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Mucosal leishmaniasis, an uncommon disease in the old world, is recognized as an occasional complication of donovani related visceral leishmaniasis. It usually affects the spleen, liver, skin, mucosa of the respiratory tract and, less commonly, the oral cavity. Our case was a young adult with non-healing lesions in the oropharyngeal region who was diagnosed as mucosal leishmaniasis during evaluation for suspected mucosal carcinoma. The diagnosis was made with the rK39 strip-test after multiple futile test Results:. A single dose of liposomal amphotericin B cured the disease. [ABSTRACT FROM AUTHOR] more...

Central nervous system (CNS) manifestation with cranial nerve palsy in multiple myeloma (MM) is a rare manifestation. Plasmacytoma originates from the bones of the skull base in 3% patients with MM but rarely develops from the soft tissues of the nasal cavity and paranasal sinuses. Here, we present a case of 68‐year‐old male patient with multiple myeloma, clivus bone plasmacytoma, and cavernous sinus syndrome. Clivus bone plasmacytoma with MM is a rare condition. Our patient presented with the pain in frontal and periorbital region, blurry vision and backache. Any patient presenting with these symptoms along with lytic bone lesions on imaging should be evaluated for MM. [ABSTRACT FROM AUTHOR] more...

A 26‐year‐old man presented with difficulty swallowing, dizziness, hiccups, and Horner's syndrome. Clinical and neuroimaging collaboration confirmed lateral medullary syndrome. Polycythemia was identified as the only attributable risk factor. However, the cause of polycythemia could not be assessed further. Polycythemia was managed with phlebotomy. [ABSTRACT FROM AUTHOR] more...

Evans syndrome and tuberculosis could be predisposing factors for one another, or there may be a common pathophysiological denominator for the co‐occurrence. Further research is needed for a better understanding of pathophysiology and treatment. [ABSTRACT FROM AUTHOR] more...

β‐thalassemia heterozygosity can cause significant iron overload when accompanied by HFE gene mutations and inappropriate iron supplementation. [ABSTRACT FROM AUTHOR]

Introduction Rosai-Dorfman disease (RDD) is a rare histiocytic proliferative disorder of unknown etiology. Usually it presents with massive painless cervical lymph node enlargement. Histologically, it shows proliferation of distinctive histiocytic cells that demonstrate emperipolesis in the background of a mixed inflammatory infiltrates. Immunohistochemically, the cells are positive for markers such as CD68 and S100. Objective To report a case of a 12-year-old patient with multiple sites of cervical lymphadenitis, which was diagnosed as RDD histopathologically as well as immunohistologically. Resumed Report A 12-year-old girl presented with multiple painless sites of cervical lymphadenitis without any systemic and other ear, nose, and throatmanifestations. The biopsy report of the lymph node showed dilatation of the sinuses, filled with histiocytes having foamy cytoplasm. Many of the histiocytes were engulfing mature lymphocytes. The sinus histiocytes were strongly positive for S-100 protein. Conclusion RDD must be considered in the differential diagnosis of massive or multiple lymphadenopathies. [ABSTRACT FROM AUTHOR] more...

Introduction: Essential thrombocythemia, a myeloproliferative condition with an increased number of circulating platelets, is a rare hematological malignancy. The aim of the study is to find out the prevalence of essential thrombocythemia among patients with myeloproliferative neoplasms presenting in haematology unit of a tertiary care centre., Methods: This was a descriptive cross-sectional study at a tertiary care centre from September, 2020 to September, 2021 (Reference number: 48 (6-11) E2077/076). All the patients with a diagnosis of essential thrombocythemia and willing to give consent were included in the study while the patients with incomplete investigations were excluded. A sample size of 72 patients was taken and convenience sampling was done. Data were entered in Microsoft Excel 2010 and analysis was done by the Statistical Package for the Social Sciences Version 22.0. Point estimate at 95% Confidence Interval was calculated along with frequency and proportion for binary data along with mean and standard deviation for continuous data., Results: Among 72 patients with myeloproliferative neoplasms, the prevalence of essential thrombocythemia was found to be 17 (23.61%) (13.80-33.42 at a 95% Confidence Interval). The mean age of patients was 55.41±11.20 years with a male to female ratio of 9:8. The mean hemoglobin level and platelet count in patients were found to be 11.20±2.1 g/dl and 677000±262067.70 cells/mm3. Twelve (70.58%) of total patients were under low risk of essential thrombocythemia while 3 (17.64%) of them were at high risk., Conclusions: The prevalence of essential thrombocythemia was similar to other studies done in similar settings., Keywords: essential thrombocythemia; hematology; mutation. more...

Paroxysmal nocturnal hemoglobinuria (PNH) is a rare, acquired stem cell disorder manifesting as non-immunological hemolytic anemia, hemoglobinuria, unusual thrombosis, and renal impairment due to deficiency of glycosylphosphatidylinositol (GPI) linked proteins in red blood cells. Patients present with features of chronic non-immune intravascular hemolysis, unexplained anemia, and thrombosis at unusual sites. It is often misdiagnosed and treated as anemia due to a low degree of suspicion. In resource-limited settings, the low degree of suspicion and paucity of investigations are the major diagnostic challenges. The even bigger challenge remains in the affordability of definitive treatment after a diagnosis has been made. Herein, we present a case of PNH in a 26-year man from rural Nepal who went undetected during the initial presentation of hemolytic anemia and later presented to us with hemolytic anemia and gastrointestinal symptoms. We made the provisional diagnosis based on the clinical presentations. However, we faced challenges in reaching the final diagnosis and providing the definitive treatment due to financial constraints and limited resources. Any patient presenting with features of chronic non-immune intravascular hemolysis, unexplained anemia, and unusual thrombosis should prompt the consideration of PNH., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2021, Pokhrel et al.) more...