Peripheral T-Cell Lymphoma of the Submandibular Salivary Gland as an Unusual Manifestation of Richter’s Syndrome: A Case Report and Literature Review

Richter’s syndrome is the development of high-grade non-Hodgkin lymphoma (NHL) or Hodgkin lymphoma in patients with chronic lymphocytic leukemia (CLL)/small lymphocytic lymphoma (SLL). In most patients with Richter’s syndrome, the high-grade NHL is diffuse large B-cell lymphoma. Only a small minority of CLL/SLL patients develop T-cell malignancies. Herein, we describe a 40-year-old male patient presenting with peripheral T-cell lymphoma not otherwise specified (PTCL-NOS) in the submandibular salivary gland, two years after the diagnosis of CLL/SLL. The PTCL-NOS consisted of small lymphocytes, which complicated diagnosis. Immunohistochemical, cytological, and molecular studies allowed the correct diagnosis of composite lymphoma (SLL/PTCL-NOS) of the submandibular salivary gland. The PTCL-NOS had a cytotoxic phenotype and aberrant expression of CD79a. There was no evidence to suggest that the PTCL-NOS of the submandibular salivary gland developed from an intimately associated submandibular lymph node or by PTCL-NOS dissemination. A review of the literature and presented case suppose that the PTCLs developed following CLL/SLL have the cytotoxic phenotype and can clinically mimic typical Richter’s syndrome

Phylogenetics of the paleartic model grass "brachypodium sylvaticum" uncovers two divergent oriental and occidental micro-taxa lineages

Brachypodium sylvaticum has been selected as a model for perennial grasses, and considerable genomic resources have been generated and a reference genome and several resequenced pangenome accessions are available for this species. Despite these genomic advances, the evolution and systematics of diploid B. sylvaticum s. l. is almost unknown. The B. sylvaticum complex is formed by up to seven taxonomically close micro-taxa which differentiate from typical B. sylvaticum s. s. based on a few morphological features. Moreover, some of them show some largely disjunct geographic distributions on both sides of their native Palearctic region. In this study, we used a phylogenomic approach including representative populations from the oriental and occidental distribution range of B. sylvaticum micro-taxa to elucidate their evolutionary relationships and assess the systematic value of the morphological features that separate them. A combined plastome and nuclear phylogenetic tree supports an early split and high divergence of the oriental lineage, showing the close relationship of the Himalayan B. sylvaticum var. breviglume lineages to the Pacific B. miserum / B. kurilense clade, and the contrasting large homogeneity and low divergence of the occidental European, N African and SW and C Asian lineage, with several B. sylvaticum s. s., B. spryginii, and B. glaucovirens samples showing identical or similar sequences. Divergence time estimate analysis suggests that the oriental lineage diverged from the common ancestor in the early Pleistocene (2.0 Ma), followed by subsequent colonization and isolations in the Himalayas (2.0 – 1.7 Ma) and the Far East (0.36 Ma) in more recent times, while the occidental lineage split in the Mid-Late Pleistocene (0.97 Ma), followed by rapid radiation and postglacial spread in the western Paleartic during the last thousand years

Трансформированный вариант диффузной В-клеточной крупноклеточной лимфомы желудка у пациентки с сочетанием болезни Шегрена и системной склеродермии (описание случая и обзор литературы)

This article describes a case of a transformed diffuse large B-cell lymphoma of the stomach in a patient with Sjogren’s disease (SjD) and systemic sclerosis (SSc), as well as a brief review of the literature on lymphoproliferative diseases in SjD and SSc.Представлены описание случая трансформированного варианта диффузной В-клеточной крупноклеточной лимфомы желудка у пациентки с болезнью Шегрена (БШ) и лимитированной системной склеродермией (ССД), а также краткий обзор литературы, посвященной лимфопролиферативным заболеваниям при БШ и ССД. Обсуждаются связи между указанными состояниями

IAPT/IOPB chromosome data 16

[EN] Inula helenioides DC., I. langeana Beck, I. maletii Maire, I. montana L., I. oculus-christi L., I. salicina L. (Asteraceae)

IgG4-RELATED DISEASE. CLINICAL NOTES

IgG4-related diseases are a new nosological entity that encompasses a few previously known diseases. IgG4-related systemic disease is diagnosed if two or more affected organs are detected. This group of diseases has two similar signs: serological (elevated serum IgG4 subclass concentrations) and histological (organ and tissue infiltration from plasmo-cytes secreting IgG4, and eosinophils, and the development of fibrosclerosis and phlebitis obliterans). The paper describes two cases. In one case, a multisystemic disease was observed virtually at its onset whereas in the other this lesion was diagnosed several years after the natural course of the disease

