De novo chronic lymphocytic leukemia/prolymphocytic leukemia or B-cell prolymphocytic leukemia? The importance of integrating clinico-morphological and immunophenotypic findings in distinguishing chronic lymphoproliferative diseases with circulating phase

B-cell prolymphocytic leukemia (B-PLL) is an extremely rare disease, accounting for approximately 1% of the lymphocytic leukemias. B-PLL generally occurs in older people. It is characterized by the presence of more than 55% prolymphocytes in the peripheral blood (PB), no or minimal lymphadenopathy, massive splenomegaly, and very high white blood cell counts. The prognosis of B-PLL patients is generally poor, with a median survival of 3 years, although a subset of patients may show a prolonged survival. Herein, we report a case of a 70-year-old male with weakness, generalized lymphadenopathy, and moderate splenomegaly at the initial presentation. Hematologic examination revealed lymphocytic leukocytosis, favoring a chronic lymphoproliferative disorder (CLPD). The key to decoding the precise CLPD was a combination of the clinical profile, morphologic findings on the peripheral blood and the bone marrow, immunophenotypic analysis, and cytogenetic study. The best diagnosis proffered was a de novo chronic lymphocytic leukemia/prolymphocytic leukemia. There was no prior history of lymphoproliferative disorder or lymphocytic leukocytosis. Discriminating this entity from other lymphoproliferative disorders is crucial as the treatment and prognosis are varied compared to the other lymphoproliferative disorders. The diagnostic conundrum encountered and the incredible utility of ancillary studies in such a scenario are highlighted in this study

Henoch-Schonlein Purpura in Northeast India: Peculiarities in Presentation

Background and Aim: Henoch-Schönlein purpura (HSP) is the most common childhood vasculitis characterized by leukocytoclastic vasculitis. This study was done to describe the presentation and immediate outcome of children admitted to HSP at our Institute. Methods: This retrospective study was conducted on children with HSP admitted to our department over a period of 7 years (January 2010 until December 2016). Results: Twenty-three children with a diagnosis of HSP were identified during the study period. The mean age was 9.4 years (4 years to 16 years). There were 15 girls and 8 boys with a male: female ratio of 1:1.9. The youngest child was 4 years old and most of the children (73.9%) were in the age group 5-12 years. Forty percent of the children presented between January and March. Major manifestations were rash (100%), joint pain (52%), renal involvement (52%), and abdominal pain (47.8%). Three (13.0%) children presented with systemic manifestations before the appearance of the rash. One child had MPGN 2 years before the onset of rash. There was no mortality. Most of the children recovered well; six (26%) had persistent hypertension and three (13%) had persistent proteinuria. Hypertensive emergency was seen in two children. One child had intussusception that resolved spontaneously. Conclusion: This study is the first study of Henoch Schonlein purpura from northeast India documenting certain peculiarities in the presentation. The results indicate a wide spectrum of presentations in HSP

Antenatal Screening for Hemoglobinopathies with HPLC

Introduction: Hemoglobinopathies are the most commonly encountered monogenic disorders of blood in Southeast Asia and Indian subcontinent. Screening of individuals at increased risk of being carriers for thalassemia and hemoglobinopathies, can identify couples with a 25% risk of having a pregnancy with a significant genetic disorder, for which prenatal diagnosis is possible. This study is done to know the prevalence of hemoglobinopathies and variant of haemoglobin using cation exchange high performance liquid chromatography (CEHPLC).Materials and Methods: 2 ml of venous blood was collected in EDTA vials from the pregnant mothers after informed consent. The blood was subjected to complete hemogram, peripheral blood smear and HPLC using Variant Hemoglobin Testing System (BioRad Laboratories). Beta Thalassemia short programme was used. Descriptive analysis was done and data is presented in numbers and percentages.Results: 467 blood samples from various ethnic groups were evaluated. 70 (14.99%) samples showed features of hemoglobinopathies by HPLC. There were 46 (9.85%) cases of HbE heterozygous,12 (2.57%) cases of HbE homozygous, 9(1.93%) cases of Beta Thalassemia Trait, 2 (0.43%) cases of double heterozygous and 1 (0.21%) case of Hb-D Iran.Conclusion: This study showed a high prevalence of hemoglobinopathies in antenatal mothers necessitating an appropriate screening strategy for antenatal mothers. We also concluded that HPLC is a sensitive technique for studying hemoglobinopathies during pregnancy and may be utilized for screening

Pediatric Granular Cell Tumor of the Breast: An uncommon neoplasm in an uncommon site and age group

Granular cell tumor (GCT) is a rare soft tissue neoplasm of Schwann cell origin. Most cases occur in adults; however, the precise incidence is unknown in children. GCT is usually a slow-growing, painless tumor involving the skin and soft tissues that is mostly located in the head and neck region, especially the tongue. The breast is one of the least common sites involved by GCT. This paper presents a 3-year-old girl who presented with a soft to firm, ill-defined swelling on the right breast with painful ulceration of the overlying skin. Fine needle aspiration rendered an initial diagnosis of fibrocystic change accompanied by apocrine metaplasia. Histologic evaluation of the excised breast mass revealed a benign granular cell tumor. Although rare, GCT of the breast should be included in the differential diagnosis for breast masses in pediatric patients. Proper diagnosis and timely management of this tumor are essential because of its malignant potential (<2% of cases) and high rate of local recurrence if not properly excised

