scholarly journals Myeloid Sarcoma of the Bladder in a Patient with Chronic myelomonocytic Leukaemia

2022 ◽  
Vol 4 (3) ◽  
pp. e36-e43
Author(s):  
Rebecca Smith ◽  
Bashir Mohamed ◽  
Jeremy Nettleton
Keyword(s):  
De Novo ◽  
English Literature ◽  
Bladder Tumour ◽  
Myeloproliferative Neoplasm ◽  
Myeloid Sarcoma ◽  
Cell Transplant ◽  
Ureteric Stenting ◽  
Show Evidence ◽  
The Right ◽  
Chronic Myelomonocytic Leukaemia

BackgroundMyeloid sarcoma is a rare extramedullary tumour of immature granulocytes, most commonly involving the skin, bone, lymph nodes, and soft tissue. It is usually associated with a diagnosis of relapsed or de novo acute myeloid leukaemia, acute lymphoblastic transformation of a myelodysplastic/myeloproliferative neoplasm, or can occur as isolated myeloid sarcoma.Case reportA 66-year-old female with a 7-year history of stable chronic myelomonocytic leukaemia presents with urgency, frequency, dysuria symptoms, and without new constitutional symptoms. She is found to have atypical, multifocal lesions on the right posterolateral wall of the bladder with associated hydronephrosis. Pathology reveals the diagnosis as myeloid sarcoma; surprisingly, bone marrow evaluation does not show evidence of acute leukaemic transformation.ConclusionsMyeloid sarcoma occurring in patients with chronic myelomonocytic leukaemia is extremely rare, and there are no cases reported in the English literature of these patients developing lesions in the bladder. The urological manifestations of an underlying haematological malignancy are best managed with a combination of systemic chemotherapy and allogeneic stem cell transplant, and in this case, the only surgical intervention required was ureteric stenting and tissue biopsy. Although rare, it is essential to consider alternative diagnoses when confronted with an atypical bladder tumour; failure to do so may result in patient harm by exposure to unnecessary intervention and delay to potentially curative treatment.

scholarly journals Leukemia secondary to myeloproliferative neoplasms

Blood ◽  
2020 ◽  
Vol 136 (1) ◽  
pp. 61-70 ◽  
Author(s):  
Andrew J. Dunbar ◽  
Raajit K. Rampal ◽  
Ross Levine
Keyword(s):  
Cancer Genomics ◽  
De Novo ◽  
Myeloproliferative Neoplasms ◽  
Myeloproliferative Neoplasm ◽  
Unmet Need ◽  
Chemotherapeutic Agents ◽  
Cell Transplant ◽  
Leukemic Transformation ◽  
Long Term Outcome ◽  
Molecular Features

Abstract Secondary acute myeloid leukemias (AMLs) evolving from an antecedent myeloproliferative neoplasm (MPN) are characterized by a unique set of cytogenetic and molecular features distinct from de novo AML. Given the high frequency of poor-risk cytogenetic and molecular features, malignant clones are frequently insensitive to traditional AML chemotherapeutic agents. Allogeneic stem cell transplant, the only treatment modality shown to have any beneficial long-term outcome, is often not possible given the advanced age of patients at time of diagnosis and frequent presence of competing comorbidities. Even in this setting, relapse rates remain high. As a result, outcomes are generally poor and there remains a significant unmet need for novel therapeutic strategies. Although advances in cancer genomics have dramatically enhanced our understanding of the molecular events governing clonal evolution in MPNs, the cell-intrinsic and -extrinsic mechanisms driving leukemic transformation at this level remain poorly understood. Here, we review known risk factors for the development of leukemic transformation in MPNs, recent progress made in our understanding of the molecular features associated with leukemic transformation, current treatment strategies, and emerging therapeutic options for this high-risk myeloid malignancy.

