Acute panmylelosis with myelofibrosis - A rare case report with review of literature.

ABSTRACT: Acute panmyelosis with myelofibrosis (APMF) corresponds to <1% cases of acute myeloid leukemia, which could be an underestimation due to missed diagnosis. Due to its rapidly fatal course, it warrants a timely and correct diagnosis. We present a case of a 44-year male who came with a short history of fever, generalised weakness, revealed pancytopenia with occasional circulating blast in the peripheral blood smear. Bone marrow aspirate was dry tap,biopsy revealed panmyelosis with myelofibrosis with increased (22%) blasts. Flowcytometric immunophenotyping, cytogenetics and molecular tests were undertaken. Together with clinical details, immunophenotypic profile, cytogenetics and molecular studies, the diagnosis of Acute panmyelosis with myelofibrosis was made and managed accordingly. 32 The WHO 2017 describes APMF as an acute panmyeloid proliferation with increased blasts (≥20% in the bone marrow or peripheral blood) and accompanying marrow fibrosis. APMF is rare with poor prognosis thus, must be differentiated especially from Acute megakaryoblastic leukemia to arrive at the correct diagnosis which will help reduce/prevent the early mortality by providing timely chemotherapy followed by upfront hemopoietic stem cell transplantation.

Pelvic Synovial Sarcoma Clinically Masquerading as an Ovarian Malignancy.

Background: Synovial sarcoma, a malignant mesenchymal tumor, commonly involves the extremities and is rarely found in the pelvis. Cytology with a biphasic pattern can suggest the diagnosis of synovial sarcoma. Case Report: A 32-year-old female presented with complaints of abdominal distension. She was initially evaluated in another hospital where she underwent ultrasound-guided fine needle aspiration cytology (FNAC) of the abdominal mass. The mass was diagnosed as ovarian adenocarcinoma, and the patient underwent 3 cycles of chemotherapy. After 3 months, she was referred to our institute for surgical excision of the mass. Contrast-enhanced computed tomography of the abdomen suggested a well-circumscribed, heterogeneous, solid-cystic mass in the left adnexal region measuring 13.9 × 10 × 9.1 cm and compressing the adjacent structures. No previous radiologic investigations were available. We reviewed the FNAC slide from the outside hospital and made a preliminary diagnosis of biphasic synovial sarcoma. The patient underwent debulking surgery consisting of panhysterectomy with excision of the pelvic mass. Microscopic examination of the pelvic mass showed a biphasic tumor composed of epithelial and mesenchymal components, suggestive of synovial sarcoma. The immunohistochemistry profile supported the morphologic diagnosis. Bilateral ovaries were unremarkable. The patient received 4 cycles of adjuvant chemotherapy and is presently asymptomatic. Conclusion: Although primary pelvic synovial sarcoma is a rare occurrence, this case illustrates that synovial sarcoma can be diagnosed or at least suspected on FNAC. Because of the importance of adjuvant chemotherapy, synovial sarcoma must be high on the list of differential diagnoses of high-grade intra-abdominal masses.

Stimuli-Responsive Self-Assembly Disassembly in Peptide Amphiphiles to Endow Block-co-Fibers and Tunable Piezoelectric Response.

Supramolecular assemblies with well-defined structural attenuation toward varied functional implications are an emerging area in mimicking natural biomaterials. In that regard, the redox stimuli-responsive ferrocene moiety can reversibly change between a nonpolar ferrocenyl and polar ferrocenium cation that endows interesting modular features to the building blocks with respect to self-assembly/disassembly. We design a series of ferrocene anchored peptide fragment NVFFAKKC using hydrophobic alkyl spacers of different chain lengths. Increasing the spacer length between the redox-responsive and self-assembling motifs increases the propensity to form robust nanofibers, which can be physically cross-linked to form hydrogels. The controlled redox response of the ferrocene moiety tandem with pH control provides access to structural control over the peptide nanostructures and tunable mechanical strengths. Further, such redox-sequestered dormant states hinder the spontaneous nucleation process that we exploit toward seeded supramolecular polymerization to form block cofibers composed of redox-responsive periphery and nonresponsive cores. Finally, such redox sequestration of peptide self-assembly renders an on-off piezoelectric response for potential utilization in peptide bioelectronics.