Demographic pattern and histopathological profile of neuroendocrine neoplasms diagnosed at a tertiary care center in North East India

Background: Neuroendocrine tumors (NENs) are a group heterogenous group of tumors that can arise in any organ in the body and have a wide range of aggressiveness. Aims and Objective: To compare the frequency of NENs in our setup to those reported in the literature to age, site, and degree of differentiation by doing a retrospective study. Materials and Methods: Cases of NENs that were diagnosed in the Department of Pathology, in our hospital over the past 5 years were studied considering the age, location, and degree of differentiation. A fresh panel of immunohistochemistry (IHC) was conducted for those cases where IHC was not done. Results: A total of 46 cases of NENs were reviewed. About 56.5% (26 cases) were males and 43.5% (20 cases) were females, with a median age of 46 years. In our study, most of the tumors are found in the gastro-entero-pancreatico-hepatobilliary group followed by the NENs of the endocrine gland (21.7%) and broncho-pulmonary group (15.2%). NENs were graded based on mitotic count and/or Ki-67 labeling index, and/or the presence of necrosis. In total 21 cases (45.7%) had G1 grading, 7 cases (15.2%) had G2 grading, 2 cases (4.3%) had a G3 grading, and 16 cases (34.7%) were graded as neuroendocrine carcinoma. Conclusion: As the majority of the studies do not include benign NENs and those arising from the endocrine glands, therefore comparison of our results can be difficult. This is the first attempt to study the NENs from North East India and to analyze their clinicopathological features

Primary Tuberculosis of Cervix: A Coincidental Finding

Abstract Background: 95% of Tuberculosis (TB) of the female genital tract (FGT) is located in tissues other than the cervix. A rare case of primary TB of the cervix which was diagnosed coincidently in a patient of endometrioma was reported in this study. Case Presentation: A 34 year old nullipara, a diagnosed case of endometrioma had a small cervical growth. Pap smear and biopsy was taken and sent for histopathological examination. Her histopathological examination revealed multiple epitheloid cell granuloma and langerhans type giant cell caeseation. Ziehl neelsen staining was positive for acid fast bacilli (AFB). The patient was started on antituberculosis under directly observed therapy along with oral contraceptive pills. Patient was on regular follow-up and clinically she was doing well. Conclusion: Although cervical tuberculosis is very rare but for an abnormal looking cervix, cervical tuberculosis should be considered in the differential diagnosis in woman of all the age groups especially in areas where tuberculosis is rampant since these cases are potentially curable with medical therapy. Many of these patients are in reproductive age group. In young women, early diagnosis prevents further damage in reproductive tract and can improve their reproductive potential. Introduction enital TB is still a major socioeconomic burden in resource poor settings. 95% of TB of the female genital tract (FGT) is located in tissues other than the cervix. A rare case of primary TB of the cervix which was diagnosed coincidently in a patient with endometrioma was reported in this study

Malignant Melanoma of the External Auditory Canal:A Rare Entity

Introduction: Although malignant melanomas (MM) are common in the head and neck region; primary malignant melanoma of the external auditory canal (EAC) is rare.   Case Report: We present the case of a 50-year-old symptomatic man with a malignant melanoma of the external auditory canal, which clinically masqueraded as a haemangioma. The patient subsequently developed extensive loco-regional metastasis, requiring extensive surgery. We describe the clinical presentation, differential diagnosis, both clinical and pathological in terms of other pigmented lesions in the external auditory canal, detailed histopathology, and literature review.   Conclusion: We want to emphasize the importance of immediate and adequate biopsy of any pigmented lesion in the head and neck region to rule out  MM. Also, we emphasize the importance of deep biopsy for proper histopathological assessment in addition to distinguishing it from benign melanocytic nevi, in order to initiate treatment

Clinico-pathological profile of ovarian cysts in a tertiary care hospital

Background: Ovarian cystic neoplasms are common in gynaecological practice. These may pose diagnostic difficulty to the pathologists. This study was conducted to analyse the clinical and histological profile of ovarian cystic neoplasms.Methods: This is a retrospective study done from January 2016 to April 2017 in a tertiary care hospital in North East India. All the patients, who were clinically and radiologically diagnosed as ovarian cysts, which had histopathological confirmation were included in the study. Data including the age, parity, clinical symptoms, laterality and histopathological findings were analysed.Results: A total of 101 patients operated for ovarian cysts in the study period were analysed. The most common clinical presentation was lower abdominal pain. There were 11 (10.9%) malignant cases, 4 (4%) were intermediate grade and borderline in nature, and 85 (84.1%) cases were benign in nature. There was 1 (1%) case of metastasis to ovary. Mature cystic teratoma was most common (20.8%) histopathological diagnosis. The second most common cyst was serous cystadenoma (19.8%).Conclusion: Ovarian cysts are commonly encountered in gynaecological practice and equally encountered by the pathologists. Most commonly found ovarian cysts were mature cystic teratoma followed by serous cystadenoma