Chronic neutrophilic leukemia (CNL) is a rare but potentially aggressive BCR::ABL1 negative myeloproliferative neoplasm, characterized by sustained mature, neutrophilic leukocytosis. The discovery of key driver mutations in the colony-stimulating-factor-3 receptor (CSF3R) gene resulted in the updated World Health Organization (WHO) diagnostic criteria in 2016. A significant number of CNL cases have been associated with plasma cell dyscrasias, predominantly multiple myeloma (MM) and monoclonal gammopathy of unknown significance (MGUS). Compared to pure CNL, mutated CSF3R is infrequently reported in CNL cases associated with monoclonal gammopathies (MG). Until now it remains unclear whether CNL and occurring plasma cell neoplasms are clonally related or CNL is developing secondary to the underlying dyscrasia. Owing to its rarity, currently no standard of care management exists for CNL and MG-associated CNL. In this case series we report the multi-center experience of five MG-associated CNL cases with a median age of diagnosis of 69 years. Three patients (66%) showed predominance of lambda light chain expression. Four (80%) eventually evolved to MM, and one CNL-MGUS patient developed secondary acute myeloid leukemia (AML). Mutated CSF3R was present in the patient who developed AML but was absent in other cases. To assess possible associated genetic aberrations we performed recurrent analysis with next-generation sequencing (NGS). Two patients (40%) deceased with a median time of survival of 8 years after CNL diagnosis. Three (60%) are currently in follow-up with no reoccurring leukocytosis. This case series, followed by a short review, provides a long-term clinical and genetic overview of five CNL cases associated with MG.

Background and purpose Enterovirus infections pose a serious threat for patients with humoral deficiencies and may be lethal, whilst the efficacy of proposed treatment options such as corticosteroids, intravenous immunoglobulins and fluoxetine remains debated. Methods Viral clearance was investigated in a patient with rituximab‐induced B‐cell depletion and chronic echovirus 13 (E13) meningoencephalitis/myofasciitis in response to intravenous immunoglobulins and fluoxetine using sequential semi‐quantitative E13 viral load measurements by real‐time reverse transcription polymerase chain reaction. Fluoxetine concentrations in plasma and cerebrospinal fluid were determined by liquid chromatography mass spectrometry. Results Intravenous immunoglobulins appeared ineffective in this case of E13 infection, whereas virus clearance in cerebrospinal fluid was obtained after 167 days of oral fluoxetine. Since treatment with corticosteroids resulted in a flare of symptoms, rechallenge with viral load measurements was not attempted. Conclusion In this report of a patient with rituximab‐associated chronic echovirus 13 meningoencephalitis, viral clearance in response to single treatment options is assessed for the first time. Our observations further support the in vivo efficacy of fluoxetine against enteroviral infections. More research is needed to establish its efficacy in different enterovirus strains.

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We report the case of a 59-year old man with portomesenteric venous gas (PMVG) due to inferior mesenteric vein fistulization caused by sigmoid diverticulitis with an unusual evolution. The patient initially presented with classic symptoms of lower abdominal pain and fever. Diagnosis of uncomplicated sigmoid diverticulitis was confirmed on computed tomography (CT) for which intravenous antibiotics were initiated. Hemocultures were positive for omnisensitive Escherichia Coli, but despite adequate intravenous antibiotic therapy, episodes of bacteraemia persisted and hemocultures remained positive. Repeat CT scan demonstrated regression of inflammation without signs of abcedation or perforation consistent with clinical findings. Endocarditis was excluded with a normal transoesophageal echocardiography. Finally, positron emission tomography-computed tomography (PET-CT) suspected a colovenous fistula and the presence of PMVG. The patient was successfully treated with laparoscopic sigmoidectomy. This case report summarises the diagnostic pathway and aims for higher awareness of non-ischemic PMVG causes.

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