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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.

Typhoid fever is caused by enteroinvasive Gram-negative organism Salmonella typhi. The well-known complications of typhoid fever are intestinal haemorrhage and perforation. In the pre-antibiotic era, these complications were quite common, but in the current antibiotic era the incidence of these complications is on the decline. We report a case of a patient with typhoid fever who developed haematochezia during the hospital stay and was found to have caecal ulcer with an adherent clot on colonoscopy. He was managed successfully with conservative measures without endotherapy and there was no rebleed.

Although neonatal renal medullary hyperechogenicity can occur in a variety of pathological conditions, it has been reported to occur transiently with or without renal abnormalities in term as well as in preterm neonates. Variably described in the literature as Tamm-Horsfall nephropathy, or stasis nephropathy, the pathogenesis is unknown, but has an excellent prognosis. The authors describe a 3-day-old neonate with transient renal medullary hyperechogenicity, with no other abnormalities, which resolved within 2 weeks.

Hemophagocytic lymphohistiocytosis (HLH) has a well known association with lymphomas, especially of T cell origin. Prognosis of lymphoma associated HLH is very poor, especially in T cell lymphomas; and, therefore, early diagnosis might alter the outcome. Though association of HLH with systemic anaplastic large cell lymphoma (ALCL) is known, its occurrence in primary cutaneous ALCL (C-ALCL) is distinctly rare. We aim to describe a case of C-ALCL (anaplastic lymphoma kinase (ALK)−) in an elderly male who succumbed to the complication of associated HLH, which was possibly triggered by coexistent virus infection. We briefly present the literatures on lymphoma associated HLH and discuss the histopathological differentials of cutaneous CD30+ lymphoproliferative disorders. We do suggest that HLH may pose diagnostic challenges in the evaluation of an underlying lymphoma and hence warrants proper evaluation for the underlying etiologies and/or triggering factors.