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Cancers Aug 2023Hematological neoplasms sharing a blastic morphology may involve the skin. The skin may be either the primary site of occurrence of hematological malignancies with... (Review)
Review
Hematological neoplasms sharing a blastic morphology may involve the skin. The skin may be either the primary site of occurrence of hematological malignancies with blastic features or cutaneous lesions are the first manifestation of an underlying systemic malignancy. The assessment of skin biopsies of hematological neoplasms with blastic features poses diagnostic problems and requires expert hematopathologists considering a wide range of differential diagnoses. The precise diagnosis of diseases sharing blastic features but with different outcomes and requiring distinct therapies is essential for patient management. The present paper mainly focuses on cutaneous involvement of the blastoid variant of mantle cell lymphoma and lymphoblastic lymphoma of B-cell or T-cell origin. The relevant literature has been reviewed and the clinical aspects, pathological features, prognosis, and therapy of both blastoid mantle cell lymphoma and lymphoblastic lymphoma involving the skin are discussed. A focus on other hematological entities with blastic features, which may involve the skin, to be taken into consideration in differential diagnosis is also given.
PubMed: 37568745
DOI: 10.3390/cancers15153928 -
Cancer Immunology, Immunotherapy : CII Nov 2023STING is a pivotal mediator of effective innate and adaptive anti-tumor immunity; however, intratumoral administration of STING agonists have shown limited therapeutic...
STING is a pivotal mediator of effective innate and adaptive anti-tumor immunity; however, intratumoral administration of STING agonists have shown limited therapeutic benefit in clinical trials. The systemic effect of the intravenous delivery of STING agonists in cancer is not well-defined. Here, we demonstrated that systemic administration of STING agonist inhibited melanoma growth, improved inflammatory effector cell infiltration, and induced bone marrow mobilization and extramedullary hematopoiesis, causing widespread changes in immune components in the peripheral blood. The systemically administered STING agonist promoted HSC expansion and influenced lineage fate commitment, which was manifested as the differentiation of HSPCs was skewed toward myeloid cells at the expense of B-cell lymphopoiesis and erythropoiesis. Transcriptome analysis revealed upregulation of myeloid lineage differentiation-related and type I interferon-related genes. This myeloid-biased differentiation promoted the production and maturation of myeloid cells toward an activated phenotype. Furthermore, depletion of Gr-1 myeloid cells attenuated the anti-tumor immunity of STING agonist. Our findings reveal the anti-tumor mechanism of systemic administration of STING agonist that involves modulating HSPC differentiation and promoting myeloid cells maturation. Our study may help explain the limited clinical activity of STING agonists administered intratumorally.
Topics: Humans; Cell Differentiation; Bone Marrow; Hematopoietic Stem Cells; Myeloid Cells; Adaptive Immunity; Neoplasms
PubMed: 37550427
DOI: 10.1007/s00262-023-03502-7 -
Cureus Jul 2023Myeloid sarcoma, also known as granulocytic sarcoma or chloroma, is an extra-medullary accumulation of malignant myeloid blast cells, leading to a solid tumor formation....
Myeloid sarcoma, also known as granulocytic sarcoma or chloroma, is an extra-medullary accumulation of malignant myeloid blast cells, leading to a solid tumor formation. Herein, we report a rare presentation of a case with acute myeloid leukemia (AML), whose disease relapse was clinically evident as acute flaccid paraplegia with a certain sensory level. On thoracic spine magnetic resonance imaging (MRI), an epidural mass compressing the spinal cord at the level of the thoracic spine segment 4 (T4) was found. The mass histology confirmed the diagnosis of myeloid sarcoma.
PubMed: 37546146
DOI: 10.7759/cureus.41421 -
African Health Sciences Mar 2023Human herpesvirus 8 (HHV-8) has been linked to the development of Kaposi's sarcoma (KS)and multiple other hematologic malignant disorders. However, the role of HHV-8 in...
BACKGROUND
Human herpesvirus 8 (HHV-8) has been linked to the development of Kaposi's sarcoma (KS)and multiple other hematologic malignant disorders. However, the role of HHV-8 in acute leukemia patients is unknown.
OBJECTIVES
The objective of this study was to determine the prevalence of HHV-8 in Tunisian acute leukemia patients and in healthy blood donors.
