Manjiri Phansalkar

Hemophagocytic lymphohistiocytosis: critical reappraisal of a potentially under-recognized condition

Hemophagocytic lymphohistiocytosis (HLH) is an uncommon, potentially life threatening, hyper inflammatory syndrome of diverse etiologies. Cardinal signs include prolonged fever, organomegaly, and persistent unexplained cytopenias. In spite of the well known diagnostic criteria put forth by HLH society, this continues to pose great diagnostic challenge in both pediatric and adult intensive care settings. We describe 4 adult (2 males, 2 females, aged 19, 29, 40, and 17 years) and 3 pediatric (2 males, 1 female, aged 1 month, 6 months, and 12 years) patients with secondary HLH who satisfied the HLH-2004 diagnostic criteria. Definite evidence of hemophagocytosis was noted in 4 patients on initial bone marrow examination. The underlying etiologies were as follows: Rickettsia tsutsugamushi (case 1), autoimmune disorder (case 2), systemic onset juvenile idiopathic arthritis (sJIA) (case 3), unknown bite (possibly a venomous snake) (case 4), Plasmodium vivax (case 5), Cytomegalo virus (case 6), and Mycobacterium tuberculosis (case 7). In one patient, hemophagocytosis was presumed to have been exacerbated by administration of granulocyte monocyte colony stimulating factor (GMCSF) for severe neutropenia. Two patients died with disseminated intravascular coagulation (DIC) and multi organ failure within few days of HLH diagnosis. Immunosuppressive therapy was started in 3 patients, and etoposide was started in one patient only. Due to lack of specificity of diagnostic criteria, diagnosing and differentiating HLH from its closest mimickers like sepsis/septic shock may be quite challenging in critically ill patients. Therefore, increasing awareness among physicians is essential for early diagnosis and effective therapy to reduce the mortality.

Jejunal diverticulosis with perforation - a challenging differential diagnosis of acute abdomen: case report.

Multiple diverticulosis of the jejunum represents a very rare entity. Jejunal diverticula are found to be the rarest of all small bowel diverticula. The disease is usually asymptomatic and often becomes clinically relevant when complicated. This rarity makes it a difficult differential diagnosis. Related complications such as diverticulitis, perforation, and bleeding and/or intestinal obstruction appear in about 10-30% of the patients which increase the morbidity and mortality rates in such individuals. Here, we present a case of jejunal diverticulosis with perforation who presented with symptoms of acute abdominal pain, vomiting and fever along with a brief review of literature.

Correlation of narrow band imaging endoscopy and histopathology in the diagnosis of nonerosive reflux disease.

Background /Aim: Narrow band imaging (NBI) is a novel, innovative high-resolution endoscopic technique, which utilizes spectral narrow band filter for the visualization of mucosal patterns and microvasculature. Nonerosive reflux disease (NERD) is a type of gastroesophageal reflux disease (GERD) and it is characterized by reflux symptoms without mucosal breaks on white light endoscopy (WLE). Biopsies from distal esophagus of GERD patients show group of histologic features such as basal cell hyperplasia, elongation of lamina propria papillae, and inflammatory cells. The present study was undertaken to evaluate diagnostic utility of NBI endoscopy and biopsy study in NERD patients and also to correlate NBI endoscopy findings with histologic features of GERD. Patients and Methods: A total of 71 cases of NERD having symptom score more than 10 and those not having erosion on WLE were recruited prospectively and underwent NBI endoscopic examination. Two mucosal biopsies were taken at 3 cm above the squamocolumnar junction. Results: Histologic features of GERD were seen in 50 (70.4%) out of 71 cases. No significant correlation between NBI endoscopic findings with histologic features of GERD was found. Conclusion: The present study showed that histopathologic evaluation of distal esophageal mucosa has promising diagnostic value over NBI endoscopy in NERD patients. Use of newly introduced NBI technique requires tremendous familiarity for the detection of the cases of NERD, which show histologic features of GERD.Background /Aim: Narrow band imaging (NBI) is a novel, innovative high-resolution endoscopic technique, which utilizes spectral narrow band filter for the visualization of mucosal patterns and microvasculature. Nonerosive reflux disease (NERD) is a type of gastroesophageal reflux disease (GERD) and it is characterized by reflux symptoms without mucosal breaks on white light endoscopy (WLE). Biopsies from distal esophagus of GERD patients show group of histologic features such as basal cell hyperplasia, elongation of lamina propria papillae, and inflammatory cells. The present study was undertaken to evaluate diagnostic utility of NBI endoscopy and biopsy study in NERD patients and also to correlate NBI endoscopy findings with histologic features of GERD. PATIENTS AND METHODS A total of 71 cases of NERD having symptom score more than 10 and those not having erosion on WLE were recruited prospectively and underwent NBI endoscopic examination. Two mucosal biopsies were taken at 3 cm above the squamocolumnar junction. RESULTS Histologic features of GERD were seen in 50 (70.4%) out of 71 cases. No significant correlation between NBI endoscopic findings with histologic features of GERD was found. CONCLUSION The present study showed that histopathologic evaluation of distal esophageal mucosa has promising diagnostic value over NBI endoscopy in NERD patients. Use of newly introduced NBI technique requires tremendous familiarity for the detection of the cases of NERD, which show histologic features of GERD.