First-Line Therapy for Patients with Advanced Hodgkin’s Lymphoma: Efficacy and Toxicity of Intensive ЕАСОРР-14 Program (NN Blokhin National Medical Cancer Research Center Data)

Aim. To assess the efficacy and toxicity of intensive 6 courses EACOPP-14 treatment with or without radiotherapy (RT) for advanced stages of Hodgkin’s lymphoma (HL). Materials & Methods. From November 2009 to February 2015, 95 patients with advanced stages of HL (IIX–IIE, III–IV) aged between 17 and 50 years (median 29 years) were selected for the participation in the protocol ЛХМосква1-3. The study population consisted of 46.3 % men and 53.7 % women. The results of the treatment were assessed in 91 patients who have received more than 2 courses of EACOPP-14. The follow up period was at least 3 months after the receiving the therapy. Consolidation RT with a total dose of 30 Gy for residual tumor lesions and/or initially large tumors was performed after the chemotherapy. Results. Complete remission was achieved in 82 (90.1 %) patients, partial remission in 2 (2.2 %), and the progression was observed in 7 (7.7 %) patients. The overall 4-year survival rate was 90.8 %, the progression-free survival was 88.2 %. The toxicity of the ЕАСОРР-14 program was slightly lower than that of 8 courses of ВЕАСОРРesc, and was comparable to the toxicity of other modifications of intensified ВЕАСОРР scheme. Hematological toxicity grade 3 and 4 was most commonly observed: leukopenia was observed after 64.9 % of courses, anemia — after 24 % of courses, thrombocytopenia — after 3.8 % of courses. The rate of infections did not singificantly differ and accounted for 24 %. The most frequent non-infectious complications were mucositis (21.1 %) and polyneuropathy (11.7 %). Complications resulted in the change of treatment in only 3 (3.01 %) of patients. The exclusion of bleomycine from the ЕАСОРР-14 program reduced the frequency of RT complications. Grade 3 pulmonitis developed in 4.5 % of cases, while radiation-induce pulmonary fibrosis verified by CT developed in 15.2 % of cases. The ЕАСОРР-14 6 courses program showed its high efficacy both with and without RT, high tolerance and the possibility of full administration for the majority of patients with the various stages of HL. Conclusion. Current research showed the efficacy of treatment without RT for patients with advanced stages of HL with negative PET results and small (< 2.5 cm) residual tumors after intensive ЕАСОРР-14 program. This approach allowed to avoid a number of late treatment complications

Inferring Species Networks from Gene Trees in High-Polyploid North American and Hawaiian Violets (Viola, Violaceae)

The phylogenies of allopolyploids take the shape of networks and cannot be adequately represented as bifurcating trees. Especially for high polyploids (i.e., organisms with more than six sets of nuclear chromosomes), the signatures of gene homoeolog loss, deep coalescence, and polyploidy may become confounded, with the result that gene trees may be congruent with more than one species network. Herein, we obtained the most parsimonious species network by objective comparison of competing scenarios involving polyploidization and homoeolog loss in a high-polyploid lineage of violets (Viola, Violaceae) mostly or entirely restricted to North America, Central America, or Hawaii. We amplified homoeologs of the low-copy nuclear gene, glucose-6-phosphate isomerase (GPI), by single-molecule polymerase chain reaction (PCR) and the chloroplast trnL-F region by conventional PCR for 51 species and subspecies. Topological incongruence among GPI homoeolog subclades, owing to deep coalescence and two instances of putative loss (or lack of detection) of homoeologs, were reconciled by applying the maximum tree topology for each subclade. The most parsimonious species network and the fossil-based calibration of the homoeolog tree favored monophyly of the high polyploids, which has resulted from allodecaploidization 9–14 Ma, involving sympatric ancestors from the extant Viola sections Chamaemelanium (diploid), Plagiostigma (paleotetraploid), and Viola (paleotetraploid). Although two of the high-polyploid lineages (Boreali-Americanae, Pedatae) remained decaploid, recurrent polyploidization with tetraploids of section Plagiostigma within the last 5 Ma has resulted in two 14-ploid lineages (Mexicanae, Nosphinium) and one 18-ploid lineage (Langsdorffianae). This implies a more complex phylogenetic and biogeographic origin of the Hawaiian violets (Nosphinium) than that previously inferred from rDNA data and illustrates the necessity of considering polyploidy in phylogenetic and biogeographic reconstruction

Diagnosis of IgG4 - related ophthalmic disease in a group of patients with various lesions of the eye and orbits

