A Case of M-Paraprotein-Associated Polyneuropathy with Stable Response to Rituximab Therapy

Monoclonal gammopathy of undetermined significance is diagnosed on the basis of an extensive search for a diagnostic reason as paraprotein secretion occurs in different diseases. One of polyneuropathies associated with M-paraproteinemia is anti-MAG demyelinating polyneuropathy (AMDP). The first-line treatment of this disease is based on prednisolone, and intravenous immunoglobulin. The second-line therapy of treatment-resistant patients is not determined. We report a case of a female patient with AMDP who received prednisolone, azathioprine, and plasmapheresis; however, stable response was reached only after the use of rituximab

Clinical and Laboratory Characteristics and Differential Diagnosis between Secondary Hemophagocytic Syndrome and Sepsis

Background. Secondary hemophagocytic syndrome (SHPS) and sepsis, although very similar in their clinical manifestations and laboratory parameters, essentially differ in terms of methods of their treatment. SHPS therapy is aimed at immunosuppression, whereas in sepsis anti-infectious treatment is required. To choose the correct therapy a rapid differential diagnosis is necessary. Aim. Search and analysis of criteria of differential diagnosis between SHPS and sepsis. Materials & Methods. The data of 102 patients were analyzed: 55 SHPS patients (median age 60 and range 18–81 years) and 47 sepsis patients (median age 60 and range 18–89 years). SHPS was diagnosed on the basis of HLH-2004 and H-Score criteria. Sepsis was confirmed by documented inflammatory lesions and systemic inflammatory reactions. Microbiologically confirmed sepsis was reported in 10 (21 %) patients. In all sepsis patients multiple organ failure was identified. Results. The study of SHPS and sepsis groups revealed significant differences (p < 0.05) in the levels of C-reactive protein, procalcitonin, creatinine, albumin, and sodium. It was also found out that splenomegaly rate and the levels of triglycerides, ferritin, alanine aminotransferase (ALT), and aspartate aminotransferase (AST) in SHPS were significantly higher than in sepsis, but the levels of glycosylated ferritin (%GF), fibrinogen, leukocytes, neutrophils, and thrombocytes were lower. The following medians (quartiles 1–3) were reported in SHPS and sepsis, respectively: triglycerides (mmol/L) were 3.1 (2.3–3.8) and 1.5 (0.8–2.7), total ferritin (ng/mL) was 7,170 (3,159.2–12,551.0) and 1,274 (559.0–3,041.5), %GF was 26.5 (16.7–37.3) and 54.5 (37.7–71.8), fibrinogen (g/L) was 2.8 (1.4–4.4) and 5.3 (2.8–6.8), ALT (IU/L) was 50 (20–102) and 30 (15.3–55.5), AST (IU/L) was 66 (40.0–105.6) and 36 (24.6–78.0), leukocytes (×109/L) were 3.7 (2.1–5.5) and 8.9 (6.5–14.5), thrombocytes (×109/L) were 56 (25.2–93.5) and 157 (97–308). According to ROC analysis the areas under the curve were as follows: 0.88 for neutrophil level, 0.85 for total ferritin, %GF, leukocytes, and thrombocytes, 0.74 for triglycerides, 0.71 for fibrinogen, 0.65 for sodium, and 0.61 for ALT and AST. Conclusion. In differential diagnosis between SHPS and sepsis most important are the levels of total ferritin, its glycosylated fraction, and triglycerides; less important are fibrinogen, neutrophils, thrombocytes and spleen size. As diagnosis and differential diagnosis between SHPS and sepsis are based on the sum total of clinical and laboratory markers, none of the specified characteristics can serve as a reliable parameter if taken separately

Role of Positron-Emission Tomography in Prognosis of Outcomes of High-Dose Chemotherapy with Autologous Hematopoietic Stem Cell Transplantation in Hodgkin’s Lymphoma

Aim. To perform a comparative analysis of the prognostic significance of positron-emission tomography (PET) with other prognostic factors of the efficacy of high-dose chemotherapy (HDCT) with autologous hematopoietic stem cell transplantation (auto-HSCT) in patients with Hodgkin’s lymphoma. Methods. Data on 84 patients with Hodgkin’s lymphoma receiving treatment over the period from October 2007 till November 2015 were analyzed. The median age was 26.6 years (range: 10–62). The median follow-up was 25 months (range: 1–81 months). The prognostic significance of sex, response to the initial chemotherapy, time to relapse, second-line chemotherapy regimen type, B-symptoms, tumor size (>5 cm in cases of relapse prior to the HDCT), serum LDH and albumin levels, CT findings, the number of chemotherapy lines, conditioning regimen before the auto-HSCT, and the metabolic activity before the HDCT (PET1, n = 82) and after auto-HSCT (PET2, n = 57) was analyzed. Results. The two-year overall (OS) and event-free (EFS) survival rates were 70.6 % and 58.7%, respectively. Prognosis was the worst in patients with CT-confirmed lymphoma progression by the initiation of HDCT. In the presence of a CT-response, the PET status of lymphoma has a prognostic significance. The 2-year OS and EFS rates of PET1-negative and PET1-positive patients were 82 % vs. 62 % (p = 0.056) and 74 % vs. 44 % (p = 0.003), respectively. In PET2-negative and PET2-positive patients, the OS and EFS rates were 90 % vs. 65 % (p = 0.013) and 72 % vs. 52 % (p = 0.014), respectively. From the prognostic point of view, PET2 findings prevailed over PET1 findings. The multivariate analysis confirmed only PET2 significance for OS prediction. Conclusion. The tumor sensitivity to the chemotherapy assessed by the CT is the most important prognostic factor. In case of a positive CT dynamics, the achievement of PET1 or PET2 negativity before or after HDCT/auto-HSCT is a favorable prognostic factor. The worst prognosis was observed in patients with tumor metabolic activity before or after HDCT/auto-HSCT