This multicenter retrospective study included 101 patients (median age 62 years) with secondary plasma cell leukemia (sPCL). The median time from initial multiple myeloma diagnosis to sPCL was 31 months. Fifty-five out of 72 patients (75%) who received any therapy were treated with immunomodulators (IMiDs) and/or proteasome inhibitors (PIs), and 14/72 (19%) underwent salvage autologous stem cell transplantation (ASCT). The overall response rate in patients who received ASCT or PI (either alone or in combination) was higher than in those who did not (93% vs. 36% and 60% vs. 30%, respectively). The median overall survival (OS) in patients who received therapy was 4.2 months (95% CI: 1.3; 8.0) with a 1-year OS of 19%. Platelet count ≤100 × 10/L at sPCL diagnosis was the only independent predictor of a poorer OS in treated patients (HR = 3.98, p = .0001). These findings suggest that patients with sPCL may benefit from salvage ASCT- and PI-based regimens.

Induction therapy in patients with multiple myeloma increases the risk of thromboembolism. We have recently shown that multiple myeloma patients tend to form denser fibrin clots displaying poor lysability. We investigated the effect of induction therapy on fibrin clot properties in multiple myeloma patients. Ex-vivo plasma fibrin clot permeability, turbidity, susceptibility to lysis, thrombin generation, factor VIII and fibrinolytic proteins were compared in 48 multiple myeloma patients prior to and following 3 months of induction therapy, mainly with cyclophosphamide-thalidomide-dexamethasone regimen. Patients on thromboprophylaxis with aspirin or heparins were eligible. A 3-month induction therapy resulted in improved clot properties, that is higher clot permeability, compaction, shorter lag phase and higher final turbidity, along with shorter clot lysis time and higher rate of D-dimer release from fibrin clots than the baseline values. The therapy also resulted in lower thrombin generation, antiplasmin and thrombin-activatable fibrinolysis inhibitor (TAFI), but elevated factor VIII. Progressive disease was associated with lower posttreatment clot permeability and lysability. Despite thromboprophylaxis, two patients developed ischemic stroke and 10 had venous thromboembolism. They were characterized by pretreatment lower clot permeability, prolonged clot lysis time, longer lag phase, higher peak thrombin generation, TAFI and plasminogen activator inhibitor -1. Formation of denser plasma fibrin clots with reduced lysability and increased thrombin generation at baseline could predispose to thrombotic complications during induction treatment in multiple myeloma patients. We observed improved fibrin clot properties and thrombin generation in multiple myeloma patients except those with progressive disease.

Background. The incidence of amyloidosis is difficult to determine because the disease is often undiagnosed or diagnosed incorrectly. In Polish studies, there are no statistics and analyses of the factors that may influence the development of amyloidosis in patients with multiple myeloma Objectives. The goal of this study was to estimate the incidence of aL amyloidosis in MM patients in Lower Silesia region. Material and Methods. 70 patients treated at the department of Hematology, Provincial Hospital in Legnica and the department of Hematology, blood Neoplasm and bone Marrow Transplantation, Medical University in Wroclaw were enrolled in the survey. 37 patients were newly diagnosed, 33 had been treated for 2-34 months. The basis for the diagnosis of amyloidosis was the presence of green colored amyloid deposits in the polarized light microscope in the adipose tissue (received from abdominal fold and stained with congo red). Results. amyloidosis was diagnosed in 18 (25.7%) patients with MM, 9/9 f/M, aged 47-83 years. 6 (33%) pts with amyloidosis had newly diagnosed MM, in 12 (67%) progression of the disease was diagnosed. amyloidosis occurred significantly more often (p = 0.048) in already treated patients. The odds ratio (OR) was 2.95. amyloidosis occurred most frequently in patients with Igg myeloma (67%), (OR = 1.98), was more often found in patients with kappa light chain versus lambda, respectively 67% and 33%. The probability of amyloidosis in patients with clinical stage III was 1.5 times higher (p = 0.05) than in other stages (OR = 1.5), in persons with renal dysfunction was twice as high (OR = 2.4) compared to the renal competence group (p = 0.05). Conclusions. aL amyloidosis in the course of MM occurs in Lower Silesia region with a comparable rate to other regions of the world. It is significantly more often diagnosed in patients with relapsed or refractory disease, in persons with clinical stage III and with renal failure (Adv Clin Exp Med 2014, 23, 2, 235-244).

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Background. AL amyloidosis is an acquired systemic disease in which a pathologic amorphous substance produced as a result of abnormal protein metabolism is deposited in the extracellular space of various tissues. Objectives. The aim of the study was to investigate the relationship between the kappa and lambda serum free light chains (sFLCs) and the development of AL amyloidosis in patients suffering from multiple myeloma (MM). Material and Methods. The investigations included 70 MM patients, 40 females and 30 males, aged 28-83 years. In 37 persons, MM was had been diagnosed recently; 33 patients had been undergoing treatment. Amyloidosis was diagnosed in 18 patients (25.7%), including nine females, nine males; six had newly diagnosed disease. Fifteen patients developed kidney failure. The control group consisted of 10 healthy donors. The concentration of sFLC ls were determined using the immunonephelometric method and expressed in mg/L. Results. In 18 MM patients with amyloidosis the concentration of κ sFLCs ranged from 0.3 to 4780 (x = 854.5, SD = 1289), and was significantly higher (p = 0.039) than in the group without amyloidosis, where the range was from 0.3 to 426.0 (x = 68.9, SD = 98.1). The highest concentration of κ sFLCs was observed in the group of five patients with amyloidosis and renal failure. The concentration of λ sFLCs in patients with amyloidosis ranged from 0.5 to 41600 (x = 3035.7, SD = 9735) and was higher than in MM patients without amyloidosis, where it ranged from 0.5 to 834.0 (x = 79.3, SD = 193). In amyloidosis patients, the concentration of λ sFLCs was significantly higher (p = 0.05) in cases of renal failure as compared with the patients with normal renal function. Conclusions. The concentration of sFLCs is a strong indicator of amyloidosis development in MM patients (Adv Clin Exp Med 2014, 23, 4, 531-538).