Purpose of the study. To provide demographic, clinical, laboratory, ultrasound, radiological, morphological/ immunomorphological phenotype of IgG4-related ophthalmic diseases, which allowsmaking a differential diagnosis with granulomatous, autoimmune, inflammatory, endocrine and hematologic diseases affecting the eye and orbits. Materials and methods. From 2004 to 2016 108 (78.2%) of the 138 patients were diagnosed with non-tumoral lesions of eye and orbits. In 48 patients (35%) at admission and 5 patients in the follow were diagnosed IgG4-related ophthalmic disease. In the analysis of 82 (f-44, m-38) patients with IgG4-related disease, localization of lesions in orbit observed in 53 (f-36, m-17) and it was the most frequent involvement in patients with IgG4-related disease (64.5%). Only 7 patients had isolated IgG4-related ophthalmic disease, whereas 46 patients (87%) had involvement of 2-7 locations, as a manifestation of IgG4-related systemic disease.During the examination, the average age of patients with IgG4-related ophthalmic disease was 47.5 years (19-73 years). Median time to diagnosis was 52.8 months before 2004 and 36 months 2004-2016. Results. We noted the predominance of females in the ratio 2: 1 inthe group of patients with IgG4-related ophthalmic disease. Edema of the eyelids, nasal congestion (55-60%), tumor-like formations of the upper eyelids and increased lacrimation prevailed at the onset of the disease, whereas such functional impairment like limited mobility and pain in eyeballs, exophthalmos, ptosis and diplopia appeared later at 15-38% with a loss visual acuity in one case. Bilateral lesion (86%), mainly affecting the lacrimal glands (93.5%), infiltration of the extraocular muscles (83.5%) and retrobulbar tissue with a thickening of the optic nerve in one third of patients were the main localizations IgG4-related ophthalmic disease. Clinical symptoms were accompanied by the appearance of moderate inflammatory activity (38%), increased levels IgG (44%), IgG4(88%) and IgE (61%). Indicators of autoimmune disorders observed in 6-22% of patients, most often in patients with simultaneous involvement of the salivary glands. Significant lymphoplasmacytic infiltration (94%) with a ratio of plasma cells (IgG4/IgG) secreting IgG4> 40% (90%) with fibrosis formation (94%) and follicle formation (71%) with a moderate amount of eosinophils (34%) were the major morphological / immunomorphological manifestations of IgG4-related ophthalmic disease. Signs of vasculitis and obliterative phlebitis were found in a small amount of patients. Conclusion. Determination of elevated levels of IgG-4 / IgE in patients with edema, pseudotumor of the eyelid, sinusitis and increase of the palpebral lobe of the lacrimal gland suggests the presence of IgG4-related ophthalmic disease. Minimally invasive incisional biopsy of lacrimal glands and salivary glands followed by morphological / immunomorphological research is needed for the correct diagnosis. Diagnostic orbitotomy in ophthalmic hospitals in such cases is inexpedient, since it leads to the development of dry eye. Massive lymphoplasmacytic infiltration with IgG4 / IgG ratio more than 40%, advanced fibrosis in biopsiesof the orbits tissue or salivary glands when combined lesions are required for the making the diagnosis of IgG4-related ophthalmic disease

Дифференциальная диагностика плазматических дискразий в ревматологической практике

