Search

Masson's tumour, also known as intravascular papillary endothelial hyperplasia, is a rare non-neoplastic lesion of vascular origin, caused by an excessive reactive proliferation of endothelial cells in normal blood vessels or vascular malformations. It can affect any part of the body, presenting most frequently in the vessels of the head, neck and upper extremities. The authors describe the case of a 76-year-old female patient presenting an anterior cervical mass, measuring 2×2 cm, which was mobile, tender and slightly painful with no alteration of the overlying skin. Complementary study with ultrasound and computed tomography was inconclusive. Total excision of the lesion was performed with histology compatible with intravascular papillary endothelial hyperplasia., Learning Points: Masson's tumour is a rare and indolent non-neoplastic lesion of vascular origin, that can be easily confused with other pathological entities and misdiagnosed.Diagnosis can be a challenge; histological characterization is essential for differentiation from other lesions, such as angiosarcoma and Kaposi's sarcoma.Masson's tumour should be considered in patients presenting a single cervical mass., Competing Interests: Conflicts of Interests: The authors declare there are no competing interests., (© EFIM 2021.)

Thrombocytopenia may be the initial presentation of sarcoidosis, which is a systemic granulomatous disorder. Various pathophysiological mechanisms have been identified. Immune thrombocytopenia often has a severe presentation but may respond favourably to immunosuppressive therapy. There are no guidelines for the treatment of thrombocytopenia in sarcoidosis. However, in emergency situations with major bleeding, it seems reasonable to apply the current guidelines recommended for immune thrombocytopenia. The authors report a case of sarcoidosis presenting with severe thrombocytopenia, petechial rash, and nasal and gingival bleeding., Learning Points: The association of thrombocytopenia with sarcoidosis has been well described and fully documented.Immune thrombocytopenia in sarcoidosis is usually severe and symptomatic at presentation but generally has a favourable course because of modern therapeutic management.Steroids may be administered as first-line treatment for sarcoidosis, but in emergency situations with a severe bleeding risk, it seems reasonable to apply the current guidelines for immune thrombocytopenia, namely methylprednisolone (1 g/day for 2 days) and/or intravenous immunoglobulin (1 g/kg/day for 3 days)., Competing Interests: Conflicts of Interests: The authors declare there are no competing interests., (© EFIM 2021.)

Involvement of the central nervous system, although uncommon, is one of the most frequent extramedullary manifestations of chronic lymphocytic leukaemia (CLL). Various conditions can lead to neurological symptoms in CLL patients and distinguishing between clinically significant CLL involvement of the CNS and other aetiologies can be challenging. The authors report the case of a 90-year-old woman with a previous diagnosis of low-risk CLL who presented to the emergency room with altered mental status. After the most frequent causes were ruled out and considering the underlying disease, CNS infiltration by clonal B-cells was hypothesised and later confirmed. Treatment was initiated, but the patient died soon afterwards., Learning Points: Central nervous system involvement in chronic lymphocytic leukaemia (CLL) is rare and characterized by a heterogeneous clinical presentation.CNS localisation of CLL should be considered in patients with any neurological symptom, irrespective of the stage and activity of CLL.Brain imaging and cerebrospinal fluid examination should be performed., Competing Interests: Conflicts of Interests: The authors declare there are no competing interests., (© EFIM 2021.)

Eosinophilic colitis is a rare heterogeneous inflammatory disorder. The pathogenesis is not well understood even though it seems to be multifactorial, with hypersensitivity as a major contributor. The clinical presentation depends on the eosinophilic infiltration of different sections within the gastrointestinal tract. Diagnosis is based on the presence of peripheral eosinophilia and histopathological evidence of colon wall eosinophilic infiltration. The authors present the case of a woman with a predominantly subserous pattern of eosinophilic colitis potentially triggered by intake of an Ulmus rubra-rich product., Learning Points: Subserous eosinophilic colitis (EC) is a rare inflammatory gastrointestinal disease that should be suspected when peripheral eosinophilia is accompanied by eosinophil-rich ascites and confirmed by the presence of eosinophilic infiltration of the colon wall.Although the aetiology of EC is not fully understood, a combination of genetic predisposition, dysbiosis and environmental factors (e.g., ingested allergens) likely contribute to it.The subserous type has the best prognosis and responds well to corticosteroids, with fewer relapses than other types., Competing Interests: Conflict of interests: The authors declare there are no competing interests., (© EFIM 2021.)

Pleuroparenchymal fibroelastosis (PPFE) is a relatively rare interstitial lung disease (ILD) consisting of elastofibrosis involving the subpleural parenchyma and visceral pleura with an upper lobe predominance. It can be idiopathic or associated with some forms of autoimmune disease. The authors describe the case of a 78-year-old woman with a previous diagnosis of Crohn's disease (CD). She presented with a protracted respiratory infection (with no significant history of previous infections), and underwent high-resolution chest computerized tomography that eventually showed alterations compatible with PPFE. After exclusion of other possible underlying causes, a possible link to CD was considered. Follow-up investigation at 12 months showed clinical and radiological stability and also stable lung function under treatment with hydroxychloroquine and prednisolone. This is the first report of PPFE in the context of CD. Future studies will be important to further investigate this pathological association and its prognostic implications., Learning Points: Pleuroparenchymal fibroelastosis (PPFE) is an uncommon interstitial lung disease consisting of elastofibrosis involving the subpleural parenchyma and visceral pleura with an upper lobe predominance.PPFE can be idiopathic or associated with several conditions including autoimmune diseases and may be a direct extra-intestinal manifestation of Crohn's disease (CD).The main disease patterns of PPFE in the context of CD are organizing pneumonia, non-specific interstitial pneumonia, granulomatous interstitial lung disease, pulmonary eosinophilia and sterile necrobiotic nodules., Competing Interests: Conflicts of Interests: The Authors declare that there are no competing interests., (© EFIM 2020.)

Bendamustine is a chemotherapeutic drug associated with frequent haematological and gastrointestinal adverse effects and, more rarely, neurological toxicity. We present the case of a 79-year-old man with follicular lymphoma, grade 2, Ann-Arbor stage IV-A, FLIPI 4, high risk, with bulky disease and vital organ compression, treated with R-CHOP and then rituximab-bendamustine, who developed encephalopathy approximately 2 months after the last cycle. After ruling out other possible aetiologies, we assumed the patient's encephalopathy was due to a delayed bendamustine side effect, possibly aggravated by the concomitant administration of rituximab. The authors hope to alert clinicians to this rare adverse effect, its difficult identification and the need to rule out other aetiologies., Learning Points: Bendamustine neurotoxicity is a rare side effect.Bendamustine neurotoxicity should be considered a diagnosis of exclusion in a patient with progressive encephalopathy treated with the drug.Drug cessation is the mainstay of treatment in cases of bendamustine-induced encephalopathy., Competing Interests: Conflicts of Interests: The Authors declare that there are no competing interests., (© EFIM 2020.)