Leukemic Transformation In Myelodysplastic Syndrome: A Case Report With Review Of Risk Factors

Myelodysplastic syndromes are a group of clonal disorders affecting the hemopoietic stem cells and characterized by peripheral cytopenias with normocellular to hypercellular bone marrow and various morphological abnormalities in one or more hemopoietic cell lines. MDS carries a high risk of progression to acute myeloid leukemia especially in subtypes with increased myeloblasts. Here, we present the case of leukemic transformation in MDSin a 41-year-old male who presented with complaints of generalized weakness, loss of appetite for 2 months and fever on and off for 1 week. The patient was diagnosed as MDS-multilineage dysplasia after blood examination and bone marrow biopsy but the patient refused for further treatment

Chediak Higashi Syndrome: A Case Report Of Rare Anomaly

Chediak Higashi syndrome (CHS) is a rare autosomal recessive immunodeficiency disorder that arises due to the mutation of a trafficking protein which leads to a decrease in phagocytosis. This results in frequent pyogenic infections, albinism, and peripheral neuropathy. Infections in these patients tend to be very serious and life-threatening. CHS is caused by mutations in a gene LYST on chromosome 1. Here, we report the case of CHS in a 4-year-old boy who presented to us with recurrent fever, splenomegaly, and hypopigmentation. This case is being presented due to its rarity and presentation of pancytopenia without hemophagocytosis

Leukemic Pleural Effusion In Aml-M0: Unusual Presentation

Extramedullary disease in AML-M0 is a rare occurrence. It is even rarer to encounter breathlessness as primary presenting complaint secondary to a leukemic pleural effusion. Leukemic pleural effusion occurs rarely in acute myeloid leukemia (AML) with minimal differentiation. Patients with AML usually present with weakness, hemorrhage, and recurrent infections. The highest incidence of extramedullary leukemic manifestation is seen in monocytic AML. Here, we report the case of an 18-year-old male who presented withbreathlessness and vocal cord palsy. Examination and investigation established diagnosis of an acute myeloid leukemia M0 with leukemic pleural effusion

Significance of AgNOR in Salivary Gland Tumours.

Diagnosis of Salivary gland tumours is challenging, because of wide variation in differentiation and overlapping morphological features. Sometimes, the difficulty encountered in distinguishing between pleomorphic adenoma, adenoid cystic carcinoma and adenocarcinoma. The objective is to study the application of AgNOR pattern in differentiating benign and malignant tumours of the salivary glands on Fine needle aspirates and their correlation with histopathology. Material and method: Cytological material was obtained by FNAC from forty three patients of salivary gland tumours. MGG and Pap stained smears were prepared for cytological interpretation. Histopathological study was done on routine formalin fixed and Haematoxylin & Eosin stained sections. Smears and sections were stained with Silver colloid stain for study of AgNOR counting. Results: AgNOR in benign tumours were small, round, uniform and less in number (1.02-1.97) while in malignant tumours they were very large, irregular, haphazardly distributed with high counts (1.23-16). Conclusion: Present study shows that count as well as morphology of AgNOR dots was helpful in differentiating between benign and malignant tumours and their grading of malignancy .

Myelomatous Effusion in Multiple Myeloma.

Multiple myeloma (MM) is a systemic disease characterized by monoclonal proliferation of plasma cells. The clinical manifestations of the disease are due to tumour cell infiltration and monoclonal immunoglobulins and / or light chains secreted by the neoplastic cells. Effusions may develop in cases of multiple myeloma due to various causes but myelomatous effusion is uncommon. Moreover, multiple myeloma presenting as bilateral pleural effusion is exceptional and a case of multiple myeloma presenting with bilateral pleural effusion and anaemia is reported.

Weak D antigen - Revisited.

D antigen is the most immunogenic antigen in the complex Rh blood group system discovered in the year 1939. There is a lot of polymorphism in its phenotype due to genetic heterogeneity. Certain mutations and /or deletions lead to a weak phenotype defined by decreased density of antigen sites which require the use of anti human globulin for detection. The need for detection of the weak D antigen was to prevent alloimmunization by this blood if transfused to a D negative patient especially to women in child bearing age group. This contention is however, controversial and not proven beyond doubt. Moreover, the use of potent monoclonal D typing antisera detects low density of weak D antigens thus obviating the use of anti human globulin. We have assessed the incidence of Rh negative and weak D blood groups in the Garhwal region of Uttarakhand and reviewed the literature regarding the controversies in the clinical significance of weak D antigen.