Amoeboma: resurfacing of a vanishing illness

Amoeboma is a rare manifestation of intestinal amoebiasis. More than 90% of patients with intestinal amoebiasis have a history of diarrhoea. This report describes the case of a 60-year-old patient who presented with a right iliac fossa (RIF) mass with normal bowel habits and was eventually diagnosed with an amoeboma. In developing countries, a traditional differential diagnosis for an RIF mass is an amoeboma, but its incidence is declining. Hence this treatable condition is often overlooked in the differential diagnosis of an RIF mass. This case report emphasises that amoeboma still exists and should be considered in a patient with an RIF mass.

Ovarian Leiomyoma Along with Uterine Leiomyomata: A Common Tumour at an Uncommon Site.

Ovarian leiomyoma is one of the rarest benign tumours of the ovary, mostly seen in women of reproductive age group. Here we report a case of ovarian leiomyoma as an incidental finding in a patient of 38-year-old woman with uterine leiomyomata. Peroperatively, her left ovary appeared bulky & she underwent total abdominal hysterectomy with left salpingo-oophorectomy. Macroscopically, in addition to uterine leiomyomata, a grey-white solid mass was seen entirely within the ovary without any capsular breach. Microscopically, the ovarian mass resembled its uterine counterpart without any evidence of atypia or necrosis. Masson trichrome stain & immunohistochemistry for desmin positivity confirmed the smooth muscle origin of the tumour cells. Despite its rarity, ovarian leiomyoma has to be considered as one of the differential diagnosis of ovarian spindle cell tumours. In difficult cases, immunohistochemistry aids the diagnosis.

Endoscopic (video) demonstration of hookworm infestation of the stomach

Hookworm infestation is common in southern India. The adult worms normally reside in the duodenum, graze the intestinal mucosa with their large buccal cavities and ingest the intestinal epithelial cells and blood. Depending on the number of worms the infection may be either asymptomatic or can cause iron deficiency anaemia due to chronic blood loss. Adult worms live in the small intestine and are not usually seen in the stomach. There are only very few case reports in the literature reporting hookworm infestation of the stomach. In this case report we present an endoscopic (video) demonstration of hookworm infestation of the stomach in a woman who presented with chronic anaemia.

Subcutaneous Fungal Cyst Masquerading as Benign Lesions - A Series of Eight Cases.

BACKGROUND Subcutaneous fungal infections are caused by penetration of the causative fungi into the subcutaneous layer and are usually localised. We present a series of eight cases with subcutaneous fungal cystic lesions masquerading as benign lesions. MATERIALS AND METHODS A retrospective study was conducted on subcutaneous fungal infections seen between January 2007 to July 2014 in the Department of Pathology. Eight patients with biopsy proven subcutaneous fungal infection were included. We collected and analysed their demographic, clinical and histopathological details. RESULTS Among eight patients, six were male and two were female. The mean age was 47 years (Range: 21-70). All the eight patients presented with non-tender cystic swelling. The size of the swellings varied from a minimum of 3x3 cm to maximum of 10x4 cm. Out of eight, hand was involved in three, forearm in one, elbow in two, leg in one and foot in one. On H&E staining, all the cases showed fibro collagenous cyst wall, lined by histiocytes, granulomatous reaction, foreign body type of giant cells with acute and chronic inflammatory infiltrate containing fungal elements. Six were identified as hyalohyphomycosis and two were identified as phaeohyphomycotic cysts based on pigmentation of hyphae. CONCLUSION Fungal infection should be suspected in all subcutaneous cystic lesions. Excised tissue should always be sent for culture and histopathology.

Bone Marrow Granuloma in Typhoid Fever: A Morphological Approach and Literature Review

Typhoid fever is one of the few bacterial infections in humans where bone marrow evaluation is routinely recommended. However, the morphological aspect of typhoid fever in bone marrow has been rarely described in the literature. We describe a 25-year-old male patient who presented with prolonged fever suspected to be of tubercular etiology. Bone marrow examination showed well-formed histiocytic and epithelioid granulomas and erythrophagocytosis; and the bone marrow aspirate culture grew Salmonella typhi A. In view of potential clinical implications, typhoid fever should be considered as a differential diagnosis to tuberculosis in the evaluation of prolonged fever; especially in high prevalent areas. We suggest that erythrophagocytosis may serve as a morphological marker in typhoid granulomas in the bone marrow; and bone marrow culture should be submitted in every suspected case for appropriate patient management.