The efficacy of ultrasound-guided triple nerve block (ilioinguinal, iliohypogastric, and genitofemoral) versus unilateral subarachnoid block for inguinal hernia surgery in adults: a randomized controlled trial.

INTRODUCTION: The present study was carried out to evaluate the efficacy of ultrasound-guided triple nerve block (ilioinguinal, iliohypogastric, and genitofemoral) versus unilateral subarachnoid block for adult male patients undergoing unilateral inguinal hernia surgery. MATERIAL AND METHODS: Sixty ASA I-III adult male patients > 18 years old, scheduled for unilateral inguinal hernia surgery were randomly allocated into 2 groups of 30 patients each. In Group A ( n = 30) the patients received ultrasound-guided nerve block (ilioinguinal, iliohypogastric, and genitofemoral), and in Group B ( n = 30) the patients received unilateral subarachnoid block. The primary outcome was to assess postoperative analgesic efficacy (visual analogue scale [VAS] scores at rest and during coughing/ambulation). The secondary outcomes were time to first rescue analgesia with morphine, the total dose of morphine used as rescue analgesia, urinary retention, time to first micturition, time to first unassisted walking, and time to discharge from the surgical recovery room. RESULTS: The mean pain scores at 1, 2, 4, and 6 hours during rest and during coughing/ambulation were significantly lower in Group A when compared to patients in Group B ( P < 0.001). There was no requirement for rescue analgesic opioids in Group A ( P < 0.001). Mean time to first micturition and mobilization occurred earlier in Group A, leading to early discharge from the recovery room ( P < 0.001). No major side effects were observed in any of the study groups. CONCLUSIONS: Ultrasound-guided triple nerve block technique can be used as a sole anaesthetic technique for inguinal hernia surgery because it not only provides optimal anaesthesia intra-operatively but also has a favourable analgesic and opioid-sparing efficacy in the early postoperative period with minimal adverse effects.

Case of angioimmunoblastic T-cell lymphoma presenting as peripheral and bone marrow plasmacytosis: A diagnostic conundrum.

ABSTRACT: Angioimmunoblastic T-cell lymphoma (AITL), a subtype of peripheral T-cell lymphoma (PTCL), is associated with unique clinical, morphological, and immunohistochemical features. The peripheral circulation might show presence of an occasional reactive plasma cell but significant plasmacytosis masquerading as plasma cell leukemia is rare. We report a case of AITL in a 42-year-old male, who presented with two-month history of generalized lymphadenopathy. On investigations, he had hypergammaglobulinemia and plasmacytosis in the peripheral blood and bone marrow masquerading as plasma cell leukemia. Immunohistochemistry and serum protein electrophoresis revealed polyclonal nature of plasma cells. Diagnosis of AITL was made on cervical lymph node biopsy. This case highlights the diagnostic challenge faced due to heterogeneity in the clinical presentation and pathological findings and to alert the clinician so that timely accurate diagnosis can be made to initiate the treatment.

Transcriptomic Profiling Unravels Novel Deregulated Gene Signatures Associated with Acute Myocardial Infarction: A Bioinformatics Approach.

Acute myocardial infarction (AMI) is a severe disease with elevated morbidity and mortality rate worldwide. This is attributed to great losses of cardiomyocytes, which can trigger the alteration of gene expression patterns. Although several attempts have been made to assess the AMI biomarkers, to date their role in rescuing myocardial injury remains unclear. Therefore, the current study investigated three independent microarray-based gene expression datasets from AMI patients (n = 85) and their age-sex-matched healthy controls (n = 70), to identify novel gene signatures that might be involved in cardioprotection. The differentially expressed genes (DEGs) were analyzed using 'GEO2R', and weighted gene correlation network analysis (WGCNA) was performed to identify biomarkers/modules. We found 91 DEGs, of which the number of upregulated and downregulated genes were 22 and 5, respectively. Specifically, we found that the deregulated genes such as ADOR-A3, BMP6, VPS8, and GPx3, may be associated with AMI. WGCNA revealed four highly preserved modules among all datasets. The 'Enrichr' unveiled the presence of miR-660 and STAT1, which is known to affect AMI severity. Conclusively, these genes and miRNA might play a crucial role the rescue of cardiomyocytes from severe damage, which could be helpful in developing appropriate therapeutic strategies for the management of AMI.