scholarly journals GASTROINTESTINAL MYELOID SARCOMA A CASE PRESENTATION AND CRITICAL REVIEW OF THE LITERATURE

2021 ◽  
Vol 13 (1) ◽  
pp. e2021067
Author(s):  
Pouyan Kheirkhah ◽  
Ana M. Avila-Rodriguez ◽  
Bartlomiej Radzik ◽  
Carlos Murga-Zamalloa
Keyword(s):  
Acute Myeloid Leukemia ◽  
Myeloid Leukemia ◽  
De Novo ◽  
Abdominal Cavity ◽  
Bone Marrow Involvement ◽  
Myeloid Sarcoma ◽  
Cell Transplant ◽  
Review Of The Literature ◽  
Hematopoietic Stem ◽  
Acute Myeloid

Myeloid sarcomas can be detected in up to 30% of acute myeloid leukemia cases or occur de-novo without bone marrow involvement. The most frequent localization of myeloid sarcomas in the abdominal cavity is the small intestine, and gastric presentations are infrequent, frequently misdiagnosed, and a high level of suspicion should exist when the characteristic histomorphology features are present. The current review features a case report with gastric presentation of myeloid sarcoma in a patient with a diagnosis of acute myeloid leukemia with trisomy 8. In addition, a review of the literature of intestinal type myeloid sarcomas shows that less than 15% of these cases have been reported in the stomach. The most common molecular aberrancy detected in intestinal myeloid sarcomas is the fusion protein CBFB-MYH11. Review of several large studies demonstrates that the presence of myeloid sarcoma does not constitute an independent prognostic factor. The therapeutic approach will be tailored to the specific genetic abnormalities present, and systemic chemotherapy with hematopoietic stem cell transplant are the most efficient strategies.

scholarly journals Myeloid Sarcoma: Evaluation of Histopathology, Immunoprofile and Cytogenetics

2021 ◽  
Author(s):  
Sanjiban Patra ◽  
Biren P Parikh ◽  
Kanwalpreet Kaur
Keyword(s):  
Myeloid Leukaemia ◽  
De Novo ◽  
Myeloproliferative Neoplasm ◽  
Molecular Data ◽  
Cross Sectional Study ◽  
Myeloid Sarcoma ◽  
Terminal Deoxynucleotidyl Transferase ◽  
Cross Sectional ◽  
Present Cross Sectional Study ◽  
Wrong Diagnosis

Introduction: Myeloid Sarcoma (MS), an uncommon tumour of immature myeloid blasts at extramedullary sites can be diagnostically challenging, if it is present de novo, precedes Acute Myeloid Leukaemia (AML) or Myeloproliferative Neoplasm (MPN). There are a few studies in literature with a substantial number of cases on clinicopathological, Immunophenotypic (IPT) and Immunohistochemical (IHC) characteristics of MS. Aim: This study was aimed to analyse myeloid sarcoma in Indian population according to the age, gender, site of involvement, differential diagnoses, IHC, IPT and cytogenetics and to add some to the existing knowledge. Materials and Methods: The present cross-sectional study was conducted at Gujarat Cancer Research Institute, Ahmedabad, with a total of 38 patients, diagnosed over past three years (January 2017 to December 2019). Clinical, morphological, IHC, IPT and molecular data of those 38 patients were retrieved and analysed. Results: Out of 38 cases, 16 (42.1%) and 13 (34.2%) cases were previously diagnosed cases of AML and Chronic Myeloid Leukaemia (CML) respectively. Nine patients (23.7%) presented as de novo. Most common site was lymph node (12/38, 31%), followed by breast, vertebra and other unusual sites. Myeloperoxidase (MPO) (31/38, 81.5%), CD117(25/35, 71.4%), CD45 (21/23, 91.3%), CD43 (14/22, 63.6%), CD68 (12/28, 42.8%), CD34 (15/37, 40.5%), CD33 (14/14, 100%), CD13 (15/16, 93.7%), HLA-DR (11/12, 91.6%), CD99 (3/29, 10.3%) and Terminal deoxynucleotidyl Transferase (TdT) (1/9, 11.1%) were expressed by tumour cells. However, epithelial mesenchymal, B and T lymphoid markers were negative. The present study found inv(16) and t(8;21) in MS in known AML patients. Among the known CML patients, two had variant positive for t (9;22) {t (2;9;22) (p23;q34;q11.2)}. Conclusion: Results of present study depicts that considering MS in differential diagnosis in its de novo presentation is necessary to prevent wrong diagnosis and help early treatment of these patients.

