Diagnostic Splenectomy: Characteristics, Pre-Operative Investigations, and Identified Pathologies for 20 Patients.

Splenectomy is indicated in cases of trauma to the spleen or hematological and immunological diseases (hereditary spherocytosis, autoimmune cytopenia). Less frequently, splenectomy is performed for diagnostic purposes to complement unsuccessful prior etiological investigations. The splenectomy remains a surgery at risk of complications and should be considered as a last-resort procedure to make the diagnosis and to be able to treat patients. We studied the medical files of 142 patients who underwent a splenectomy for any reason over a 10-year period and identified 20 diagnostic splenectomies. Diagnostic splenectomies were mainly performed to explore unexplained splenomegaly for 13 patients and fever of unknown origin for 10. The other patients had surgery for other causes (cytopenia, abdominal symptoms, suspicion of relapsing malignant hemopathies). Splenectomy contributed to the final diagnosis in 19 of 20 cases, corresponding mostly to lymphoid hemopathies (14/20). The most frequent disease was diffuse large B-cell lymphoma (8/20). Splenectomy did not reveal any infectious disease. The most relevant pre-operative procedures to aid the diagnosis were 18F-fluorodeoxyglucose positron emission tomography/computed tomography (FDG PET/CT) and immuno-hematological examinations. Diagnostic splenectomy is useful and necessary in certain difficult diagnostic situations. Highlights: Diagnostic splenectomy is still useful in 2020 to diagnose unexplained splenomegaly or fever of unknown origin. Lymphoma was the most common final diagnosis. FDG PET/CT was the most useful tool to aid in the diagnosis.

Isolated 5' Signals Are an Atypical Pattern To Be Considered as Positive for ALK Rearrangement: A Brief Report of Three Cases and Review of the Literature.

Anaplastic lymphoma kinase (ALK) rearrangement is reported in 3% to 8% of patients with lung adenocarcinoma and can be detected by fluorescent in situ hybridization (FISH) or indirectly by immunohistochemistry. In FISH assay, isolated 5' signal (loss of 3' signal) is usually considered negative. We report three young nonsmoking patients with stage IV lung adenocarcinoma. Strong ALK expression in tumor cells detected by immunohistochemistry was observed in all cases, but FISH revealed an isolated 5' signal pattern. Massive parallel "next-generation" sequencing was performed in two patients and confirmed ALK rearrangement. The three patients were treated and responded to crizotinib after 14, 10, and 31 months.

[Cutaneous pseudolymphoma as an alternative presentation of IgG4 related disease]. / Le pseudolymphome cutané, une autre présentation du syndrome d'hyper-IgG4.

Hyper-IgG4 syndrome is a rare entity characterized by fibro-inflammatory lesions of organs, an excess of IgG4 positive plasma cells in histology and high serum level IgG4. Many organs can be affected (pancreas, kidney, salivary glands) and the list continues to grow. The skin damage is rarely reported in the literature and is usually associated with other typical lesions of this syndrome. We report the case of a 53-year-old female followed since 2005 for lymphadenopathy, associated with axillary nodular skin lesions. The assessments made at that time had retained the diagnosis of pseudolymphoma with implementation of multi-line therapy. Six years later, and the persistence of the lesions, plasma cells marked by anti-IgG4 and the serum IgG4 has attached injuries to the syndrome hyper-IgG4. The patient is treated with low dose corticosteroids with a good and protacted response. Cutaneous pseudolymphoma could be a new presentation of the syndrome of hyper-IgG4 in the absence of any other injury usually associated with this entity. This case illustrates the interest for proposing a plasma cell labeling with anti-IgG4 in any case of cutaneous pseudolymphoma.