings in the left shoulder, left thigh, and lower back. Magnetic resonance cholangiopancreatography (MRCP) revealed periampullary mass lesion causing dilated common bile duct (CBD) and intrahepatic bile ducts; hence, endoscopic retrograde cholangiography with plastic stenting was done. Biopsy from the shoulder lesion revealed a mesenchymal tumor, and immunohistochemistry (IHC) confirmed the lesion as myeloid sarcoma. Myeloid sarcoma is an extramedullary tumor, a subtype of acute myeloid leukemia, and presentation as biliary lesions with multiple anatomical sites is very rare. The patient was started on chemotherapy after the normalization of bilirubin. The patient showed improvement of skin lesions and normalization of liver function test (LFT) after 3 weeks of chemotherapy.
Topics: Humans; Jaundice, Obstructive; Sarcoma, Myeloid; Cholestasis; Bile Ducts, Intrahepatic; Common Bile Duct
PubMed: 38084550
DOI: 10.4103/ijpm.ijpm_1108_21 -
International Journal of... Oct 2023Myeloid sarcoma (MS) or chloroma is a localized mass composed of blastic cells of granulocytic lineage. It is a subtype of acute myeloid leukemia and usually presents as...
Myeloid sarcoma (MS) or chloroma is a localized mass composed of blastic cells of granulocytic lineage. It is a subtype of acute myeloid leukemia and usually presents as a complication of acute myeloid leukemia, myeloid dysplastic syndrome, or myeloproliferative disorder. MS occurs in 2.5-9.1% of patients with AML, precedes the clinical disease, coincidence with the onset or at relapse and in rare conditions, it can occur with no evidence of hematologic disorders. Here, we presented seven cases of MS in unusual locations or with rare presentations at presentation or relapse. We concluded that MS should be considered in the differential diagnosis of any high-grade tumor, especially in a patient with previous history of any myeloid neoplasm.
PubMed: 38076783
DOI: 10.18502/ijhoscr.v17i4.13922 -
Cureus Oct 2023Myeloid sarcoma (MS) is the occurrence of primitive granulocytic precursors in various extramedullary sites. It can occur as an isolated disease, present concomitantly,...
Myeloid sarcoma (MS) is the occurrence of primitive granulocytic precursors in various extramedullary sites. It can occur as an isolated disease, present concomitantly, or during the relapse of various myeloid neoplasms. A high index of clinical suspicion is warranted owing to its varied clinical presentation, rarity of diagnosis, inadequate immunohistochemical techniques, and challenging treatment. The occurrence of myeloid sarcoma of the testis, either as an independent entity or as an initial presentation of acute myeloid leukemia (AML), is exceedingly uncommon, with only a few documented cases in the literature. In this case study, we present a patient who initially presented with testicular swelling, later identified as MS, and subsequently diagnosed as AML through a bone marrow aspirate. This report discusses the diagnostic difficulties encountered and the available therapeutic options for managing MS.
PubMed: 38022116
DOI: 10.7759/cureus.47715 -
Annals of Hematology Feb 2024We present the case of a 58-year-old female patient who presented with an extramedullary B-ALL relapse after prior allogenic HSCT and blinatumomab therapy. The patient...
Liver failure after treatment with inotuzumab and polychemotherapy including PEG-asparaginase in a patient with relapsed Philadelphia chromosome-negative acute lymphoblastic leukemia.
We present the case of a 58-year-old female patient who presented with an extramedullary B-ALL relapse after prior allogenic HSCT and blinatumomab therapy. The patient died from complications of a drug-induced acute liver failure after a salvage therapy combining inotuzumab ozogamicin (InO)-based induction followed by consolidation with high dose MTX and pegaspargase based on the GMALL protocol for older ALL patients. After a diagnosis of the extramedullary relapse in the form of a retro vesical chloroma, the patient received an individualized multi-agent chemotherapy based on induction chemotherapy for older patients in combination with InO. After four administrations of InO, in combination with vincristine, dexamethasone, cytarabine, and cyclophosphamide, CT-imaging showed a reduction in volume of the chloroma and response to therapy. Consolidation with high-dose methotrexate and pegaspargase was administered. The patient developed toxic liver damage manifested by hyperbilirubinemia and progressive hepatic encephalopathy. The diagnostic criteria for VOD were met, and therapy with defibrotide was initiated. Liver biopsy revealed no histological signs of VOD but instead steatohepatitis indicative of drug-induced toxicity. The patient ultimately died of hemorrhagic shock through postinterventional hemorrhage after liver biopsy. In conclusion, although InO shows promising results in the therapy of r/r ALL with and without additional chemotherapy, the combination with MTX and pegaspargase in an intensively pretreated patient with relapse after HCST may impart an increased risk for liver-related toxicity. Special caution is required when assessing fitness for further liver toxic regimens. A key takeaway is also the reminder that InO can cause liver damage not only in the form of VOD but also through direct hepatocellular toxicity.