METHODS
An indirect immunofluorescence test was used to detect the presence of anti-HHV8 antibodies. Nested PCR was used for the detection of HHV-8 DNAemia in samples of plasma.
RESULTS
The seroprevalence of HHV-8 was significantly higher in acute leukemia patients (21,4% ,15/70) than in healthy blood donors (7,1%, 5/70), (p= 0.02). Gender, type of disease, status of disease, prior blood transfusion, and outcome were not associated with HHV-8 seroprevalence. However, among acute leukemia patients, HHV-8 seroprevalence was statistically associated with older age > 40 years of age, (p=0.002). HHV-8 DNAemia was detected (1,4%) in only one patient of acute myeloid leukemia (AML) and none of the healthy blood donors.
CONCLUSIONS
The seroprevalence of HHV-8 infection in Tunisian adult acute leukemia patients was three times as high compared to healthy blood donors, suggesting that patients with acute leukemia might be at increased risk of HHV-8 infection.
Topics: Humans; Adult; Seroepidemiologic Studies; Antibodies, Viral; Sarcoma, Kaposi; Herpesviridae Infections; Herpesvirus 8, Human; Acquired Immunodeficiency Syndrome; Leukemia, Myeloid, Acute
PubMed: 37545962
DOI: 10.4314/ahs.v23i1.52 -
Cureus Jul 2023Myeloid sarcoma is rare and nasal chloroma is an uncommon initial manifestation of acute myeloid leukaemia. The correct diagnosis is a big challenge. In this report, we...
Myeloid sarcoma is rare and nasal chloroma is an uncommon initial manifestation of acute myeloid leukaemia. The correct diagnosis is a big challenge. In this report, we present a case of myeloid sarcoma of the nasal cavity with extension to the soft tissues of the face. A 53-year-old woman with a past medical history of thalassemia, not followed up, presented with a progressive greyish swelling in her right cheek associated with a nasal obstruction more marked on the right side and unilateral lacrimation. The diagnosis of myeloid sarcoma was based on histopathology and immunohistochemistry. Bone marrow aspiration testing revealed blasts that met the criteria for acute leukaemia. She received external radiotherapy at a total dose of 30 Gy in 15 fractions without systemic therapy, because she refused to get chemotherapy. She remained under surveillance and symptomatic treatment. The patient was examined four months after the end of the irradiation and showed a spectacular improvement in her clinical symptomatology with a clear decrease in nasal mass.
PubMed: 37533617
DOI: 10.7759/cureus.41273 -
Journal of Clinical Medicine Jul 2023Myeloid sarcoma (MS), also known as granulocytic sarcoma or chloroma, is a rare disease entity characterized by the emergence of an extramedullary tumor, which may be...
BACKGROUND
Myeloid sarcoma (MS), also known as granulocytic sarcoma or chloroma, is a rare disease entity characterized by the emergence of an extramedullary tumor, which may be antecedent, coexisting, or manifest secondarily to an ongoing malignancy of lymphoid origin. Owing to its low prevalence, scientific reports addressing this matter comprise mainly retrospective studies with a limited number of participants, rather low-quality research, and only few case reports. Despite MS's rarity, the need for enhancing their diagnostic tools and refinement of their therapeutic regimens is broadly recognized among physicians.
CASE SUMMARY
In this case series, we present the clinical histories of two patients diagnosed with MS. The former (Case 1) exhibited MS of the sternum alongside chronic myeloid leukemia (CML), while in case of the latter (Case 2) MS presented as the initial manifestation of a current acute myeloid leukemia (AML). Treatment for both patients included chemotherapy (CHTH) and radiation (RT); however, patient 1 with CML died due to cardiorespiratory insufficiency secondary to an infection, while patient 2 is in clinical remission (CR) for 16 months since their MS diagnosis. Furthermore, a comprehensive analysis of previously reported cases was conducted which incorporated MS in patients with AML and CML.
CONCLUSION
The objective of this report was to emphasize the heterogeneity among the clinical manifestations of MS, to underline the relevance of the histopathological and molecular diagnostic tools in opting for the appropriate therapy, and that, in spite of it occurring rather uncommonly, physicians should think of MS in the presence of pathological masses in patients under risk of hematological malignancies.
