Background Patients with hematological malignancies (HM) are at high risk of mortality from SARS-CoV-2 disease 2019 (COVID-19). A better understanding of risk factors for adverse outcomes may improve clinical management in these patients. We therefore studied baseline characteristics of HM patients developing COVID-19 and analyzed predictors of mortality. Methods The survey was supported by the Scientific Working Group Infection in Hematology of the European Hematology Association (EHA). Eligible for the analysis were adult patients with HM and laboratory-confirmed COVID-19 observed between March and December 2020. Results The study sample includes 3801 cases, represented by lymphoproliferative (mainly non-Hodgkin lymphoma n = 1084, myeloma n = 684 and chronic lymphoid leukemia n = 474) and myeloproliferative malignancies (mainly acute myeloid leukemia n = 497 and myelodysplastic syndromes n = 279). Severe/critical COVID-19 was observed in 63.8% of patients (n = 2425). Overall, 2778 (73.1%) of the patients were hospitalized, 689 (18.1%) of whom were admitted to intensive care units (ICUs). Overall, 1185 patients (31.2%) died. The primary cause of death was COVID-19 in 688 patients (58.1%), HM in 173 patients (14.6%), and a combination of both COVID-19 and progressing HM in 155 patients (13.1%). Highest mortality was observed in acute myeloid leukemia (199/497, 40%) and myelodysplastic syndromes (118/279, 42.3%). The mortality rate significantly decreased between the first COVID-19 wave (March–May 2020) and the second wave (October–December 2020) (581/1427, 40.7% vs. 439/1773, 24.8%, p value < 0.0001). In the multivariable analysis, age, active malignancy, chronic cardiac disease, liver disease, renal impairment, smoking history, and ICU stay correlated with mortality. Acute myeloid leukemia was a higher mortality risk than lymphoproliferative diseases. Conclusions This survey confirms that COVID-19 patients with HM are at high risk of lethal complications. However, improved COVID-19 prevention has reduced mortality despite an increase in the number of reported cases.

Orbital alveolar soft part sarcoma: case report and literature review Alveolar soft part sarcoma (ASPS) is a malignant soft part tissue tumour of uncertain phenotypic expression that mainly affects young adults and children and represents less than 1% of all sarcomas. 1À4 There are only 63 cases of primary orbital ASPS reported in the PUBMED, none from Canada, with a female preponderance and a predilection for affecting the left eye. 1À3 Herein we describe an unusual case of orbital ASPS primary of the orbit in a 31-year-old female with a previous history of breast cancer.A 31-year-old female presented a mild proptosis of the right eye. After magnetic resonance imaging (MRI), the preliminary diagnosis was a metastasis of her previous breast carcinoma. At age 26 years, she was diagnosed with a grade 3 invasive ductal carcinoma of the right breast, stage T2 N2.Genetic screenings were negative.The first MRI showed a solitary lesion in the right orbit measuring 16 £ 15 £ 14 mm. The location was intramuscular inferior right, and the lesion was well circumscribed and reconfigured the orbit's floor. It caused swelling of the superior right portion of the optic nerve. It was iso-intense T2 and T1 with an enhanced homogeny after gadolinium injection.Two months later, a second MRI showed an increase in size of the lesion, measuring 18 £ 18 £ 17 mm (Fig. 1A). Biopsy demonstrated a tumour composed of uniform polygonal epithelioid cells in a solid and alveolar architecture outlined by delicate sinusoidal vascular channels. The cells had abundant granular eosinophilic cytoplasm and vesicular nuclei with large prominent nucleoli and a very low mitotic rate with no necrosis (Fig. 1C and D). Periodic acidÀSchiff with diastase stains revealed needle-shaped crystals (Fig. 1E). Immunohistochemical stains performed demonstrated tumour strong positivity for Fig. 1-A, CT scan of the orbit showed a well-circumscribed lesion in the right orbit. B, Microscopic examination shows a monotonous and uniform tumour with a sinusoidal architecture (hematoxylin-eosin [H&E], 20 £). C, The tumour is composed of monomorphic cells with vesicular nuclei and a central nucleoli, eosinophilic and granular cytoplasm, with rare cellular pleomorphism and rare mitotic figures (H&E, 400 £). D, Vascular invasion was identified at the periphery of the tumor (H&E, 40 £). E, Needle-shaped crystals were identified in the cytoplasm of some of the tumoural cells by periodic acid-Schiff (PAS) with diastase stain (PAS diastase, 400 £). F, The tumoural cells show a diffuse nuclear positivity for TFE3 by immunohistochemistry (TFE3, 100 £).

