We present a case of a 15-year-old girl with a pulsatile, rapidly enlarging mass at the root of the nose suspected to be malignant. Excisional biopsy showed worrisome histological features; however, a final diagnosis of cellular schwannoma was reached excluding the possibility of malignant peripheral nerve sheath tumour by histological and immunohistochemical attributes. Cellular schwannoma, a pseudosarcomatous entity, is a rare benign neoplasm that may cause bone erosion and may be mistaken for a malignancy, clinically and histologically. Diagnosis of cellular schwannoma is essential to prevent mismanagement as it never metastasises and responds to local excision as opposed to aggressive treatment required by a malignant neoplasm.
Introduction: High resolution electrophoresis (HRE) and immunofixation (IFX) of serum and urine are integral to the diagnostic work-up of multiple myeloma. Unusual electrophoresis patterns are common and may be misinterpreted. Though primarily the responsibility of the hematopathologist, clinicians who are responsible for managing myelomas may benefit from knowledge of these. In this review article we intend to discuss the patterns and importance of electrophoresis in present day scenario. Methods: Patterns of HRE and IFX seen in our laboratory over the past 15 years were studied. Results: Monoclonal proteins are seen on HRE as sharply defined bands, sometimes two, lying from c-to a-globulin regions on a background of normal, increased or decreased polyclonal c-globulins, showing HRE to be a rapid and dependable method of detecting M-protein in serum or urine. Immunofixation complements HRE and due to its greater sensitivity, is able to pick up small or light chain bands, not apparent on electrophoresis, including biclonal disease even when electrophoresis shows only one M-band. Special features liable to misinterpretation are discussed. Familiarity with the interpretation of the varied patterns seen in health and disease is essential for providing dependable laboratory support in the management of multiple myeloma.
SUMMARYWe present a case of a salivary gland tumour in a 25-year-old woman with lymphadenopathy and a clinical suspicion of lymphoma. The patient had a history of rapidly enlarging mass near angle of jaw which was resected and sent for histopathological examination. A final diagnosis of acinic cell tumour with dedifferentiation was made by histomorphological and immunohistochemical studies. Acinic cell tumour can mimic any salivary neoplasm phenotypically because of its varied architectural patterns of presentation with varied cell types, hence called the harlequin of salivary gland. Acinic cell tumour with dedifferentiation is a rare aggressive variant and requires adjuvant radiotherapy for better prognosis, hence the need for accurate diagnosis and communication to the surgeon BACKGROUND
Introduction Hemophagocytic lymphohistiocytosis (HLH) is an immune deregulation disorder with varied clinical presentation which clinically overlaps with widespread tropical infections. Methods We conducted a retrospective chart review of children diagnosed with HLH at our center from February‐2017 to October‐2020. Results Out of the nine diagnosed patients, genetic predisposition was present in three children; two had identified infectious triggers. The mean age of presentation was 30 months with male predominance. The most common clinical findings were fever, organomegaly, and pancytopenia. The median value of fibrinogen was‐156 mg/dL, ferritin‐12 957 ng/mL and for triglycerides‐349 mg/dL, respectively. In children with identified genetic predisposition, serum ferritin levels were usually more than 10 000 ng/mL. The majority of our patients had evidence of hemophagocytosis on bone marrow examination. In our experience, although nonspecific, very high ferritin and serum triglycerides with low fibrinogen in a patient with bi‐cytopenia, pancytopenia was the most suggestive evidence of HLH. Genetic evaluation in our series identified three children, one with primary HLH genetic mutation and two with underlying immune deficiency syndrome. The presence of HLH in the accelerated phase of Chediak‐Higashi and AD Hyper IgE syndrome with HLH is extremely rare. Leishmaniasis (in nonendemic area) and Ebstein‐Barr virus (EBV) was identified as an infectious trigger in two cases. Most of our cases received treatment as per HLH 2004 protocol. Three children died during the initial diagnosis and treatment. HLH with subcutaneous panniculitis‐like T‐cell lymphoma recovered well. Conclusion HLH remains a life‐threatening disorder associated with a variety of underlying illnesses as highlighted by our case series.
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