Anion-responsive self-assembled hydrogels of a phenylalanine-TREN conjugate allow sequential release of propranolol and doxorubicin.

Stimuli-responsive self-assembled and supramolecular hydrogels derived from peptide amphiphiles have opened exciting new avenues in biomedicine and drug delivery. Herein, we screened a series of phenylalanine-amphiphiles possessing polyamine and oxyethylene appendages for their self-assembly and anion-responsiveness and found that the tris(aminoethyl)amine (TREN) containing amphiphile NapF-TREN formed injectable hydrogels that could be disrupted upon the addition of stoichiometric amounts of tetrahedral monovalent anions such as H2PO4- and HSO4-, while the addition of other anions such as Cl-, HPO42-, CO32-, HCO3- or SO42- did not affect the gel stability. The anion-gelator interaction was investigated by 1H and 31P NMR spectroscopy as well as by Isothermal Titration Calorimetry (ITC). These studies confirmed a 1 : 1 stoichiometry and revealed negative enthalpy and negative entropy for the binding of H2PO4- with NapF-TREN. Microscopic investigations by TEM, AFM, and SAXS revealed that H2PO4- anions induced a nanofiber-to-nanoglobule morphological change in the aqueous self-assemblies of NapF-TREN. However, upon ageing the samples, slow reformation of the nanofibers was also observed, reflecting the reversibility of the anion-gelator interaction. The anion- and pH-responsive nature of the NapF-TREN hydrogels was exploited to program sequential release of entrapped drugs propranolol and doxorubicin.

Multiple tuberous xanthomas diagnosed on fine-needle aspiration cytology - Report of a rare case.

Xanthomas are papulonodular skin lesions present in lipoprotein metabolism disorders, which result in cholesterol deposits in subcutaneous tissue, tendons, ligaments, periosteum, etc. A 28-year-old male presented with multiple soft-tissue swellings, prominently over joints. Fine-needle aspiration (FNA) from multiple sites had similar appearance with foamy histocytes and giant cells. We describe an unusual case of tendinous and tuberous xanthoma diagnosed by cytology. Acquaintance with FNA cytology findings in xanthomas can help to avoid the need of surgical biopsy, as xanthomas can regress on medical therapy alone.

COVID-19, Mucormycosis and Cancer: The Triple Threat-Hypothesis or Reality?

COVID-19 has been responsible for widespread morbidity and mortality worldwide. Invasive mucormycosis has death rates scaling 80%. India, one of the countries hit worst by the pandemic, is also a hotbed with the highest death rates for mucormycosis. Cancer, a ubiquitously present menace, also contributes to higher case fatality rates. All three entities studied here are individual, massive healthcare threats. The danger of one disease predisposing to the other, the poor performance status of patients with all three diseases, the impact of therapeutics for one disease on the pathology and therapy of the others all warrant physicians having a better understanding of the interplay. This is imperative so as to effectively establish control over the individual patient and population health. It is important to understand the interactions to effectively manage all three entities together to reduce overall morbidity. In this review article, we search for an inter-relationship between the COVID-19 pandemic, emerging mucormycosis, and the global giant, cancer.

Intestinal mucormycosis in a patient with COVID-19: A case report.

The novel coronavirus disease (COVID-19) has spread to all the continents posing a serious threat to global health. It can present with myriad symptoms and complications including susceptibility to fungal co-infections. We hereby describe a case of mucormycosis in a 17 year old COVID-19 positive female with no known comorbidities presenting with abdominal pain and distention and ultimately developing intestinal perforation. Early identication of the symptoms is essential to make a correct and early diagnosis to prevent complications.

Adenosquamous Carcinoma of the Extrahepatic Bile Duct.

