--Immunophenotypic Aberrancies in Acute Leukemia: A Tertiary Care Centre Experience.

OBJECTIVES: Acute leukemias (AL) are a heterogeneous group of hematological malignancies with the presence of 20% or more blasts in the peripheral blood or bone marrow. Malignant cells display characteristic patterns of surface antigenic expression. Aberrant phenotypes are defined as patterns of antigen expression on neoplastic cells different from the process of normal hematopoietic maturation. We sought to evaluate the occurrence of aberrant phenotypes in newly diagnosed cases of AL. METHODS: The study included 100 patients in whom both bone marrow aspiration and flow cytometry were performed. Patients with blasts > 20% of all ages were included in the study. Flow cytometric analysis was done using the monoclonal antibody panel of peripheral blood/bone marrow. RESULTS: Out of 100 cases, 53 were categorized as acute myeloid leukemia (AML), 43 as acute lymphoid leukemia (ALL), and four cases of mixed phenotypic acute leukemia (MPAL). ALL were subcategorized based on immunophenotyping into B-ALL and T-ALL, which comprised 88.4% and 11.6%, respectively, of total ALL (43.0%) cases. Cluster of differentiation 33 (CD33) and CD13 were the most commonly expressed antigens in AML, with CD7 being the most common aberrancy. CD19 was expressed in all B-ALL cases followed by cCD79a, CD10, Tdt (86.8%) with CD13 being the most common aberrancy. cCD3, CD7, and CD5 were expressed in all T-ALL cases with aberrant antigen expression in 80.0% of T-ALL cases. MPAL cases showed expression of B/myeloid antigens. CONCLUSIONS: The diagnosis and classification of leukemia rely on the simultaneous application of cytomorphology, cytochemistry, flow cytometry, cytogenetics, and molecular techniques. Flow cytometry is of great help in the diagnosis of AL, particularly in ALL for lineage assignment and in classifying MPAL. It also helps in detecting aberrant antigen expression and assisting in minimal residual disease detection.

A rare case of mesenteric vessel thrombosis post caesarian section-An underdiagnosed entity.

INTRODUCTION: Respiratory distress is an uncommon clinical event after caesarean section and occurs due to pulmonary thromboembolism. Various causes of pulmonary thromboembolism are thrombophlebitis, ovarian venous thrombosis and mesenteric vein thrombosis. PRESENTATION OF CASE: We report a case of 30 year old female who presented with respiratory distress after eight days of uneventful caesarian section. On emergency explorative laparotomy, small gut was found to be gangrenous, so resection of the segment was performed. On histopathological examination, there was ischaemic necrosis of bowel with presence of large thrombus in mesenteric vessel. On correlating radiological findings of pulmonary thromboembolism and mesenteric vessel thrombosis with bad obstetric history, a possibility of Antiphospholipid syndrome (APS) was suggested in this case. Unfortunately, patient died the day following laparotomy so there was insufficient time to evaluate the patient for thrombophilic disorders. DISCUSSION: Pregnancy and perpeurium are associated with higher risk of thrombosis as these are hypercoagulable states. Operative delivery and history of thrombophilia in previous pregnancies (APS) are other predisposing factors which lead to increased thrombotic state and pulmonary thromboembolism. CONCLUSION: High index of suspicion for thrombophilic disorders is required in postpartum patients presenting with respiratory distress as prompt diagnosis and urgent intervention can save patient's life.

'Pleomorphic adenoma of the tongue: A common entity at the uncommon location'.

The salivary gland tumors comprise of 3% of head and neck tumors. Pleomorphic adenoma, also known as mixed tumor, is the most common benign neoplasm of the major and minor salivary glands. The occurrence of pleomorphic adenoma of the tongue is very rare, and very few cases have been reported in the literature. A 41-year-old male presented with swelling on tongue. Cytological and histological examination revealed pleomorphic adenoma. Complete excision of the mass was done transorally under general anaesthesia. High index of suspicion and an adequate clearance of the tumor with a cuff of surrounding dispensable normal tissues is the key to successful treatment of such tumors. The authors consider the rarity of this case and present a rare case of pleomorphic adenoma of the tongue.

Scorecard - An Innovative Simplified Tool to Supplement the Existing Monitoring Mechanism to Assess and Improve Performance of Antiretroviral Treatment Centers.