A Challenging Case of A Myeloid Sarcoma Misdiagnosed as High Grade B-Cell Lymphoma

2020 ◽  
Vol 154 (Supplement_1) ◽  
pp. S97-S97
Author(s):  
A Herrmann ◽  
B Mai ◽  
S Elzamly ◽  
A Wahed ◽  
A Nguyen ◽  
...  
Keyword(s):  
Bone Marrow ◽  
B Cell ◽  
Cell Lymphoma ◽  
B Cell Lymphoma ◽  
Myeloid Sarcoma ◽  
Proliferation Index ◽  
High Grade ◽  
Cell Markers ◽  
Thoracolumbar Region ◽  
The Right

Abstract Introduction/Objective A 46-year-old female presented with severe back pain associated with progressive bilateral lower extremity weakness and paresthesia, urinary retention, and constipation. Computed tomography revealed a retroperitoneal mass encasing the right psoas muscle, obstructing the right kidney, and extending to the thoracolumbar region resulting in severe spinal compression. An epidural tumor resection was subsequently performed at an outside hospital. Methods Histological sections showed sheets of blastoid neoplastic cells with intermediate to large nuclei, irregular membranes, fine chromatin, and prominent nucleoli. Immunohistochemical stains showed that these cells were positive for CD43, CD79a (weak, focal), BCL2, C-MYC, and PAX5 (weak, focal) and negative for CD10, CD20, CD30, ALK1, BCL6, MUM1, and Tdt. The Ki-67 proliferation index was 75-80%. With this immunophenotype, this patient was diagnosed with a high grade B-cell lymphoma and transferred to our institution for further work-up. On review of the slides, further immunohistochemical testing was requested which revealed positivity for CD117 and myeloperoxidase (MPO). Results The overall morphological and immunophenotypical features are most compatible with myeloid sarcoma (MS) with aberrant expression of B-cell markers and this patient’s diagnosis was amended. Interestingly, the patient’s bone marrow examination only showed 2% myeloblasts with left shifted granulocytosis and concurrent fluorescence in situ hybridization (FISH) studies were negative. Conclusion A literature review showed that 40-50% of MS are misdiagnosed as lymphoma. MS can frequently stain with B-cell or T-cell markers, as seen in this case, which makes it challenging for an accurate diagnosis and sub- classification. In addition, our case is interesting in that there was only extramedullary presentation without bone marrow involvement. Typically, MS develops after the diagnosis of acute myeloid leukemia (AML) with an incidence of 3–5% after AML. It can also manifest de novo in healthy patients, who then go on to develop AML months to years later. Therefore, this patient will require close follow-up.

scholarly journals Targeting the epichaperome as an effective precision medicine approach in a novel PML-SYK fusion acute myeloid leukemia

2021 ◽  
Vol 5 (1) ◽  
Author(s):  
Mayumi Sugita ◽  
David C. Wilkes ◽  
Rohan Bareja ◽  
Kenneth W. Eng ◽  
Sarah Nataraj ◽  
...  
Keyword(s):  
Acute Myeloid Leukemia ◽  
Myeloid Leukemia ◽  
Stem Cell Transplant ◽  
Myeloproliferative Neoplasm ◽  
Cell Transplant ◽  
Allogeneic Stem Cell Transplant ◽  
Target Engagement ◽  
Cell Level ◽  
Evaluation And Monitoring ◽  
Acute Myeloid

AbstractThe epichaperome is a new cancer target composed of hyperconnected networks of chaperome members that facilitate cell survival. Cancers with an altered chaperone configuration may be susceptible to epichaperome inhibitors. We developed a flow cytometry-based assay for evaluation and monitoring of epichaperome abundance at the single cell level, with the goal of prospectively identifying patients likely to respond to epichaperome inhibitors, to measure target engagement, and dependency during treatment. As proof of principle, we describe a patient with an unclassified myeloproliferative neoplasm harboring a novel PML-SYK fusion, who progressed to acute myeloid leukemia despite chemotherapy and allogeneic stem cell transplant. The leukemia was identified as having high epichaperome abundance. We obtained compassionate access to an investigational epichaperome inhibitor, PU-H71. After 16 doses, the patient achieved durable complete remission. These encouraging results suggest that further investigation of epichaperome inhibitors in patients with abundant baseline epichaperome levels is warranted.