Background: Most malignant tumors arising from the biliary tract are adenocarcinomas. Adenosquamous carcinoma is an uncommon variant of extrahepatic bile duct cancer that is associated with more aggressive behavior than adenocarcinoma. Case Report: A 58-year-old male presented with complaints of obstructive jaundice and dull aching pain in the abdomen. At his initial evaluation in another hospital, ultrasound of the abdomen and magnetic resonance cholangiopancreatography revealed common bile duct dilatation with an ill-defined lesion in the periampullary region. Endoscopic retrograde cholangiopancreatography showed infiltration of the ampulla. Biopsy specimen taken during stenting of the common bile duct suggested dysplasia. Because of recurrent jaundice, the patient was referred to our institution, and contrast-enhanced computed tomography (CT) of the abdomen showed a hypoenhancing lesion with ill-defined margins in the head of the pancreas. The patient underwent a Whipple procedure. Microscopic examination of the pancreaticoduodenectomy specimen showed an invasive adenosquamous tumor arising from the distal part of the common bile duct that infiltrated the body and head of the pancreas, involved the wall of the duodenum, and reached the duodenal mucosa. Morphologic diagnosis was supported by immunohistochemistry profile. Postoperative contrast-enhanced CT of the abdomen showed multiple enlarged mesenteric lymph nodes and multiple lesions in both lobes of the liver, suggestive of metastasis. The patient died 1 week after surgery. Conclusion: Because the clinical, pathologic, and prognostic characteristics of adenosquamous carcinoma are poorly known, early diagnosis of this rare entity is warranted for patient management.

Cervical Thymic Cyst with parathyroid tissue - a diagnostic conundrum.

Cervical thymic cysts are relatively rare benign cystic lesions that tend to be diagnosed clinically as branchial cysts, which usually present as painless, enlarging neck masses. They can occur anywhere along the normal path of descent of thymic primordia from the angle of the mandible to the sternal notch, with mediastinal extension observed in approximately 50% of cases. They are usually seen in the first decade of life on the left side with a male predominance. Here we report a case of a 15-year-old boy who presented to the hospital with left-sided neck swelling for about 2 months. The neck's contrast-enhanced computed tomography (CECT) revealed a large, well-defined cystic swelling in the left neck region, showing peripheral enhancement, seen from the submandibular region to the superior mediastinum extending into the retrosternal region. Direct fine needle aspiration (FNA) was done, which showed a benign lesion with inflammatory and cystic characteristics, leading to the possibility of a branchial cyst. The cyst was completely excised surgically. Histopathology showed a thymic cyst with parathyroid tissue. The presence of thymic tissue with Hassall's corpuscles is essential for the diagnosis. Knowledge of the clinical presentation, cyto-histological findings, and differential diagnosis of cystic cervical lesions in the pediatric population is important to diagnose this rare entity. Hence, though uncommon, when one comes across a cystic cervical region mass in children, a diagnosis of cervical thymic cyst should be kept in mind. Nonetheless, a definitive diagnosis depends on imaging findings as well as intraoperative findings and histopathological examination.

ALK-positive anaplastic large cell lymphoma of skeletal muscle masquerading as soft tissue sarcoma.

Anaplastic large cell lymphoma (ALCL) is a subtype of T cell non-Hodgkin's lymphoma and can present as an extranodal disease. Primary ALCL of skeletal muscle is rare. We report a case of ALK-positive ALCL involving the left thigh and anterior chest wall in a 12-year-old male child. The fine needle aspiration cytology from the lesion showed cellular smears with singly scattered and occasional clusters of large pleomorphic atypical cells. A diagnosis of poorly differentiated malignant neoplasm was rendered. On core biopsy, the histomorphological features posed a diagnostic challenge with a myriad of morphological mimickers. The diagnosis was established by excluding specific entities by relevant immunostains and confirming the diagnosis by strong expression of CD30 and ALK on immunohistochemistry. Fluorescence in-situ hybridisation confirmed the characteristic t(2:5) translocation. Presentation of ALCL with skeletal muscle involvement is uncommon, and the diagnosis relies on broadening the diagnostic possibilities and judicious use of immunohistochemical markers.