Plasma cell dyscrasias (PCD) present certain difficulties in differentially diagnosing with autoimmune rheumatic diseases (RD) as they have some clinical and laboratory manifestations that are characteristic of the latter. Objective: to generalize the experience in diagnosing PCD at the Research Institute of Rheumatology (RIR), Russian Academy of Medical Sciences (RAMS). Subjects and methods. Nineteen patients admitted to the RIR, RAMS, for rheumatological referral diagnoses were diagnosed as having different types of PCD, both PCD in the presence of RD (n = 10) and primary PCD without RD (n = 9). Immunochemical studies of serum and urinary proteins and bone marrow trepanobiopsy were performed in all the patients. Histological studies were made of the minor salivary glands (n = 12), lymph nodes (n = 3), parotid salivary glands - PSG (n = 5), spleen (n = 1), cranial bones (n = 2) and humerus (n = 1). The immunophenotype of tumor lymphocytes in the biopsy and trepanobiopsy specimens were determined by an immunoflurenscence method with standard monoclonal antibody panels and the paraffin sections of biopsy specimens were examined by an immunoperoxidase technique. Biopsy and trepanobiopsy speciments and myelograms were assessed by the researchers of the N.N. Blokhin Russian Cancer Research Center, RAMS. Results. Over 10 years of follow-up, 19 patients (13 females and 6 males) were diagnosed as having different types of PCD: multiple myeloma (MM) (n = 9), extramedullary plasmacytoma of lymph nodes (n = 2), solitary plasmacytoma (SP) of bone (n = 2), Bence-Jones myeloma (BJM) (n = 2), primary amyloidosis (PA) (n = '), lymphoplasmacytic lymphoma (n = '), Waldenström's macroglobulinemia (WM) (n = 2). In the presence of RA, '0 patients with PCD developed Sjogren's disease (SD) (n = 7), rheumatoid arthritis (RA) + SD (n = 2), RA (n = '); and 9 patients had primary PCD (MM (n = 5), BJM (n = '), WM (n = '), SP (n = '), and PA (n = ')). These 9 patients with different rheumatological diagnoses were long followed up and referred to the RIR, RAMS, to specify these. One third of the patients had recurrent conjunctivitis, enlarged PSG and xerostomia (55%). Arthralgias, arthritis of minor and large joints, flexion contractures of hands, ossalgias, and pains in the lumbar spine and ribs were present in '0-45% of the patients. Vascular lesions, such as Reynaud's phenomenon, recurrent purpuras, lower extremity ulcerative lesions, and toe gangrenes, were observed in '0-35% of the patients with PCD. Lesions in the reticuloendothelial system, such as lymphadenopathy, splenomegaly ('5%), hepatomegaly (45%), and fever (25%), were also detected. Conclusion. Many clinical and laboratory manifestations of primary PCD and RD are similar and only the absence of classical laboratory markers of autoimmune disease, as well as the presence of serum monoclonal immunoglobulins and urinary Bence Jones protein suggest the presence of PCD, both primary PCDs and those with RD.Плазматические дискразии (ПД) представляют определенные трудности для дифференциальной диагностики с аутоиммунными ревматическими заболеваниями (РЗ), так как имеют некоторые клинические и лабораторные проявления, присущие последним. Цель исследования - обобщить опыт диагностики ПД в НИИ ревматологии РАМН. Материал и методы. У 19 пациентов, поступивших в НИИ ревматологии РАМН с направительными диагнозами РЗ, диагностированы различные варианты ПД, как на фоне имеющегося РЗ (у 10), так и первичные ПД без признаков РЗ (у 9). Всем больным проводили иммунохимическое исследование белков сыворотки крови и мочи, трепанобиопсию костного мозга. Выполняли гистологическое исследование малых слюнных желез (у 12), лимфатических узлов (у 3), околоушных слюнных желез - ОСЖ (у 5), селезенки (у 1), костей черепа (у 2) и плечевой кости (у 1). Иммунофенотип опухолевых лимфоцитов в биоптатах и трепанобиоптатах определяли им-мунофлюоресцентным методом со стандартными панелями моноклональных антител, а парафиновые срезы биоптатов изучали им-мунопероксидазным методом. Биоптаты, трепанобиоптаты и миелограммы оценивали сотрудники РОНЦ им. Н.Н. Блохина РАМН. Результаты исследования. За '0лет наблюдения у 19 больных (13 женщин и 6 мужчин) диагностированы различные варианты ПД: множественная миелома (ММ) - у 9, внекостная плазмоцитома лимфатических узлов (ВП) - у 2, солитарная плазмоцитома костей (СП) - у 2, миелома Бенс-Джонса (МЫ) - у 2, первичный амилоидоз (ПА) - у 1, лимфоплазмоцитарная лимфома (ЛПЛ) - у 1, макроглобулинемия Вальденстрема (МВ) - у 2. У 10 больных ПД развились на фоне РЗ: болезни Шёгрена - у 7, ревматоидного артрита (РА) + синдрома Шёгрена - у 2, РА у - 1, и у 9 больных ПД были первичными (ММ - у 5, МЫ - у 1, МВ - у 1, СП - у ', ПА - у '). Эти 9 больных длительно наблюдались с различными ревматологическими диагнозами и были направлены в НИИ ревматологии РАМН для уточнения таковых. У трети больных присутствовали рецидивирующие конъюнктивиты, увеличение ОСЖ и ксеростомия (у 55%). Артралгии, артрит мелких и крупных суставов, сгибательные контрактуры кистей, оссалгии, боли в поясничном отделе позвоночника и в ребрах имелись у '0-45% больных. Сосудистые поражения: феномен Рейно, рецидивирующие пурпуры, язвенные поражения нижних конечностей и гангрена пальцев стоп наблюдались у '0-35% больных ПД. Выявлены также поражение ретикулоэндотелиальной системы: лимфаденопатия, спленомегалия ('5%), гепатомегалия (45%) и лихорадка (25%). Заключение. Многие клинико-лабораторные проявления первичных ПД и РЗ являются сходными и только отсутствие классических лабораторных маркеров аутоиммунного заболевания, а также наличие моноклональных Ig в сыворотке крови и белка BJ в моче позволяет предположить наличие ПД, как первичных, так и на фоне РЗ