INTRODUCTION: All 26 antiretroviral treatment (ART) centers of Gujarat were monitored by Gujarat State AIDS Control Society under the National AIDS Control Program. A comprehensive tool is needed to identify gap in service delivery and to prioritize monitoring visits. OBJECTIVES: To supplement the existing monitoring system, identify strengths/weakness of ART centers, and give recommendations. METHODOLOGY: Scorecard was developed in spreadsheet format with 17 scoring indicators on monthly base from March 2014 onward. The centers were classified in three color zones: green (score ≥80%), yellow (score <80% and ≥50%), and red (score <50%). Visits were prioritized at centers with more indicators in yellow/red zone. The performance of centers was compared for March 2014 and March 2015. RESULTS: The statistically significant improvement was observed in indicator "ART initiation within 2 months of eligibility," while after removing red zone from analysis, four more indicators named "eligible patients transferred out before ART initiation, general clients started on ART, antenatal women started on ART, and pre-ART follow-up CD4 done" reflect statistically significant improvement. Quadrant analysis was done for some indicators, which provide insight that less number of eligible patients may be a reason for low initiation of ART at one center, and at four other centers, the possible reasons for low retention are high death rate and high lost to follow-up rate. Based on these findings, the recommendations were made to regular mentoring centers, improve coordination between ART center and care and support centers (CSCs), and conduct verbal autopsy. CONCLUSION: Scorecard is a simple and cost-effective tool for monitoring, and by highlighting low-performing indicators, it helps in improving quality of services provided at ART centers.

Gelatinous Transformation of Bone Marrow: A Prospective Tertiary Center Study, Indicating Varying Trends in Epidemiology and Pathogenesis.

Gelatinous bone marrow transformation (GTBM) also known as serous atrophy is a degenerative change in the hematopoietic bone marrow and is a rare well recognized pathological entity. It was earlier described mainly in association with anorexia nervosa and psychiatric eating disorders, but recently it has been reported in ulcerative colitis, tuberculosis, chronic renal diseases, immuno suppressed states (mainly HIV infection), malignancies and Kala azar. Treatment is based on treating the underlying disease. Our objective was to study the epidemiology and pathogenesis of diseases causing gelatinous transformation of bone marrow, at a tertiary center level. A prospective study was conducted on 732 samples of bone marrow aspirate with the aim of identifying gelatinous transformation in bone marrow aspirates which was confirmed by Alcian blue stain at pH 2.5. The presence of this material was correlated with the hematological profile of the patient, the presenting signs and symptoms and provisional clinical diagnosis at initial visit. Incidence of gelatinous transformation was calculated to be nearly 4.8 % and the condition was more common in males (23) as compared to females (12) (Male:Female = 2:1). Forty percent of the cases were seen in children followed by 37 % in adolescents and young adults. The older individuals comprised only 23 % of the cases. The bone marrow was hypocellular in 21 (60 %), normocellular in 10 (28.5 %) and hypercellular in four cases (11.5 %). Five cases with GTBM progressed to aplastic anaemia of which three were in children.

Primary Ewing's sarcoma of cervical vertebra: An uncommon presentation.

Ewing's sarcoma is a malignant primary bone tumor primarily seen in the long bones. Primary Ewing's sarcoma of the cranium is quite uncommon occurring in 1% of the cases. We report the occurrence of this rare lesion in a 24-year-old male presenting with progressively increasing swelling in left mastoid region mimicking a mastoid abscess which was later diagnosed on Fine needle aspiration cytology (FNAC) as a small round cell tumor as Ewing's sarcoma. Contrast enhanced computed tomography (CECT) revealed a typical moth eaten appearance in the first and second cervical vertebra.

Oncocytic carcinoma of lip: A rare neoplasm of minor salivary gland.

Oncocytic carcinoma is an extremely rare neoplasm of the salivary gland, with only a few cases reported in literature till date. We report the occurrence of this rare lesion in lip in a 43-year-old female presenting with a progressively increasing swelling for which excision was done. Fine needle aspiration was done and the smears revealed tumor cells with well-defined cell borders, round to oval, central to eccentrically located moderately pleomorphic nuclei with fine chromatin, prominent nucleoli and abundant eosinophilic granular cytoplasm. Microscopic examination of the resected tumor showed solid sheets, nests, islands and cords of oncocytic cells diffusely infiltrating the surrounding tissues. After 5 months, the patient again presented with bilateral submandibular and right axillary lymphadenopathy revealing metastatic deposits from oncocytic carcinoma. We report this case of oncocytic carcinoma because of its unusual location, the minor salivary gland of lip being a rare site for the tumor.

Cytodiagnosis of histoplasmosis: case reports from two patients with variable clinical presentation.

Histoplasmosis has emerged as an important opportunistic fungal infection in immunocompromised patients. Histoplasma is a dimorphic fungus that primarily involves lung and the environmental reservoir is soil. Although several cases of histoplasmosis have been reported in India but cytological diagnosis was made in a few cases. We are presenting two cases of histoplasmosis diagnosed on fine-needle aspiration cytology. In the first case, pulmonary histoplasmosis was diagnosed on transbronchial needle aspiration of lung in a 41-year-old immunocompetent male, while second case was of disseminated histoplasmosis in 40-year-old immunocompromised female diagnosed on cytology of cervical lymph node. FNAC is a simple, safe, and rapid technique to establish the initial diagnosis, thus promoting early treatment and favorable outcome especially in the immunocompromised patients.

Variable clinical presentations of histoplasmosis: a report of six cases.

Histoplasma is a dimorphic fungus that primarily involves the lungs and the environmental reservoir is soil. It has emerged as an important opportunistic fungal infection in immunocompromised patients. Six cases of histoplasmosis with variable clinical presentations diagnosed either on cytology or histopathology are discussed - three were HIV-positive. The possibility of histoplasmosis should always be borne in mind, especially in immunocompromised patients, as it can have variable clinical presentations.