scholarly journals Occurrence of a Clonal T-Cell Population in a Case of Chronic Myelomonocytic Leukemia

2021 ◽  
Vol 14 ◽  
pp. 263485352199150
Author(s):  
Anupama Patil ◽  
Balasaheb Wanve ◽  
Pradeep Kar ◽  
Shanthi Velusamy
Keyword(s):  
T Cell ◽  
Cell Population ◽  
De Novo ◽  
Treatment Guidelines ◽  
Myeloproliferative Neoplasm ◽  
Genetic Mutation ◽  
Pcr Analysis ◽  
Low Grade ◽  
Monocytic Leukemia ◽  
Laboratory Findings

Chronic myelo-monocytic leukemia (CMML) is an aggressive myeloid neoplasm with some features of a myelodysplastic syndrome (MDS) and others of a myeloproliferative neoplasm (MPN). Rarely, patients with CMML have a co-existing lympho-proliferative disorder (LPD). In most cases, the lymphoid neoplasm is diagnosed first, and the CMML is considered to be a secondary therapy-induced form of leukemia. We report herein a unique case of de-novo CMML, with an underlying clonal T-cell population and describe its clinical presentation and laboratory findings. A 70-year old male presented with a 3-month history of cough, dsypnea, abdominal distension, and low-grade fever. Physical and radiological examination revealed hepatosplenomegaly but no lymphadenopathy. Peripheral blood had absolute monocytosis with marrow showing CMML with 10% blasts along with dysplasia in myeloid and erythroid lineages. Flow cytometry indicated possibility of chronic myelo-monocytic leukemia with 13% monocytic cells along with an additional clonal population of gamma/delta T cells (15%) with aberrant immunophenotype. Polymerase chain reaction (PCR) analysis was positive for clonal T-cell rearrangement. A diagnosis of CMML with an underlying clonal T-CLPD was made. The synchronous occurrence of CMML and T-cell neoplasm may be attributed to a genetic mutation common to both. Currently, there are no treatment guidelines for group of patients; hence individualized therapeutic strategies should be implemented to enable symptomatic improvement and provide optimum care.

1305 Ureteral Stenting in Conservative Management of Grade IV Blunt Renal Injury

2021 ◽  
Vol 108 (Supplement_6) ◽  
Author(s):  
A. Mukhtar A Mukhtar ◽  
M. Gareeballah Yousif Hijazi ◽  
B.A. Abdalaziz Alshareif ◽  
M. Yahia Ibrahim
Keyword(s):  
Ct Scan ◽  
Conservative Management ◽  
Renal Injury ◽  
Motor Vehicle ◽  
Nonoperative Management ◽  
Fluid Collection ◽  
Dramatic Improvement ◽  
Ureteric Stenting ◽  
Delayed Hemorrhage ◽  
The Right

Abstract Post-traumatic urinomas are well-described complications associated with the nonoperative management of major blunt renal injuries. A 16-year-old male sustained a motor vehicle accident. Brought after 30 minutes to emergency department, upon arrival he was fully conscious, complaining of severe right hypochondrial and loin pain, abdomen was tender and guarded over the right side, urinary catheter inserted revealed gross haematuria, the patient was resuscitated accordingly, fast ultrasound scan showed minimal fluid collection in the Morison's pouch, the right kidney was swollen with perinephric fluid collection and poor cortico-medullary differentiation. Urgent CT scan findings were deep avulsion of the right kidney. The Patient was planned for conservative management, admitted to high dependency ward, CT scan repeated, and the size of urinoma increased compared to the initial CT, so he was planned for retrograde pyelography and ureteric stenting. Intra-operatively the right ureter was canulated, contrast injected. The pelvi-ureteric junction was intact, extravasation of contrast in the upper pole of the kidney. The right ureter was stented using a size 6 multiloop stent, with the tip directed into the upper pole calyx. The Patient showed dramatic improvement, haematuria cleared and the patient was discharged well after 12 days and the stent was removed after 6 weeks. Despite the improvements with nonoperative management, complications are described and include delayed hemorrhage, delayed massive hematuria and renal scaring with loss of function. Ureteric stenting is playing a major part in the conservative management of high-grade renal injury particularly grade IV type.

