Renu G’Boy Varghese

Successful treatment of extensive basidiobolomycosis with oral itraconazole in a child

Background  Basidiobolomycosis is a rare chronic subcutaneous infection caused by Basidiobolus ranarum, which is usually treated with potassium iodide. Extensive deforming lesions in children can occur owing to lack of early diagnosis and/or inappropriate treatment.

Comparative Evaluation of Simultaneous Bone Marrow Aspiration and Bone Marrow Biopsy: An Institutional Experience

Bone marrow aspirations and bone marrow biopsies are important diagnostic procedures. A comparative study of both the procedures done simultaneously was retrospectively reviewed in 160 cases where the clinical history is correlated with BMA and BMB results. The advantage of each method is analyzed. Correlation of our findings with that given in the literature is done to give a guideline for both techniques. We have found that 61.25% of the cases showed a positive correlation between bone marrow aspiration and bone marrow biopsy. However, we found that tuberculous granulomas and Hodgkin disease involvement of the marrow were detected better in bone marrow biopsies. The advantage of both the procedures done together provided more material and enabled us to study the cytomorphology of the cells, with the pattern of distribution of the cells depending on the cases. However, when both the procedures are done simultaneously, a proper technique is required so as to yield good diagnostic material.

Hemophagocytic lymphohistiocytosis: An unusual complication in disseminated Mycobacterium tuberculosis.

Background: Hemophagocytic lymphohistiocytosis (HLH) is an uncommon, potentially fatal, hyperinflammatory syndrome that may rarely complicate the clinical course of disseminated Mycobacterium tuberculosis (MTB). The clinical course of tuberculosis-associated HLH (TB-HLH) has been reported to be unpredictable. Materials and Methods: Here we describe the clinicopathological features, laboratory parameters, management, and outcome data of a patient who satisfied the 2004 diagnostic criteria for HLH secondary to disseminated MTB; we also do a systematic review of the international literature on TB-HLH. The literature review (January 1975–March 2014) found that HLH complicated the clinical course of 63 tuberculosis patients (41 males, 22 females, mean age = 45 ± 23.5 years) with a high mortality rate of 49% (31/63 died). The mean serum ferritin level (n = 44/63) was 5963 ng/mL (range 500–38,539 ng/mL); and a higher proportion (54.2%) of patients had pancytopenia at presentation. On univariate analysis (n = 53/63), age >30 years [hazard ratio (HR): 2.79, 95% confidence interval (CI):1.03–7.56, P = 0.03], presence of comorbidities (HR 4.59, CI: 1.08–19.52, P = 0.04), marked hemophagocytosis in bone marrow (HR: 2.65, CI: 1.16–6.05, P = 0.02), and nonusage/delayed usage of antitubercular therapy (ATT) (HR: 3.44, CI: 1.51–7.87, P = 0.003) were associated with decreased survival, though none of these parameters attained statistical significance (P > 0.05) in multivariate analysis. Usage of corticosteroids and/or immunomodulator drugs (HR 1.00, CI: 0.66–3.22, P = 0.35) did not alter the outcome in these patients. Conclusion: HLH should be considered as a differential diagnosis in patients with tuberculosis who present with cytopenias, organomegaly, and coagulopathy. Strong clinical suspicion and early usage of ATT might be useful in reducing the morbidity and mortality. The utility of immunosuppressive/immunomodulator therapy lacks general concensus among treating physicians, and warrants further studies.

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.

Cyclin D1 expression in multiple myeloma by immunohistochemistry: Case series of 14 patients and literature review.

Background: Cyclin D1 dysregulation is an early and unifying oncogenic event in patients of multiple myeloma (MM). This may be detected up to 30% cases by immunohistochemistry (IHC) and up to 40-50% cases by molecular studies. However, studies on the clinical significance of cyclin D1 dysregulation in MM have been inconclusive. We aimed to study the pattern of cyclin D1 expression in MM by IHC and correlate with selected clinicopathologic features. Materials and Methods: Formalin fixed, decalcified, bone marrow trephine sections from 14 symptomatic patients of MM (13 newly diagnosed and one relapsed) were subjected to cyclin D1 IHC by using a rabbit monoclonal antibody to cyclin D1 (clone EPR2241). Results: Cyclin D1 expression (in ≥10% tumor cell nuclei) was observed in 8 of 14 cases (57%). Cyclin D1 positive (+) group had significantly lower hemoglobin level (P = 0.03) than cyclin D1 negative (−) group (n = 6); though both groups showed no statistical significance (P > 0.05) in regard to age, gender, Durie and Salmon stage, lytic bone lesions, light chain phenotype, creatinine, calcium, lactate dehydrogenase, leukocyte and platelet count and bone marrow histology. Ten of 14 (71.5%) showed a favorable response (follow-up; 7 days to 34 months) to thalidomide and/or bortezomib based chemotherapeutic regimen. Four of eight cyclin D1− patients showed complete response, two had a partial response (PR) and two died of the disease; whereas 4/6 cyclin D1 − patients had PR, one refused definitive therapy and one was lost to follow-up (P > 0.05, Fischers exact test). Conclusion: IHC may be a feasible tool for the demonstration of cyclin D1 expression on adequately processed trephine biopsy specimen in MM patients in a resource poor setting. Negative IHC results should be correlated with molecular techniques for prognostication.

Pulmonary alveolar microlithiasis: an interesting case report with systematic review of Indian literature

Pulmonary alveolar microlithiasis is a rare disease characterized by intra-alveolar presence of microliths. This study reports an interesting case of pulmonary alveolar microlithiasis and provides a systematic review of cases reported from India. A 23-year-old female presented with a history of cough, wheeze, chest pain, and episodic wheeze for five months. Pulmonary function tests demonstrated an obstructive pattern, and chest Xray showed fine micronodular opacities predominantly involving the middle and lower zones of both lungs. Transbronchial lung biopsy revealed the diagnosis. She responded well to inhaled steroid therapy. A systematic review of literature was performed and identified 73 cases of pulmonary alveolar microlithiasis reported from India. The mean (SD) age of the patients was 28.8 (14.9) years, with an almost equal male:female ratio. Many patients were asymptomatic at presentation. Breathlessness and cough were the most common symptoms, and the disease progressed into respiratory failure associated with cor pulmonale. About one-third of the cases were initially misdiagnosed and treated as pulmonary tuberculosis. Extra-pulmonary manifestations and comorbidities were also evident in our series. This systematic review helps to determine epidemiological and clinical characteristics of pulmonary alveolar microlithiasis. Further research is needed to elucidate the etiopathogenesis, diagnosis, and therapeutic options, which are beneficial in developing and identifying costeffective treatment for pulmonary alveolar microlithiasis.

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.

Plasmablastic multiple myeloma following clear cell renal cell carcinoma

We aim to describe the clinicohaematological profile of an elderly male with plasmablastic multiple myeloma (MM) (IgG λ, International System Stage II) with an unfavourable outcome following chemotherapy. The serum interleukin-6 level was found to be markedly elevated (2464 pg/mL, reference; <50 pg/mL). Thirty-six months prior to MM diagnosis, he underwent left radical nephrectomy for a stage III (pT3N0M0) clear cell renal cell carcinoma (RCC, Fuhrman grade 2). The unique MM-RCC association, shared risk factors, myeloma pathobiology and clinical implications are discussed with a brief literature review.

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.