Cervical Thymic Cyst with parathyroid tissue - a diagnostic conundrum

Cervical thymic cysts are relatively rare benign cystic lesions that tend to be diagnosed clinically as branchial cysts, which usually present as painless, enlarging neck masses. They can occur anywhere along the normal path of descent of thymic primordia from the angle of the mandible to the sternal notch, with mediastinal extension observed in approximately 50% of cases. They are usually seen in the first decade of life on the left side with a male predominance. Here we report a case of a 15-year-old boy who presented to the hospital with left-sided neck swelling for about 2 months. The neck's contrast-enhanced computed tomography (CECT) revealed a large, well-defined cystic swelling in the left neck region, showing peripheral enhancement, seen from the submandibular region to the superior mediastinum extending into the retrosternal region. Direct fine needle aspiration (FNA) was done, which showed a benign lesion with inflammatory and cystic characteristics, leading to the possibility of a branchial cyst. The cyst was completely excised surgically. Histopathology showed a thymic cyst with parathyroid tissue. The presence of thymic tissue with Hassall's corpuscles is essential for the diagnosis. Knowledge of the clinical presentation, cyto-histological findings, and differential diagnosis of cystic cervical lesions in the pediatric population is important to diagnose this rare entity. Hence, though uncommon, when one comes across a cystic cervical region mass in children, a diagnosis of cervical thymic cyst should be kept in mind. Nonetheless, a definitive diagnosis depends on imaging findings as well as intraoperative findings and histopathological examination.

Genome-Wide Scanning of Potential Hotspots for Adenosine Methylation: A Potential Path to Neuronal Development.

Methylation of adenosines at N6 position (m6A) is the most frequent internal modification in mRNAs of the human genome and attributable to diverse roles in physiological development, and pathophysiological processes. However, studies on the role of m6A in neuronal development are sparse and not well-documented. The m6A detection remains challenging due to its inconsistent pattern and less sensitivity by the current detection techniques. Therefore, we applied a sliding window technique to identify the consensus site (5'-GGACT-3') n ≥ 2 and annotated all m6A hotspots in the human genome. Over 6.78 × 107 hotspots were identified and 96.4% were found to be located in the non-coding regions, suggesting that methylation occurs before splicing. Several genes, RPS6K, NRP1, NRXN, EGFR, YTHDF2, have been involved in various stages of neuron development and their functioning. However, the contribution of m6A in these genes needs further validation in the experimental model. Thus, the present study elaborates the location of m6A in the human genome and its function in neuron physiology.

Phase transformation and enhanced blue photoluminescence of zirconium oxide poly-crystalline thin film induced by Ni ion beam irradiation.

Swift heavy ions (SHI) irradiation of Nickel (Ni) beam with different ions fluence bring the modifications in the functional properties of radio frequency (RF) grown zirconium oxide (ZrO2) nanocrystalline thin films. X-ray diffraction analysis affirms the monoclinic to tetragonal phase transformation and diminishing of peak at higher fluence 1 × 1014 and 2 × 1014 ions/cm2 induced by electronic excitation caused by SHI. Zirconium oxide thin films exhibit the same thickness (195 nm) of virgin and irradiated samples and whereas the nanocrystalline thin films have the elemental composition in proper stoichiometry (1:2) as analyzed by rutherford backscattering spectroscopy (RBS). Photoluminescence measurements confirm the blue emission of virgin and irradiated sample recorded at excitation wavelength 270 to 310 nm. The intensity of obtained emission bands varies with fluence which is interpreted in terms of generation and annihilation of defect centers. The characteristic Ag and Bg Raman modes of monoclinic and tetragonal ZrO2 are obtained at different positions. Moreover, the nanocrystalline ZrO2 thin films exhibits the most prominent absorption phenomenon in the visible range and the irradiation cause significant decrease in band gap to 3.69 eV compare to the virgin ZrO2 sample (3.86 eV). XPS analysis indicates the shifting of the core levels Zr 3d and O 1s towards higher binding energy and spin-orbit splitting of different states. The findings in this research justify that the irradiated thin films can be a potential candidate for designing of new materials, intense radiation environments, nuclear reactors, nuclear waste systems, clean energy sources.