Topics: Female; Humans; Middle Aged; Philadelphia Chromosome; Sarcoma, Myeloid; Precursor Cell Lymphoblastic Leukemia-Lymphoma; Inotuzumab Ozogamicin; Antineoplastic Combined Chemotherapy Protocols; Liver Failure; Recurrence; Asparaginase; Polyethylene Glycols
PubMed: 37999763
DOI: 10.1007/s00277-023-05495-w -
Frontiers in Oncology 2023fusion is a recurrent event commonly observed in adult male patients diagnosed with T-cell acute lymphoblastic leukemia (T-ALL) and has occasionally been reported in... (Review)
Review
fusion is a recurrent event commonly observed in adult male patients diagnosed with T-cell acute lymphoblastic leukemia (T-ALL) and has occasionally been reported in other diseases such as acute myeloid leukemia (AML), myeloid sarcoma (MS), acute undifferentiated leukemia (AUL), chronic myeloid leukemia (CML) and B-cell acute lymphoblastic leukemia (B-ALL). This fusion gene is derived from chromosome del(9)(q34.11;q34.13) or t(9;9)(q34;q34) and may have an inhibitory effect on primitive progenitor differentiation. The prognosis of the reported patients is varied, with these patients often show resistance to chemotherapy regimens that include high doses of glucocorticoids. The optional treatment has not been determined, more cases need to be accumulated and evaluated. The scope of this review is to summarize the general features and prognostic significance in leukemia associated with the fusion gene and to discuss the methods of detection and treatment, aiming at providing some useful references for relevant researchers in the field of blood tumor.
PubMed: 37909026
DOI: 10.3389/fonc.2023.1269531 -
Life Science Alliance Dec 2023The tumor microenvironment is a dynamic network of stromal, cancer, and immune cells that interact and compete for resources. We have previously identified the Vanin1...
The tumor microenvironment is a dynamic network of stromal, cancer, and immune cells that interact and compete for resources. We have previously identified the Vanin1 pathway as a tumor suppressor of sarcoma development via vitamin B5 and coenzyme A regeneration. Using an aggressive sarcoma cell line that lacks Vnn1 expression, we showed that the administration of pantethine, a vitamin B5 precursor, attenuates tumor growth in immunocompetent but not nude mice. Pantethine boosts antitumor immunity, including the polarization of myeloid and dendritic cells towards enhanced IFNγ-driven antigen presentation pathways and improved the development of hypermetabolic effector CD8 T cells endowed with potential antitumor activity. At later stages of treatment, the effect of pantethine was limited by the development of immune cell exhaustion. Nevertheless, its activity was comparable with that of anti-PD1 treatment in sensitive tumors. In humans, expression correlates with improved survival and immune cell infiltration in soft-tissue sarcomas, but not in osteosarcomas. Pantethine could be a potential therapeutic immunoadjuvant for the development of antitumor immunity.
Topics: Humans; Mice; Animals; CD8-Positive T-Lymphocytes; Coenzyme A; Pantothenic Acid; Sarcoma; Tumor Microenvironment
PubMed: 37833072
DOI: 10.26508/lsa.202302200 -
Frontiers in Oncology 2023Myeloid sarcoma (MS) is a rare extramedullary parenchymal tumor composed of immature myeloid cells, occurring mainly in the lymph nodes, skin, soft tissue, testicles,...
Myeloid sarcoma (MS) is a rare extramedullary parenchymal tumor composed of immature myeloid cells, occurring mainly in the lymph nodes, skin, soft tissue, testicles, bones, peritoneum, and gastrointestinal tract, and rarely in the pancreas. Herein, we report the case of a 68-year-old female patient who visited our hospital for medical assistance due to acute abdominal pain. Abdominal computed tomography (CT) and magnetic resonance imaging showed a mass approximately 8 cm in diameter in the pancreatic tail, which was suspected to be a malignant tumor. To further assess the presence of distant metastases, the patient underwent fluorine-18-fluorodeoxyglucose positron emission tomography (F-FDG PET)/CT, which revealed an increased F-FDG uptake in the corresponding lesions. Subsequently, the patient underwent surgical treatment, and postoperative pathology and immunohistochemistry revealed that the mass was MS. Moreover, we reviewed the clinical features, imaging findings, and histopathology of pathologically confirmed pancreatic MS in the published literature.
PubMed: 37829333
DOI: 10.3389/fonc.2023.1259236