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.

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 (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.

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This case report highlights that in an older child even longstanding extensive basidiobolomycosis can be safely treated with itraconazole. Surgery is not usually necessary in these patients.

A 56-year-old male presented with dragging upper abdominal pain of one week duration. There was a history of dyspepsia, abdominal discomfort and vomiting after meals for past one week and low grade fever associated with chills and rigors.On examination, the patient was febrile with a temperature of 99 0 F, pulse rate -110/min, blood pressure -130/90 mm Hg. Abdominal examination revealed generalized tenderness with fullness over the right hypochondrium.Laboratory work up revealed Hb-13g/dl, TLC-10,000/cu.mm, DLC -P90 L7 E1 M2, urea -49mg/dl, creatinine -0.5mg/dl. Abdominal radiograph revealed air under the diaphragm. Abdominal CT scan and ultrasound showed multiple diverticuli in the small intestine [Table/ Fig-1] and air under the diaphragm suggesting perforation.Emergency midline laparotomy was performed. Intra-operatively multiple jejunal diverticuli were found on the mesenteric border. They were located between 8 cm to 20 cm from duodeno-jejunal (DJ) flexure. Serosal purulent exudates and multiple inter-loop adhesions were noted. There was purulent material in the peritoneum as well. Proximal jejunal resection with end to end anastomosis was done. The post operative period was uneventful and the patient was discharged on 10 th day after the surgery. One month follow up was uneventful.Gross examination of resected specimen revealed a segment of small bowel, 65 cm in length with eight outpouchings on the serosal aspect along the mesenteric border, of which largest measured 4x3x1 cm with focal areas of exudates on the serosal aspect and adhesions; smallest measured 2x1.5x0.5cm [Table/ Fig-2]. Three ABStRACtMultiple 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.

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, Fischer's 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.

BackgroundHemophagocytic lymphohistiocytosis (HLH) is an uncommon, potentially fatal, hyperinflammatory syndrome that may rarely complicate the clinical course of Orientia tsutsugamushi disease (scrub typhus).MethodsHere we describe the clinicopathological features, laboratory parameters, management, and outcome of three adult patients (1 female, 2 males) with scrub typhus associated HLH from a tertiary center. A brief and concise review of international literature on the topic was also added.ResultsAll three patients satisfied the HLH-2004 diagnostic criteria; one had multi-organ dysfunction with very high ferritin level (>30,000 ng/ml), and all had a dramatic recovery following doxycyclin therapy. Literature review from January 1990 to March 2014 revealed that scrub typhus associated HLH were reported in 21 patients, mostly from the scrub endemic countries of the world. These included 11 females and 10 males with a mean age of 35 years (range; 8 months to 81 years). Fifteen of 17 patients (where data were available) had a favorable outcome following early serological diagnosis and initiation of definitive antibiotic therapy with (N=6) or without (N=9) immunosuppressive/immunomodulator therapy. Mutation analysis for primary HLH was performed in one patient only, and HLH-2004 protocol was used in two patients.ConclusionWe suggest that HLH should be considered in severe cases of scrub typhus especially if associated with cytopenia (s), liver dysfunction, and coagulation abnormalities. Further studies are required to understand whether an immunosuppressive and/or immunomodulator therapy could be beneficial in those patients who remain unresponsive to definitive antibiotic therapy.