Primary Data on ATTR-Amyloidosis Prevalence Among Elderly Patients With Left Ventricular Hypertrophy in Russia.

AIM: To estimate the prevalence of amyloid cardiomyopathy (CM) caused by transthyretin amyloidosis (ATTR) and immunoglobulin light chain (AL) amyloidosis among patients aged >65 years with interventricular septal (IVS) hypertrophy of ≥14 mm. MATERIAL AND METHODS: From January through August 2023, 60 patients (mean age 7.2±7.3 years, 34 (56.67%) men) were enrolled. Patients meeting the inclusion criteria underwent an echocardiographic study with determining the myocardial longitudinal strain, myocardial scintigraphy with 99mTc-pyrfotech, myocardial single-photon emission computed tomography, measurement of N-terminal fragment of brain natriuretic peptide and troponin I, and the immunochemical study of serum and urine proteins with measurement of free light chains. In the presence of grades 2 and 3 radiopharmaceutical uptake according to scintigraphy, a molecular genetic study was performed for differential diagnosis of wild-type transthyretin amyloidosis (wtATTR) and hereditary/variant (hATTR) ATTR-CM. RESULTS: According to data of myocardial scintigraphy with 99mTc-pyrfotech, grade 3 uptake in the absence of monoclonal secretion was detected in 5 (8.3%) cases and grade 2 radiotracer uptake in the absence of monoclonal secretion was detected in 6 (10%) patients. Myeloma complicated by AL amyloidosis and primary AL amyloidosis were found in 5 (8.3%) patients. CONCLUSION: Among patients aged ≥65 years with IVS hypertrophy ≥14 mm, amyloid CM was detected in 20% of cases (12 patients), including 5 cases (8.3%) of AL amyloidosis and 7 cases (11.7%) of ATTR amyloidosis.

[Myeloproliferative masks of multiple myeloma: A review of literature and clinical case reports]. / Mieloproliferativnye «maski¼ mnozhestvennoi mielomy (obzor literatury i opisanie klinicheskikh nabliudenii).

Concurrences of multiple myeloma with myeloproliferative diseases or secondary myeloid leukemoid reactions are rather rare. The paper describes 3 cases of multiple myeloma: the first case concurrent with neutrophilic leukocytosis; the second case with secondary erythropoetin-dependent erythrocytosis, and the third case with chronic myeloid leukemia. In such cases, an accurate diagnosis requires molecular testing, besides routine clinical and laboratory studies. The paper discusses therapeutic strategy in cases of a concurrence of 2 competing tumors of the blood system: to treat them simultaneously or the most aggressive tumor now, as well as a relationship between multiple myeloma and chronic myeloid leukemia, other myeloproliferative disorders, and secondary myeloid leukemoid reactions.

[Multiple myeloma predominantly involving the spleen].

Extramedullary disease is an uncommon manifestation in multiple myeloma (MM) and can be observed at onset or develop at disease progression or relapse. Splenic involvement is very rare. The paper describes a 52-year-old female patient with MM who in 1990 was diagnosed with monoclonal gammopathy of undetermined significance with IgGkappa secretion and a considerably enlarged spleen. Specific therapy with bortezomib and dexamethasone was initiated in 2006 and proved to be inefficient. After splenectomy there was a 50% reduction in IgGkappa concentration. Splenic histological examination revealed monoclonal infiltration by the pleomorphic plasma cells expressing a kappa light chain.

[Clinical efficiency of transfusion of pathogen-inactivated platelet concentrates].

AIM: To evaluate the effect of pathogen-inactivated platelet concentrates (PIPC) on posttransfusion platelet increments, hemorrhagic syndrome relief, and transfusion intervals. SUBJECTS AND METHODS: This prospective study included 29 hemoblastosis patients (13 women, 16 men), median age 38 years (20-66 years). Pathogens were inactivated by the photodynamic method using the Intecept system. Each patient received two PC transfusions: one PIPC transfusion and one control one. Posttransfusion platelet increments one hour and one day after PC transfusion, the course of hemorrhagic syndrome, and the time to next platelet transfusion were analyzed. RESULTS: Pathogen inactivation with amotosalen and ultraviolet irradiation reduced posttransfusion platelet increments in recipients by 24% after one hour and by 29% after one day after PIPC transfusion versus control ones. CONCLUSION: The clinical efficiency of transfusions of amotosalen-induced PIPC was comparable with that of untreated platelet concentrates. Despite a reduction in post-transfusion platelet increment with the use of PIPC, this caused no significant increase in the frequency of transfusions.