Haemophagocytic lymphohistiocytosis (HLH) is a clinicopathologic syndrome, characterised by hyperinflammation due to inherited or acquired defects in the immune function, leading to unchecked proliferation of histiocytes and lymphocytes resulting in multiorgan dysfunction. HLH can be primary (familial) occurring in young children caused by underlying genetic defects in natural killer cells/cytotoxic T cells or secondary HLH occurring in older children or adults following infections, rheumatological disorders or malignancies. HLH is a medical emergency, having varied clinical presentations and lacks a pathognomonic clinical or laboratory abnormality. Clinical presentations include unexplained fever, hepatomegaly, splenomegaly, skin rash, cytopenias, liver dysfunction, coagulation abnormality and neurological manifestations. It carries a poor prognosis. Early diagnosis based on HLH 2004 criteria and initiation of treatment is crucial in the management strategy, which is likely to improve the outcome of this life-threatening disease. The treatment strategies include immunosuppressive drugs, immunomodulatory therapy and autologous hematopoietic stem cell transplant in selected cases. Here with authors report a case of young adult, presenting with fever, thrombocytopenia, splenomegaly, and multi organ dysfunction, diagnosed as a case of secondary HLH based on the HLH 2004 guidelines.
Background: Chorangioma is a benign vascular placental tumour. It is composed of fetal capillary proliferation within the chorionic villi supported by a variable stroma. Smaller lesions are incidental, are often missed and carry no clinical significance. Larger lesions are associated with feto-maternal complications and are infrequently sent for histopathological examination.Methods: The study was conducted at the department of pathology, at a tertiary care obstetrics and gynaecology hospital. The study was a retrospective study which covered 7 years. Paraffin embedded blocks of placental specimens containing mass were taken up for the study. Sections were stained with haematoxylin and eosin (H and E). The results are compared and correlated with clinicopathologic factors. The statistical data are analysed manually.Results: A total of seven cases were included in the study, 4 cases were primi gravida, 1 case each in second, third and fourth gravida. Pregnancy outcome was intrauterine death in 1 case, dead born in 1 case, alive healthy children in 5 cases, birth weight was normal in 4 children, low birth weight in 2 children and 1 was extremely low birth weight. Of the total of 7 placental specimens 3 showed extraplacental mass and rest 4 showed intraplacental mass. All cases showed solitary lesions and measured > 5cm (large). Histopathological examination of all 7 specimens showed features of chorangioma.Conclusions: Careful inspection of the placenta is necessary following all deliveries. Any suspicious lesions should be documented and evaluated by histopathological examination there by predicting feto maternal complications and help the clinicians in better management of the mother and child accordingly. Meagre documentation of such cases prompted us to present this series of 7 cases of large chorangiomas with a mixed fetal outcome.
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