Rosai-Dorfman disease: A case report with extranodal thyroid involvement.

Rosai-Dorfman Disease (RDD) or Sinus Histiocytosis with Massive Lymphadenopathy (SHML) is a rare disorder typically manifesting as enlarged lymph nodes with or without systemic involvement. These cases are often clinically misdiagnosed as lymphoma. Recognising this entity to distinguish it from lymphoma and other causes of histiocytosis is important because of different treatment modalities for these disorders. Extranodal involvement is also common, often with a particular predilection for the head and neck region. We report a rare case of Rosai-Dorfman Disease with both nodal and extranodal involvement in a 33-year-old woman. The patient had bilateral cervical lymphadenopathy and diffuse thyroid enlargement. Thyroid gland involvement in RDD diagnosed on fine needle aspiration cytology (FNAC) has rarely been reported in literature. FNAC is a useful and reliable tool for the diagnosis of RDD and the biopsy can be avoided in these patients, thus reducing inconvenience to patients.

Oral cysticercosis-a rare presentation.

Cysticercosis is a condition that occurs when man is infected with larvae of Taenia solium. Oral cysticercosis is a rare event, and it represents difficulty in clinical diagnosis. A case of oral cysticercosis in 11 year old girl is presented which complained of painless swelling for 6 months. A FNAC was performed which revealed bluish pink fibrillary material and interspersed nuclei and fragments of wall of larvae. Patient was treated with antihelminthic.

Inflammatory psuedo-tumor of the spleen.

Inflammatory pseudo-tumor of spleen, a benign reactive lesion of unknown etiology and pathogenesis, is extremely rare with isolated case reports in literature. These are usually misdiagnosed preoperatively, both clinically and radiologically; metastasis or lymphoproliferative disorders with pathological studies allow reliable diagnosis of the disease. We report the unusual occurrence of this lesion in the spleen.

Transferrin receptor expression in reactive and neoplastic lesions of lymphnodes.

Transferrin, the major iron binding protein in human plasma transports iron to various tissues. The first step in cellular iron uptake is binding of transferrin complex to the cell surface membrane by specific molecule known as transferrin receptors. Transferrin receptors are found in limited sites in normal tissues, in contrast, the receptors are widely distributed in majority of carcinomas and sarcomas. Presence of increased transferrin receptors implies a stage of moderate or less differentiation corresponding to elevated proliferative activity and therefore, has a prognostic value. Demonstration of transferrin receptors and its distribution pattern within a tumour as well as its quantitative determination can provide data helpful for, both, an additional understanding of tumour biology and as an approach for planning therapy. In present study, we analysed 60 cases, 30 each of reactive lymphadenitis and lymphomas for transferrin receptors using immunohistochemical technique (DAKO, Code-K0673). Grade II and Grade III intensity was recorded in the germinal centers and the histiocytes in sinus histocytosis indicating the proliferating cells and activated histocytes. Most of the low grade non-Hodgkin lymphomas (83.66%) showed weak (Grade I) positivity for transferrin receptors. Intermediate grade lymphomas showed moderate (Grade II) to high intensity (Grade III) for transferrin receptors (57.14% and 42.85%) respectively. Seventy five percent of high grade lymphomas showed strong (Grade III) positivity. All the 9 cases of Hodgkin lymphoma (100%) showed grade III positivity. Proportion of the cells within a tumour expressing transferrin receptors in high density are therefore likely to represent the growth fraction of the tumour.

Role of cyclodextrins in ophthalmics.

Cyclodextrins are oligosaccharides having outer hydrophilic surface and central hydrophobic cavity. These agents form inclusion complexes with poorly water-soluble drugs; hence they show an important implication for use in ophthalmics because of their applications in solubilising and stabilising the ocular drugs. Most of the drugs being used in ophthalmics were not tailor-made for the eye and considering the poor bioavailability of <1% from the corneal surface, presentation of the drug in a soluble form and at high concentration is important. Provision of a high drug concentration at the corneal surface increases the percent drug permeation indicating the usefulness of cyclodextrins as penetration enhancers. A decrease in irritation potential of some drugs upon incorporation of cyclodextrins is also reported. Polymer-cyclodextrin multicomponent systems further extend the role of cyclodextrins in improving the solubility and bioavilability of ocular drugs. Large hydrophilic cyclodextrins like hydroxypropyl-beta-cyclodextrin and sulphobutylether-beta-cyclodextrin are safe for the use in aqueous eye drop solutions especially since they do not cross the lipophilic cornea. Various aspects about the applications of cyclodextrins in ophthalmics, the formulation considerations and expected toxicity of cyclodextrins (especially if high concentration is used) is discussed in this review. Strategies like use of polymers to reduce the effective concentration of cyclodextrin required without compromising solubility are also included. Further the concept of incorporating the drug-cyclodextrin complexes into liposomes or niosomes for a better targeting of the drug at appropriate tissue destination is discussed as a possible future option.