Case Report: Large Granular Lymphocyte Leukemia (LGLL)—A Case Series of Challenging Presentations

Large granular lymphocyte leukemia (LGLL) represents a rare group of diseases with considerable difficulties in their correct diagnostic workup and therapy. The major challenges lie in their distinction from reactive (including autoimmune) lymphoproliferations. Moreover, monoclonal LGL proliferative diseases are in fact a heterogeneous group of disorders, as recognized by the three subtypes in the current WHO classification. It distinguishes two chronic forms (the focus of this case series), namely T-LGLL and chronic lymphoproliferative disorders of Natural Killer cells (CLPD-NK) as well as aggressive NK-cell leukemia. In the clinical routine, the variable presentations and phenotypes of T-LGLL and CLPD-NK are underappreciated. The relevant differential diagnoses range from benign reactive T-cell expansions to other mature T-cell leukemias to highly aggressive γδ-lymphomas. T-LGLL or CLPD-NK patients suffer from a wide variety of symptoms often including, but not limited to, cytopenias or classical autoimmune phenomena. They receive treatments ranging from mere supportive measures (e.g. antibiotics, growth factors, transfusions) over strategies of immunosuppression up to anti-leukemic therapies. The diagnostic pitfalls range from recognition of the subtle T-cell proliferation, repeated establishment of monoclonality, assignment to a descript immunophenotypic pattern, and interpretations of molecular aberrancies. Here, we report a series of selected cases to represent the spectrum of LGLL. The purpose is to raise awareness among the scientifically or practically interested readers of the wide variety of clinical, immunological, and phenotypic features of the various forms of LGLL, e.g. of T-cell type, including its γδ forms or those of NK-lineage. We highlight the characteristics and courses of four unique cases from two academic centers, including those from a prospective nationwide LGLL registry. Each case of this instructive catalogue serves to transport a key message from the areas of (chronic inflammatory) contexts in which LGLL can arise as well as from the fields of differential diagnostics and of various treatment options. Implications for optimization in these areas are discussed.

Differential Diagnostics of Toxic Epidermal Necrolysis (Lyell’s Syndrome) in ICU: Case Reports

Toxic epidermal necrolysis (TEN) is a critical life-threating condition developing as the total detachment of epidermidis and characterized by severe pathological reactions of all body systems. The current article describes two cases of TEN with similar clinical and laboratory signs. In one case the diagnosis of TEN was subsequently refused.The objective: analysis of methods of clinical and differential diagnostics of conditions accompanied with massive epidermidis detachment in ICU patients.Results. The immunomorphological evaluation of skin specimen obtained from the patient with a torpid form of TEN showed linear IgG fixation in the intercellular space of stratum basale, stratum spinosum and stratum granulosum and C3 fixation in the intercellular space of stratum basale.Conclusion. The complex of anamnesis data and pathomorphological evaluation of skin are crucial for the diagnosis and treatment of patients with atypical TEN.

Endovideosurgical technology in the diagnosis and treatment of benign ovarian tumors

The article deals with the new possibilities of laparoscopic technique in diagnosis and treatment of ovarian tumors. The authors elaborate the criteria of differential diagnostics of benign and malignant ovarian tumors at early stages. The research is based on the analysis of 222 cases of different ovarian tumors. The authors state the necessity of rational preoperative preparation and obligatory screening in patients with ovarian tumors before and after the operation with the use of tumor marker CA-125.

Аsthma

Asthma is one of the most common respiratory tract diseases (about 7% of adults and about 10% of adolescents and children suffer from asthma in the Russian Federation). Astma frequencies of occurrence dont depend from sex or age in population. Numbers of asthmatic patients in the world increase every year. The majority of patients suffering asthma well respond to conventional therapies and successful control disease, but 20-30% of the patients have severe phenotypes asthma resisting for known medicines, therefore they achieve control for asthma very rare. Patients with severe asthma frequently need hospitalizations (up to 30%) and intensive care (4-7%). The clinical asthma guideline has goals to optimize patient care, up-to-date information about the epidemiology, etiology and pathogenesis of the disease. The text contains actual data about asthma classification, its clinical signs, modern diagnostics (clinical, laboratory and instrumental) inclunded the differential diagnostics of astma. There are chapters about asthma treatment, rehabilitation and prevention in the guideline. Authors detailed describe existing healthcare options for asthmatic patients and the features of diasgnostics and care in partial groups of population (adolescents, pregnant or nursing women, persons with occupational or exercise-induced or severe phenotype asthma). The clinical guideline are recommended for medical doctors (independendently from qualification), under- and postgraduate students, universities tutors, residents and researchers.

