AL cardiac amyloidosis is a relatively rare disorder that belongs to the group of infiltrative cardiomyopathies. Diagnosis of primary amyloidosis is challenging due to many unspecific symptoms and sings, which often leads to late diagnosis when treatment options are limited. Primary amyloidosis particularly needs to be excluded in patients with heart failure with preserved ejection fraction. Therapy in cardiac amyloidosis has to main vectors: 1) chemotherapy to eliminate amyloidogenic plasmatic cells 2) heart failure treatment. The main challenge for cardiologists is to support hemodynamics until response to chemotherapy occurs. In the article the issue of diagnostics, risk stratification and treatment of primary cardiac amyloidosis is addressed.

CR of the disease (median follow-up of 80 months). In 3/14 pts had early relapses. From 89/126 pts, who completed therapy only R-CHOP and maintenance therapy R, 50/89 (56%) pts have developed recurrence of the disease. 39/89 (44%) pts have CR (the median follow-up of 80 months). In 11/203 (5 %) patients there was a resistant course of the tumor. 5-year OS in the R-B group is higher than in the R-CHOP group: 92% (Standard Error, ± 4%) vs. 87% ( ± 3%). Frequency analysis shows that the number of deaths in different regimens differ statistically significantly: 4/62 (6.5%, R-B) vs. 21/105 (20.0%, R-CHOP), Fisher's exact test, p = 0.05; relative risk (R-CHOP/R-B): 2.8 (95% CI: 0.9-7.7). Summary/Conclusion: In the analysis of 203 pts, independent predictors of OS and EFS in FL were determined. The high risk of adverse events associated with 3A cytological type of tumor. R-B is more effective in FL of 1-2 cytological type than 3A. In the presence of bulky and FLIPI 3-5 relapse occurs three times more often after R-CHOP (76%) than after high-dose therapy. R-B effectively sanitizes the bone marrow allows you to perform the successful mobilization of blood stem cells before autoSCT. Analysis of the results of immunohistochemistry in patients resistant to therapy showed that in all cases the tumor expressed CD20, in 8/11(73%) cases -CD10, in 7/11(64%) cases -BCL-2, and in one case(1/11 (9%) -there was a peripheral ''aureole'' of T-lymphocytes, framing tumor nodules, which have a deterrent effect.

Introduction. Heavy-chain diseases (HCDs) are rare B-cell lymphoproliferative diseases that do not have a classical clinical picture. A characteristic feature of this disease is the secretion of fragmented heavy chains of various immunoglobulin isotypes. Currently, there are four known variants of this disease: μ, γ, α, and δ.Aim. To describe the clinical observation of μ-HCD, hidden under the mask of systemic amyloidosis, and the associated diffi culties of primary diagnosis.Main Findings. A rare clinical case of μ-HCD in combination with systemic amyloidosis (light chain amyloidosis-AL), transthyretin amyloidosis (transthyretin amyloidosis-ATTR), and non-amyloid deposits in a 64-year-old patient is presented. The severity of the condition was due to the clinical picture of chronic heart failure, polyneuropathy. Upon examination, Waldenstrom’s macroglobulinemia was diagnosed while a diagnosis of amyloidosis was not established. Immuno-chemotherapy was performed under the RB program (rituximab and bendamustine). The effect of the therapy was minimal and short-term. The patient’s condition progressively worsened, and the patient died due to acute cardiovascular failure. The main diagnosis was revised in favor of μ-HCD. The autopsy revealed widespread amyloid and non-amyloid lesions of organs and tissues. Conflict of interest: the authors declare no conflict of interestFinancial disclosure: the study had no sponsorship