PubMed: 37510960
DOI: 10.3390/jcm12144845 -
Medicine Jul 2023Acute myeloid leukemia (AML)/myeloid sarcoma (MS) is risk-stratified based on cytogenetics. Although most congenital AML/MS have a dismal prognosis, certain genetic...
RATIONALE
Acute myeloid leukemia (AML)/myeloid sarcoma (MS) is risk-stratified based on cytogenetics. Although most congenital AML/MS have a dismal prognosis, certain genetic variants such as t (8, 16) [KAT6A::cAMP response element-binding protein (CREB) - binding protein fusion] and more recently t (8, 22) [KAT6A::EP300 fusion] have shown spontaneous remissions. KAT6A located on chromosome 8p11 encodes KAT6A protein, a histone/lysine acetyltransferase enzyme. Numerous partner genes associated with KAT6A include cAMP response element-binding protein (CREB) - binding protein (16p13), EP300 (22q13), LEUTX (9q13), NCOA2, NCOA3, and ASXL2.
PATIENT CONCERNS
In this article, we describe an otherwise healthy infant who presented with skin nodules on the face and scalp without any systemic or CNS involvement. A biopsy of the cutaneous lesion was consistent with congenital MS.
DIAGNOSES
Through molecular testing, we found that our patient had the KAT6A::EP300 mutation. This is one of the rare recurrent cytogenetic abnormalities that are linked to congenital AML.
INTERVENTION
Our patient underwent spontaneous remission with watchful waiting.
OUTCOME
Our patient has remained in spontaneous remission for 24 months.
LESSONS
Even though the KAT6A::EP300 mutation in adults is a poor prognostic marker, a similar mutation in congenital AML has a higher likelihood of spontaneous remission. Hence, conservative management might be an initial management strategy for clinically stable patients.
Topics: Humans; Infant; Biomarkers; Chromosome Aberrations; Cyclic AMP Response Element-Binding Protein; E1A-Associated p300 Protein; Histone Acetyltransferases; Leukemia, Myeloid, Acute; Remission, Spontaneous; Sarcoma, Myeloid
PubMed: 37505185
DOI: 10.1097/MD.0000000000034258 -
World Journal of Surgical Oncology Jul 2023Pancreatic follicular dendritic cell sarcoma (FDCS) is an exceptionally rare and low-to-moderate malignancy, with only seven reported cases to date. Clinical diagnosis...
INTRODUCTION
Pancreatic follicular dendritic cell sarcoma (FDCS) is an exceptionally rare and low-to-moderate malignancy, with only seven reported cases to date. Clinical diagnosis of FDCS is challenging due to the lack of distinct biological and radiographic features.
CASE PRESENTATION
A 67-year-old woman presented to the hospital with a 4-day history of severe abdominal pain. Imaging studies (CT and MRI) revealed a large cystic mass located at the tail of the pancreas, which was suspected to be myeloid sarcoma (MS) based on EUS and CT-guided pancreatic puncture. Postoperative pathology and immunohistochemistry confirmed the diagnosis of pancreatic FDCS. After the diagnosis was confirmed, the patient received postoperative chemotherapy with the CHOP regimen. At 11 months of follow-up, there was no evidence of recurrence. Seven published cases have been reviewed to comprehensively summarize the clinical characteristics, diagnosis, and treatment options of FDCS.
CONCLUSION
While imaging can be useful in detecting pancreatic FDCS, it should be interpreted with caution as it can be challenging to differentiate from other pancreatic tumors. Pathology and immunohistochemistry are considered the gold standard for diagnosis, with CD21, CD23, and CD35 being specific tumor cell markers. However, preoperative diagnosis of pancreatic FDCS remains difficult, and the pancreatic puncture may further increase the risk of misdiagnosis. The disease is highly prone to recurrence and metastasis, and surgery is the preferred method for both diagnosis and treatment of localized disease.
Topics: Female; Humans; Aged; Dendritic Cell Sarcoma, Follicular; Pancreas; Pancreatic Neoplasms; Abdominal Pain; Biomarkers, Tumor
PubMed: 37480085
DOI: 10.1186/s12957-023-03115-5