Inner ear barotrauma and inner ear decompression sickness: a systematic review on differential diagnostics

Introduction: Inner ear barotrauma (IEBt) and inner ear decompression sickness (IEDCS) are the two dysbaric inner ear injuries associated with diving. Both conditions manifest as cochleovestibular symptoms, causing difficulties in differential diagnosis and possibly delaying (or leading to inappropriate) treatment. Methods: This was a systematic review of IEBt and IEDCS cases aiming to define diving and clinical variables that help differentiate these conditions. The search strategy consisted of a preliminary search, followed by a systematic search covering three databases (PubMed, Medline, Scopus). Studies were included when published in English and adequately reporting one or more IEBt or IEDCS patients in diving. Concerns regarding missing and duplicate data were minimised by contacting original authors when necessary. Results: In total, 25 studies with IEBt patients (n = 183) and 18 studies with IEDCS patients (n = 397) were included. Variables most useful in differentiating between IEBt and IEDCS were dive type (free diving versus scuba diving), dive gas (compressed air versus mixed gas), dive profile (mean depth 13 versus 43 metres of seawater), symptom onset (when descending versus when ascending or surfacing), distribution of cochleovestibular symptoms (vestibular versus cochlear) and absence or presence of other DCS symptoms. Symptoms of difficult middle ear equalisation or findings consistent with middle ear barotrauma could not be reliably assessed in this context, being insufficiently reported in the IEDCS literature. Conclusions: There are multiple useful variables to help distinguish IEBt from IEDCS. Symptoms of difficult middle ear equalisation or findings consistent with middle ear barotrauma require further study as means of distinguishing IEBt and IEDCS.

A high-throughput neutralization assay for yellow fever serodiagnostics

Quick and accurate detection of neutralizing antibodies (nAbs) against yellow fever is essential in serodiagnosis during outbreaks, for surveillance and to evaluate vaccine efficacy in population-wide studies. All this requires serological assays that can process a large number of samples in a highly standardized format. Albeit being laborious, time-consuming and limited in throughput, classical plaque reduction neutralization test (PRNT) is still considered gold standard for the detection and quantification of nAbs due to its sensitivity and specificity. Here we report the development of an alternative fluorescence-based serological assay (SNTFLUO) with an equally high sensitivity and specificity that is fit for high-throughput testing with the potential for automation. Finally, our novel SNTFLUO was cross-validated in several reference laboratories and against international WHO standards showing its potential to be implemented in clinical use. SNTFLUO assays with similar performance are available for the Japanese encephalitis, Zika and dengue viruses amenable for differential diagnostics.

Revisiting the Awareness and Understanding the Associations between Intracranial Tumors and Optic Neuropathy

The aim of this paper is to report clinically various cases of intracranial tumors in patients referred to glaucoma clinic for consultation. The secondary aim was to increase the awareness of intracranial tumors in atypical cases of glaucoma. We present the retrospective analysis of five patients referred to glaucoma clinic for consultation. Due to atypical course of the disease, in addition to standard glaucoma examinations, all patients had a neurologic full visual field, color vision, and MRI done. In all patients, intracranial malignancies were found, some patients underwent surgery of the lesions with consecutive clinical improvements. Interestingly, in some patients, coexisting glaucoma was diagnosed. Patients were selected deliberately to present a wide spectrum of possible clinical scenarios when glaucoma may be complicated by intracranial tumors. Sometimes, the relevance of intracranial tumors with respect to their influence on the clinical picture of the optic nerve cannot be established. To conclude, in the “atypical cases of glaucoma” the assessment of the optic nerve may indicate the necessity of neuroimaging in differential diagnostics.

DISC HERNIA - DIFFERENTIAL DIAGNOSTICS, OPTION OF PSEUDOTUMOROUS FLOW

Грыжа диска (ГД) является распространенным заболеванием и наносит большой вред как физическому, так и психическому здоровью пациентов, страдающих этим заболеванием. Главным этиологическим фактором заболевания служит дегенерация диска. В настоящее время общее определение грыжи межпозвонкового диска в клинической медицине довольно запутанно. В настоящее время общее определение грыжи межпозвонкового диска в клинической медицине довольно запутанно. Окончательный диагноз ГД основывается на совокупности анамнеза, клинических симптомов и результатах визуализации. Herniated disc is a common disease and causes great harm to both the physical and mental health of patients suffering from this disease. The main etiological factor of the disease is disc degeneration. Currently, the general definition of a herniated disc in clinical medicine is quite confusing. Currently, the general definition of a herniated disc in clinical medicine is quite confusing. The final diagnosis of HD is based on a combination of anamnesis, clinical symptoms, and imaging results.

RUBINSTEIN-TEIBI SYNDROME

Rubinstein-Teibi syndrome (RTS) is a multisystem pathology characterized by mental retardation and delayed physical development combined with characteristic external manifestations that make up a recognizable picture of the disease. The literature review highlights the clinical manifestations of RTS, considers the issues of geno-phenotypic correlations and differential diagnostics, describes the possibilities and prospects of diagnostics and treatment, taking into account the results of modern research.