Combination approach to diagnosis and treatment of an elderly patient with chronic Ph-negative myeloproliferative neoplasm and concomitant surgical pathology. Clinical observation

Chronic myeloproliferative neoplasms (CMPN), Ph-negative, are of clonal nature, develop on the level of hematopoietic stem cell and are characterized by proliferation of one or more hematopoietic pathways. Currently, the group of Ph-negative CMPN includes essential thrombocythemia, primary myelofibrosis, polycythemia vera, myeloproliferative neoplasm unclassifiable.Identification of mutations in the Jak2 (V617F), CALR, and MPL genes extended understanding of biological features of Ph-negative CMPN and improved differential diagnosis of myeloid neoplasms. Nonetheless, clinical practice still encounters difficulties in clear separation between such disorders as primary myelofibrosis, early-stage and transformation of essential thrombocythemia into myelofibrosis with high thrombocytosis. Thrombocytosis is one of the main risk factors for thromboembolic complications, especially in elderly people.A clinical case of an elderly patient with fracture of the left femur developed in the context of Ph-negative CMPN (myelofibrosis) with high level of thrombocytosis is presented which in combination with enforced long-term immobilization and presence of additional risk created danger of thrombosis and hemorrhage during surgery and in the postoperative period

Морфологические и иммунологические особенности стимулированного Г-КСФ аутологичного и аллогенного костного мозга, применяемого для трансплантации в клинической онкологии

58 samples of stimulated bone marrow (39 – oncology patients, 19 healthy donors) obtained for transplantation in cancer patients were analysed and compared to control group (13 bone marrow samples from women with mastopathies). It was noted the significant increase of blast cells in cancer patients compared to donors and control group (p<0.037). Neutrophyl maturation index was higher in stimulated bone marrow (p<0.001) and decrease of lymphocyte percentage (p<0.008) compared to control group: elevation of CD3+ and CD8+ lymphocytes (p<0.0001) as well as decrease of NK-cells CD56+ CD3-  and/ or CD16+ CD3-).При анализе 58 образцов стимулированного костного мозга (39 онкологических больных и 19 здоровых доноров), полученного для трансплантации онкологическим больным, и сопоставлении основных показателей с исторической нормой и 13 образцами костного мозга больных контрольной группы (мастопатия) выявлен ряд особенностей стимулированного костного мозга. Показано достоверное увеличение количества бластных клеток костного мозга у онкологических больных в сравнении с донорами и группой контроля (р<0,037). В образцах стимулированного костного мозга (онкологические больные и доноры) выявлен более высокий индекс созревания нейтрофилов (р<0,001) и снижение процента зрелых лимфоцитов (p<0,008) в сравнении с контрольной группой. Среди лимфоцитарных популяций под влиянием ГКСФ как у онкологических больных, так и у доноров отмечено нарастание суммарного числа зрелых CD3+ Т-клеток и CD8+ Т-киллеров (p<0,0001), а также снижение количества субпопуляций NK-клеток CD56+ CD3–  и/или CD16+ CD3– (р<0,006) в сравнении с группой контроля

Difficulties of lymphoid neoplasia diagnosis in patients with rheumatoid arthritis

In the article is presented a detailed description of two rare cases of lymphoid neoplasia (αβT-cell large granular lymphocyte leukaemia and primary γδT-cell lymphoma of the spleen) in patients with rheumatoid arthritis. These examples illustrate several diagnostic problems we often face while searching for lymphoid tumors in patients with rheumatoid arthritis. A literature review is attached

Primary hematologic malignancies with the onset of involvement of the major salivary glands in rheumatologic practice

Objective: to identify the main types of lymphoma with the onset of involvement of the major salivary glands (MSGs), which are encountered in rheumatologic practice, and to evaluate the efficiency of minimally invasive incisional biopsies of the MSGs/lacrimal glands in the diagnosis of different diseases affecting the parotid, submandibular, sublingual, and lacrimal glands.Patients and methods. A total of339patients (291 women and 48 men) aged 18 to 78 years (median age, 55 years) with MSG involvement were examined at the V.A. Nasonova Research Institute of Rheumatology in 2004 to 2017. Minimally invasive incisional biopsies of the parotid, submandibular salivary (SMSG), and lacrimal glands were carried out to verify the diagnosis.Results and discussion. Different variants of hematological malignancies with MSG involvement were diagnosed in 187 (55%) patients; nonneoplastic diseases were in 152 (45%) patients. Primary tumors with the onset of MSG involvement were detected in 52 (15.3%) patients. Hematologic malignancies were diagnosed in 32 (61.5%) cases and 20 (38.5%) patients had various benign neoplastic or infectious diseases with MSG involvement. The prevailing hematologic malignances with MSG involvement were non-Hodgkin lymphomas (NHL), indolent MALT-lymphomas in 12 (23%) patients, follicular lymphomas in 6 (11.5%), and AL-amyloidosis affecting the SMSG in 9 (17.3%). The diagnosis was morphologically verified using minimally invasive incisional biopsy of the salivary/lacrimal glands; in all cases, the biopsy was informative for full morphopathological examination and diagnosis verification.Conclusion. Minimally invasive incisional biopsy of the MSG/lacrimal glands should be actively used in dentistry, rheumatology and blood oncology hospitals for verification of nosological diagnoses when the MSG and lacrimal glands are involved. A sufficient number of tissues obtained using these techniques allows one to abandon severe surgical interventions (partial and complete parotidectomy, SMSG removal, and orbitotomy) currently used for diagnostic purposes in various types of hospitals