#3117 Fregoli syndrome in primary and secondary psychosis: a case level meta-analysis

2021 ◽  
Vol 92 (8) ◽  
pp. A18-A18
Author(s):  
Maria Teixeira-Dias ◽  
Amber Kaur Dadwal ◽  
Graham Blackman
Keyword(s):  
Right Hemisphere ◽  
English Literature ◽  
Meta Analysis ◽  
First Episode ◽  
Bias Assessment ◽  
Random Effects Models ◽  
Episode Psychosis ◽  
Treatment Responses ◽  
The Right

Objectives/AimsFregoli syndrome is a rare delusion characterised by the misidentification of an individual, typically of someone who the patient has an emotional link towards. The pathoaetiology of Fregoli syndrome remains largely a mystery, however, it has been described in patients experiencing either a primary or secondary (organic) psychosis. We sought to compare the neuropsychiatric features of Fregoli syndrome in primary and secondary psychosis.MethodsA patient-level meta-analysis was conducted. Five databases were searched for any descriptions of Fregoli syndrome. The patients and the psychotic episodes details alongside the co-occurring neuropsychiatric features and treatment responses were extracted. A risk of bias assessment was carried by scoring the methodological quality of all case studies. Random-effects models were used to pool the data and odds ratios and 95% confidence intervals were estimated for each of the neuropsychiatric features extracted between primary and secondary psychoses groups.ResultsA total of 119 patients (62 with primary psychosis, 50 with secondary psychosis and 7 with mixed or unknown aetiology) with Fregoli syndrome were identified in the English literature. Persecutory features were more likely to occur in patients with primary Fregoli syndrome (OR = 0.26, 95% CI[0.10;0.67], p < 0.01). In addition, Fregoli syndrome in the context of a first-episode psychosis (OR = 11.00, 95% CI [2.45;49.39], p < 0.01) and in the presence of neuroimaging abnormalities (OR = 20.19, 95% CI [4.36; 93.47], p < 0.01) was significantly associated with secondary aetiology. Patients in the secondary psychosis group (n=14) showed more right hemisphere lesions than patients in the primary psychosis group (n=1), however this trend was not significant (p = 0.10). Furthermore, no statistical differences between psychoses groups were found for the demographic, clinical and neurophysiological features analysed.ConclusionsThis is the first meta-analysis investigating the features of Fregoli syndrome in primary and secondary psychosis.Findings suggest that secondary causes of Fregoli syndrome are associated with a first-episode of psychosis and that neuroimaging abnormalities, particularly in the right hemisphere, are associated with a secondary organic cause.

Appendicitis: a rare adverse event in colonoscopy

2021 ◽  
Vol 14 (7) ◽  
pp. e242523
Author(s):  
Samer Al-Dury ◽  
Mohammad Khalil ◽  
Riadh Sadik ◽  
Per Hedenström
Keyword(s):  
Emergency Department ◽  
Adverse Event ◽  
Abdominal Pain ◽  
Acute Appendicitis ◽  
Physical Examination ◽  
De Novo ◽  
Perforated Appendicitis ◽  
Lower Quadrant ◽  
The Right ◽  
Right Lower Quadrant

We present a case of a 41-year-old woman who visited the emergency department (ED) with acute abdomen. She was diagnosed with perforated appendicitis and abscess formation on CT. She was treated conservatively with antibiotics and discharged. On control CT 3 months later, the appendix had healed, but signs of thickening of the terminal ileum were noticed and colonoscopy was performed, which was uneventful and showed no signs of inflammation. Twelve hours later, she developed pain in the right lower quadrant, followed by fever, and visited the ED. Physical examination and blood work showed signs consistent with acute appendicitis, and appendectomy was performed laparoscopically 6 hours later. The patient recovered remarkably shortly afterwards. Whether colonoscopy resulted in de novo appendicitis or exacerbated an already existing inflammation remains unknown. However, endoscopists should be aware of this rare, yet serious complication and consider it in the workup of post-colonoscopy abdominal pain.

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