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Context: Guillain-Barré syndrome is a polyradiculoneuropathy of heterogeneous manifestations, necessarily presenting progressive appendicular weakness of variable intensity associated with reduction or abolition of deep reflexes. One of its variants is known as Miller Fisher Syndrome (MFS), characterized by ophthalmoparesis, ataxy and areflexia. It usually has, by etiology, immunomediated reactions induced by acute infections, including HIV. Objectives: This report aims to describe a case of MFS with concomitants HIV infection, attended in a tertiary hospital in northern Paraná and compared it with the literature. [1,7] Case report: A 27-year-old white male patient who presented binocular diplopia, dysarthria, dysphagia, generalized hypotonia, myasfasciculations, sensory ataxia and arreflexia, with a score on the Medical Research Council (MRC) muscle strength scale of 54 points. Treatment with empirical intravenous human immunoglobulina ( IVIG ) was performed pending the result of serologies for etiological screening. He was discharged after eleven days of hospitalization with partial symptomatic improvement and results indicative of acute HIV infection. We compared this case with those described in the published literature Discussion: We compared this case with those described in the published literature and given the low incidence found in the literature of patients with the stage of HIV viremia and the opening of the picture of SMF, the concrete pathophysiology itself is still unknown. The mechanism, however, in which the literature proposes in two theories: (1) an autoimmune action against myelin due to abnormal immunoregulation by HIV; (2) direct action of neurotropic strains of HIV-1. [2.3] Our literature review shows that since 1995, cases of associated guillain barré syndrome have already been reported in the course of HIV viremia. Treatment with immunoglobulin at a dose of 400mg / kg / day for 5 days was done and the symptoms improved. [1,4,6,7] Conclusion: It is exposed, then, a case of HIV-induced MFS whose relationship has consistency, temporality, biological plausibility, coherence and analogy compatible with current literature.

Background: Infection with SARS-CoV-2 can cause COVID-19, which mainly affects the respiratory system. However, neurological complications are frequent, including the group of acquired immune-mediated demyelinating neuropathies (NDAI), including Miller-Fisher syndrome (SMF), characterized by the triad of ophthalmoparesis, ataxia and areflexia. Infection with C. jejuni is the main precipitant of NDAI, but viruses are also related. Objectives: This report aims to describe a case of SMF with concomitant SARS-CoV-2 infection, seen at a tertiary hospital in Northern Paraná and to compare it with the literature. Design and setting: case report of a patient at the hospital neurology service of the Evangelical Hospital, Londrina, Paraná, Brazil. Methods: Report a case of SMF right after confirmation of COVID-19 seen at a tertiary hospital in Northern Paraná and compare it with the literature. Results: Male, 70 years old, white, evolved with progressive and symmetrical crural paraparesis 17 days after confirmation of COVID-19. Upon admission, he presented ataxia, paraesthesia, and crural myotactic areflexia with no signs of pyramidal release, and a score on the Medical Research Council (MRC) muscle strength scale of 58 points. Analysis of CSF with albumin-cytological dissociation. Treatment was performed with intravenous human immunoglobulin (IVIG) and discharged after six days of hospitalization with partial symptomatic improvement. We compared this case with those described in the published literature. Not found Brazilian case published to date. Conclusion: Rare case report of SMF by SARS-CoV-2. The relationship has consistency, temporality, biological plausibility, coherence and analogy compatible with the current literature. Studies with a higher level of evidence are needed to determine the strength of such an association.